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*APSFA's Favorites
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**APS
Foundation of America, Inc |
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Founded in June 2005, the APS Foundation
of America, Inc. is dedicated to fostering and facilitating
joint efforts in the areas of education, support, research,
patient services and public awareness of Antiphospholipid
Antibody Syndrome in an effective and ethical manner. |
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**APS
Friends & Support Forum |
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A forum run by Heidi & Tina founders of
the APS Foundation of America, Inc., a non profit
organization. This forum is an information source and a
friendly support group for people who have Antiphospholipid
Antibody Syndrome or for anyone who's lives are touched by
it. It is sometimes referred to as APS, APLS, or APLA and is
known as Hughes Syndrome or "Sticky Blood" in the UK. APS is
associated with recurrent clotting events including
premature stroke, repeated miscarriages, phlebitis, venous
thrombosis and pulmonary thromboembolism. If this disease
touches your life in some way, please feel free to join in
our discussions! :) We're glad to have you visit! |
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**GoodSearch.com |
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What if APS Foundation of America - APSFA
earned a penny every time you searched the Internet? Well,
now we can! GoodSearch.com is a new search engine that
donates half its revenue, about a penny per search, to the
charities its users designate. You use it just as you would
any search engine, and it’s powered by Yahoo!, so you get
great results. Just go to http://www.goodsearch.com and be
sure to enter APS Foundation of America - APSFA as the
charity you want to support. Just 500 of us searching four
times a day will raise about $7300 in a year without anyone
spending a dime! And, be sure to spread the word! |
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*Antiphospholipid Antibody Syndrome (APS) Booklet |
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written by the APS Foundation of America,
Inc. This pamphlet is a layman’s terms summary of
Antiphospholipid Syndrome (APS). It covers such topics as
diagnosis, symptoms, treatment, and coping. It is meant for
patients newly diagnosed, however would also be good for
informing friends and family about your disease. |
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*Antiphospholipid Antibody Syndrome (APS) Booklet |
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written by the APS Foundation of America,
Inc. |
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*APS Foundation of America Brochure |
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written by the APS Foundation of America,
Inc. |
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*Medic Alert |
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HIGHLY recommended! This jewerly could
save your life! |
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*National Alliance for Thrombosis and Thrombophilia (NATT) |
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The National Alliance for Thrombosis and
Thrombophilia (NATT) is a nationwide, non-profit patient
advocacy group representing the interests of people with
blood clots and clotting disorders, including people with
the APLA syndrome. NATT’s mission is to address major
treatment issues, such as preventing thrombosis and its
complications, and reducing death and illness related to
thrombosis. NATT wants patients to get involved. |
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Antiphospholipid Antibodies |
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Circulation. 2005;112:e39-e44. © 2005
American Heart Association, Inc. Caron P. Misita, PharmD;
Stephan Moll, MD |
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Antiphospholipid Antibody Syndrome and Pregnancy |
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Background: Antiphospholipid syndrome
(APS) is a recently recognized autoimmune condition that may
manifest with fetal loss, thrombosis, or autoimmune
thrombocytopenia. Women with these clinical features should
be tested for lupus anticoagulant (LAC) and anticardiolipin
(aCL) antibodies; most patients with APS have both LAC and
aCL immunoglobulin G (IgG) antibodies. The diagnosis of APS
requires the presence of both clinical and biological
features. Systemic lupus erythematosus (SLE) is a chronic
systemic disease with diverse clinical and laboratory
manifestations. LAC (and aCL) predisposes to clotting in
vivo, predominantly by interfering with the antithrombotic
role of phospholipids (PLs); therefore, it is associated
with clinical thrombosis, not bleeding. The antiphospholipid
(aPL) autoantibodies bind moieties on negatively charged PLs
or moieties formed by the interaction of negatively charged
PLs with other lipids, PLs, or proteins. aPL antibodies
belong to the large family of antibodies that react with
negatively charged PLs, including cardiolipin,
phosphatidylglycerol, phosphatidylinositol,
phosphatidylserine, phosphatidylcholine, and phosphatidic
acid. Last Updated: September 4, 2005 |
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Do you
have Lupus Anticoagulants? |
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The APSFA is currently working with a
company called Precision BioLogic and they are looking for
people who are positive with the Lupus Anticoagulant (LA) to
donate plasma. We are strongly urging people who qualify to
take part in this opportunity as it will only make future
testing more accurate! |
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eMedicine - Antiphospholipid Antibody Syndrome : Article by
Barry L Myones, MD |
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Last Updated: October 26, 2004 Promtes an
INR in the range of 2.5-3.5. |
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eMedicine - Antiphospholipid Syndrome : Article by Steven
Carsons, MD |
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Last Updated: December 5, 2004 Based on
the most recent evidence, a reasonable target for the
international normalized ratio (INR) is 2.6-3 for a minimum
of 6 months for a first thrombosis. Patients with recurrent
thrombotic events while well maintained on the above regimen
may require an INR of 3-4 and generally receive
anticoagulation therapy for life. For severe or refractory
cases, a combination of warfarin and aspirin may be used. |
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FDA Vitamin K List |
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General Management of the Patient with a Positive
Antiphospholipid Antibody Test: What Evidence Is Available
For You and Your Physician To Consider? |
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Written by: Gale A McCarty, MD, FACP,
FACR You are an individual and your particular case may
involve some features that separate you from the study
patients. What is most likely to have the right balance of
“helpful vs. harmful” effects on you is the major concern of
your physician to prevent the effects of aPL antibodies in
contributing to blood clots and cell or organ damage in you.
Let’s look at the major approaches used to manage your
positive aPL test. |
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General Management of Thromboses (Blood Clot) II—What
Evidence is Available For You and Your Physician To
Consider? |
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Written by: Gale McCarty, MD, FACP, FACR.
As always, the physician treating the individual APS patient
has to balance the wisdom of these guidelines (which are
general guidelines and not mandates applicable to ALL
patients) with the needs of his/her patient to find the best
answer. |
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HYDROXYCHLOROQUINE - EVERYTHING OLD IS NEW AGAIN! |
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By: Gale McCarty, MD, FACR, FACP.
Hydroxychloroquine (HCQ, or its trade name-Plaquenil) has a
long and honored history of use in systemic lupus
erythematosus (SLE) as a general medication to decrease
activity of the immune system and decrease symptoms. For
years it has been approved for use by the FDA for lupus and
rheumatoid arthritis, and has been used most frequently for
skin and joint manifestations. It is considered a mainstay
of therapy for any patient with SLE by many lupus experts
and rheumatologists. It has many mechanisms of action, some
related to decrease in the activity of the immune system,
and some related to effects on blood clotting mechanisms.
HCQ belongs to the class of drugs call anti-malarials, which
includes Chloroquine and Atabrine. (This does not mean that
anyone thinks that SLE or APS is caused by the agent that
causes malaria-like most discoveries in medicine, it was the
chance observation that patients with some autoimmune
diseases who got anti-malarial drugs to prevent malaria when
traveling to likely areas of infection noted their symptoms
improved on HCQ). One of the most complete and excellent
reviews of all the literature on the anti-malarials to which
all patients and their physicians are directed is Dr. Dan
Wallace’s Chapter 59 in the Wallace-Hahn Dubois’ Lupus
Erythematosus textbook. Another excellent review on APS
therapy in general has been published by Dr. Robert Roubey. |
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I Have the Lupus Anticoagulant, But I Don’t Have Lupus? |
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By: Thomas L. Ortel, MD, PhD “...although
the ‘lupus’ anticoagulant was first described in several
patients with lupus, most patients with lupus anticoagulants
actually don’t have any of the other clinical manifestations
of lupus." |
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Listen to the Patient — Anticoagulation Is Critical in the
Antiphospholipid (Hughes) Syndrome |
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© 2003. The Journal of Rheumatology
Publishing Company Limited. Promotes an INR of greater than
3.0. |
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Lupus Anticoagulant: Testing While on Anticoagulant Therapy:
Can It Be Done? |
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Written by: Thomas L Ortel, MD, PhD. In
conclusion, it is optimal to test for a lupus anticoagulant
when the patient is on no anticoagulant therapy. All of the
test results can be interpreted more easily in that setting.
Sometimes this can be difficult to arrange, however, and
testing needs to be performed while the patient is still
taking anticoagulants. In this situation, the doctor needs
to work carefully with the laboratory, to understand how the
tests are being performed and to make sure that the results
are interpreted correctly. |
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Medical Progress- Antiphospholipid Antibody Syndrome |
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PDF File from the New England Journal of
Medicine |
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Migraine, memory loss, and "multiple sclerosis ".
Neurological features of the antiphospholipid (Hughes’)
syndrome |
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Postgraduate Medical Journal
2003;79:81-83 © 2003 Fellowship of Postgraduate Medicine.
Promotes an INR of greater than 3.0. |
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Monitoring Warfarin Therapy in Patients with Lupus
Anticoagulants |
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Recommended therapeutic international
normalized ratios (INRs) for oral anticoagulation in
patients with lupus anticoagulants who sustain a
thromboembolic event are controversial. Patients with lupus
anticoagulants often have a prolonged prothrombin time,
which may complicate management of anticoagulant therapy. |
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New subsets of the antiphospholipid syndrome in 2006:
"PRE-APS" (probable APS) and microangiopathic
antiphospholipid syndromes ("MAPS"). |
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Autoimmun Rev, December 1, 2006; 6(2):
76-80. The concept of "probable" antiphospholipid syndrome
(APS) is almost identical with several conditions which may
presage the development of the APS with its major
complications of large vessel thromboses resulting in deep
vein occlusions in the lower limbs (DVT) particularly and
strokes. These conditions comprising livedo reticularis,
chorea, thrombocytopenia, fetal loss and valve lesions.
These conditions, comprising livedo reticularis, chorea,
thrombocytopenia, fetal loss and valve lesions may be
followed, often years later by diagnosable APS. The issue
whether these patients should be more aggressively treated
on presentation in order to prevent the thrombotic
complications. A new subset of the APS is proposed viz.
microangiopathic antiphospholipid syndrome ("MAPS")
comprising those patients presenting with thrombotic
microangiopathy and demonstrable antiphospholipid antibodies
who may share common although not identical provoking
factors (e.g. infections, drugs), clinical manifestations
and haematological manifestations (severe thrombocytopenia,
hemolytic anaemia) and treatments viz. plasma exchange.
Patients without large vessel occlusions may be included in
the MAPS subset. These conditions include thrombotic
thrombocytopenic purpura (TTP), hemolytic-uremic syndrome
(HUS), and the HELLP syndrome. Patients with catastrophic
antiphospholipid syndrome (CAPS) who do not demonstrate
large vessel occlusions also fall into this group.
Disseminated intravascular coagulation (DIC) has also been
reported with demonstrable antiphospholipid antibodies and
also manifests severe thrombocytopenia and small vessel
occlusions. It may cause problems in differential diagnosis. |
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Predicting Thrombosis Risk in Individuals with
Antiphospholipid Antibodies |
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Written by: Thomas L Ortel, MD, PhD
“...several studies have shown that the presence of a lupus
anticoagulant in the blood is associated with a higher risk
for a clot than the presence of an anticardiolipin
antibody." |
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Rare Diseases Clinical Research Network |
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The Rare Diseases Clinical Research
Network was created to facilitate collaboration among
experts in many different types of rare diseases. Our goal
is to contribute to the research and treatment of rare
diseases by working together to identify biomarkers for
disease risk, disease severity and activity, and clinical
outcome, while also encouraging development of new
approaches to diagnosis, prevention, and treatment. |
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Rare Thrombotic Diseases Consortium (RTDC) |
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The Rare Thrombotic Diseases Consortium
(RTDC) is an integrated group of academic medical centers,
patient support organizations, and clinical research
resources dedicated to conducting clinical research in
different forms of and improving the care of patients with
thrombotic diseases. Funded by the National Institutes of
Health (NIH), the RTDC is part of the Rare Diseases Clinical
Research Network. The operations of the RTDC are directed
from Duke University. Other primary RTDC study sites include
the University of North Carolina, University of Wisconsin,
Centers for Disease Control and Prevention, and the Mayo
Clinic. |
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Sapporo Criteria for Diagnosing APLA Syndrome |
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SERONEGATIVE ANTIPHOSPHOLIPID ANTIBODY SYNDROME (SNAPS)…AND
SNAPPING TO IT!! |
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By: Gale McCarty, MD, FACR, FACP. “You
don’t have the syndrome because your tests are low level or
negative…” or “You have livedo, a heart valve problem, and
thrombocytopenia, but these aren’t listed as criteria for
diagnosis” are comments made frequently by healthcare
providers from many specialties to patients with clinical
features suggesting the Antiphospholipid Antibody Syndrome
(APS). |
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Surgical Management of the Primary Dental Patient on
Warfarin |
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Written to inform Dentists about treating
Warfarin Patients |
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Thrombosis and the Antiphospholipid Syndrome |
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Hematology 2005 © 2005 The American
Society of Hematology. Summary: Even with the most complete
datasets, it is still important for the physician to develop
a therapeutic plan appropriate for the individual patient,
based on clinical presentation, co-morbid conditions, and
other variables. With uncommon disorders and limited
datasets, such as with the antiphospholipid syndrome,
decision-making becomes even more difficult. Table 3
presents a strategy that the author uses when evaluating and
developing a treatment plan for a patient with
antiphospholipid syndrome and thrombosis, based on the
available studies summarized in this article. Critical areas
for future research include identifying which patients with
antiphospholipid antibodies are at highest risk for
thrombotic complications, developing new antithrombotic
agents that are effective and safe, and investigating novel
approaches to eliminate the autoantibody and, hopefully, the
increased prothrombotic state. |
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Thrombosis Interest Group of Canada |
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The Thrombosis Interest Group of Canada
consists of a group of 40 specialists in fields related to
thrombosis who collaborate to write evidence-based or
consensus-based clinical guides on the investigation,
management, and diagnosis of thrombotic disorders. |
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Warfarin in Antiphospholipid Syndrome — Time to Explore New
Horizons |
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The Journal of Rheumatology Feb. 2005
Promotes an INR greater than 3.0. |
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What is “Micro-Clotting”? |
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By: Thomas L. Ortel, MD, PhD Simply put,
micro-clotting, better referred to as “microvascular
thrombosis”’ describes blood clotting that is occurring in
some of the smallest blood vessels in the body. |
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Top of Page |
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*In the News & Community - APSFA
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American Stroke Association: Stroke Connection Magazine,
November/December 2006, Page 44 |
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Antiphospho....what?! |
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The APS Foundation of America, Inc. is
the only United States nonprofit health agency dedicated to
bringing national awareness to Antiphospholipid Antibody
Syndrome (APS), the major cause of multiple miscarriages,
thrombosis, young strokes and heart attacks. We are a
volunteer run, community based 501(c)3 non-profit Public
Charity organization and is dedicated to fostering and
facilitating joint efforts in the areas of education,
support, public awareness, research and patient services. |
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Antiphospholipid Antibody Syndrome (APS) Sufferers Start
Non-profit Health Agency: Announcing the APS Foundation of
America official website www.apsfa.org |
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Press Release - October 27, 2007 on PRWEB |
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Antiphospholipid Antibody Syndrome Awareness Month |
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Michigan Proclamation for 2007 |
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APS Awareness PSA |
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APS Awareness PSA for WFLS |
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PSA airing on 93.3 WFLS- Real Country
Variety, Fredericksburg, VA |
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APS Awareness Starts Here |
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Written by: Charles Strickler. What is
APS? How do we increase awareness of APS? What research
needs to be done to improve treatment options? These were
some of the many questions discussed at the awareness and
fundraising dinner that was hosted by me, my wife, Mary
Strickler and our co-hosts, David and Kim Penberthy. We held
the dinner May 17th, and it seemed more than appropriate to
have such an event just prior to APS Awareness Month in
June! |
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APS FOUNDATION OF AMERICA, INC. JOINS WITH NORD TO CELEBRATE
THE 25th ANNIVERSARY OF THE ORPHAN DRUG ACT |
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APSFA Press Release - January 2008 |
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APS in the Community |
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On April 22nd the APSFA made an
appearance at the annual March of Dimes WalkAmerica event in
Springfield, Virginia. |
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APS is a snake in the grass and can bite or kill without
warning |
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APSFA Press Release - Week 4 - 2007 |
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APS Linked to Birth Difficulties |
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APSFA Press Release - Week 2 - 2007 |
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APSFA To the Rescue |
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Written by: Seren Estrada Thanks to the
support of the APSFA, I expect to continue my education with
the help of the DSSO. It also goes to show that the APS
Foundation of America, Inc is truly dedicated to advocating
for people with APS. |
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Eau Claire, Leader-Telegram, November 29, 2006, Page 1C |
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Grand Rounds at Marshfield Clinic |
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written by Tina Pohlman |
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June is APS Awareness Month PSA |
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June is Antiphospholipid Antibody
Syndrome (APS) Awareness month. |
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June is APS Awareness Month ~ Get In the Flow! |
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Written by: Tina Pohlman |
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June is APS Awareness Month: Get in the Flow! |
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The APS Foundation of America, Inc.
(APSFA) has declared June as National Antiphospholipid
Antibody (APS) Awareness Month. We are educating the public
and medical community about this disorder, urging people to
Get in the Flow! |
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JUNE IS APS AWARENESS MONTH: GET IN THE FLOW! |
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APSFA Press Release - Week 1 - 2007 |
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Lupus Foundation of America: Pacific Northwest Chapter: In
the Lupe! December 2006, Page 5 |
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Lupus Foundation of Ontario: Lupus Talk, October 2006 to
December 2006, page 5 |
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MO-KAN Spreads APS Awareness |
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Written by Dana Stuart. “Mo-Kan” is a
phrase commonly used in the Missouri-Kansas region to
describe various missions or goals that Missouri and Kansas
businesses and residents CAN accomplish together. Frequently
used for public service announcements, advertisements, and
other miscellaneous uses, “Mo-Kan” has become the unofficial
slogan for several alliances, which have formed for some
very worthy causes. Now “Mo-Kan” spreads APS Awareness! |
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One Fine Day |
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Written by: Dana Stuart |
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Our Journey with Mystery Diagnosis |
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written by Michelle LaRue |
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Proclamation 2007 - State of CT |
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Thank you for your support! |
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Proclamation 2007 - State of KY |
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Thank you for your support! |
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Proclamation 2007 - State of MO |
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Thank you for your support! |
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Proclamation 2007 - State of OH |
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Thank you for your support! |
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Proclamation 2007 - State of PA |
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Thank you for your support! |
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Proclamation 2007 - State of WI |
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Thank you for your support! |
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Quarterly Newsletter "Antiphospho....What?" |
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Volume 6 - Summer 2007. written by the
APS Foundation of America, Inc. |
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Quarterly Newsletter "Antiphospho....What?" |
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Volume 2 - Summer 2006. written by the
APS Foundation of America, Inc. |
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Quarterly Newsletter "Antiphospho....What?" |
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Volume 1 - Spring 2006. written by the
APS Foundation of America, Inc. |
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Quarterly Newsletter "Antiphospho....What?" |
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Volume 5 - Spring 2007. written by the
APS Foundation of America, Inc. |
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Quarterly Newsletter "Antiphospho....What?" |
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Volume 3 - Fall 2006. written by the APS
Foundation of America, Inc. |
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Quarterly Newsletter "Antiphospho....What?" |
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Volume 4 - Fall 2006. written by the APS
Foundation of America, Inc. |
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Quarterly Newsletter "Antiphospho....What?" |
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Volume 7 - Fall 2007. written by the APS
Foundation of America, Inc. |
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Quarterly Newsletter "Antiphospho....What?" |
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Volume 8 - Winter/Spring 2008. written by
the APS Foundation of America, Inc. |
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Quarterly Newsletter "Antiphospho....What?" |
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Volume 9 - Spring/Summer 2008. Written by
the APS Foundation of America, Inc. |
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Rare condition often overlooked |
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By Shannon Farr, Saturday, June 30, 2007 |
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Rare disorder's diagnosis draws media attention |
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Published: Sunday, November 20, 2005
12:03 AM CST. A Ventura teen’s struggle to obtain a
diagnosis for a relatively rare auto-immune disorder will be
featured on the Discovery Health Channel. Angie Abbas, 18,
who has Antiphospholipid Antibody Syndrome (APS), will be
featured in a segment of “Mystery Diagnosis” in early 2006.
The date has not been determined. Angie’s diagnosis was
difficult because APS mimics other disorders, LaRue said. In
addition, Angie’s primary symptom, a severe twitching of the
head and neck, is not typical. In the meantime, producers of
“Mystery Diagnosis” had learned about Angie through the APS
Foundation of America, a relatively new organization in
which Angie and her mother had become active. |
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Sisters' ailment identified |
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07/23/2007 - By Christy Murdoch , For the
Herald-Standard |
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Strokes and heart attacks could be caused by APS |
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APSFA Press Release - Week 3 - 2007 |
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Thrombosis Education Days ~ MI & Chicago |
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written by Heidi Ponagai. The National
Alliance of Thrombosis and Thrombophilia (NATT) and the MSU
Center for Bleeding and Clotting Disorders held an education
day on March 24th in Lansing, Michigan for people who have
had blood clots, who have clotting disorders, and their
families and friends. We were fortunate enough to get a
booth at the seminar to display and distribute APSFA
brochures and booklets to people. |
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Ventura teen on ‘Mystery Diagnosis’ on March 6 |
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VENTURA — A program featuring a Ventura
teen’s struggle to obtain a diagnosis for a relatively rare
auto-immune disorder will be aired March 6 on the Discovery
Health Channel, the show’s producers have announced. Angie
Abbas, 18, who has Antiphospholipid Antibody Syndrome (APS),
will be featured at 9 p.m. Central Standard Time on March 6.
“Mystery Diagnosis” is a series that tells the stories of
how real people have obtained difficult diagnoses. It is
produced through True Entertainment LLC of New York City.
The program was filmed in October at a Minneapolis
production studio, Mercy Medical Center-North Iowa in Mason
City and Angie’s home in rural Ventura. The Discovery Health
Channel is Channel 203 on Mediacom’s digital-plus package or
Channel 93 through CL Tel. |
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WFLS 93.3 APSFA Air Check - Sample 1 |
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These are air checks that are currently
hitting the DC Metro Area. About 1 million listeners at any
given time. |
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WFLS 93.3 APSFA Air Check - Sample 2 |
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These are air checks that are currently
hitting the DC Metro Area. About 1 million listeners at any
given time. |
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WFLS 93.3 APSFA Air Check - Sample 3 |
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These are air checks that are currently
hitting the DC Metro Area. About 1 million listeners at any
given time. |
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Top of Page |
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APS - *General
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*Antiphospholipid Antibody Syndrome |
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Last Updated: October 26, 2004 Author:
Barry L Myones, MD, Director of Research, Pediatric
Rheumatology Center, Texas Children's Hospital at Houston;
Associate Professor, Departments of Pediatrics & Immunology,
Pediatric Rheumatology Section, Baylor College of Medicine
Coauthor(s): Deborah McCurdy, MD, Director of Rheumatology,
Department of Pediatric Rheumatology, Children's Hospital of
Orange County Barry L Myones, MD, is a member of the
following medical societies: American Academy of Pediatrics,
American Association for the Advancement of Science,
American Association of Immunologists, American College of
Rheumatology, American Heart Association, American Society
for Microbiology, Clinical Immunology Society, and Texas
Medical Association Editor(s): Terry Chin, MD,
Allergy/Immunology/Pulmonology; Co-Director Cystic Fibrosis
and Home Ven, Associate Professor of Pediatrics, Department
of Pediatrics, Loma Linda University and Children's
Hospital; Mary L Windle, PharmD, Adjunct Assistant
Professor, University of Nebraska Medical Center College of
Pharmacy; David D Sherry, MD, Professor of Pediatrics,
University of Pennsylvania; Director of Clinical
Rheumatology, Division of Rheumatology, Children's Hospital
of Philadelphia; Daniel Rauch, MD, Director, Pediatric
Hospitalist Program, Associate Professor, Department of
Pediatrics, New York University School of Medicine; and
Norman T Ilowite, MD, Chief, Division of Rheumatology,
Schneider Children's Hospital; Professor, Department of
Pediatrics, Albert Einstein College of Medicine |
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*Antiphospholipid Antibody Syndrome (APS) Booklet |
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written by the APS Foundation of America,
Inc. This pamphlet is a layman’s terms summary of
Antiphospholipid Syndrome (APS). It covers such topics as
diagnosis, symptoms, treatment, and coping. It is meant for
patients newly diagnosed, however would also be good for
informing friends and family about your disease. |
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*Antiphospholipid Antibody Syndrome (APS) Booklet |
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written by the APS Foundation of America,
Inc. |
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*Antiphospholipid Syndrome (APS) |
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Written by: Jan M. Pankey, M.D.
Children’s Hospital & Research Center Oakland, California
for the National Alliance for Thrombosis and Thrombophilia
(NATT) |
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*APS & Surgery Brochure |
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written by the Hospital for Special
Surgery |
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*APS & You |
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written by the APS Foundation of America,
Inc. This pamphlet is a layman’s terms summary of
Antiphospholipid Syndrome (APS). It covers such topics as
diagnosis, symptoms, treatment, and coping. It is meant for
patients newly diagnosed, however would also be good for
informing friends and family about your disease. |
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*APS Information Brochure |
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written by the Hospital for Special
Surgery. |
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*Current News Articles Dealing with APS & Related Problems |
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This link contains full news articles and
many up to date medical journal abstracts dealing with APS
and its related diseases. Free Registration is required. |
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*The Antiphospholipid Syndrome |
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written by Dr. Yaniv & Professor Yehuda
Shoenfeld. Copyright 2004. Redistributed with permission
from authors. |
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*What is Antiphospholipid Antibody Syndrome? |
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written by the Rare Thrombotic Disease
Consortium. Redistributed with permission from the authors. |
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*Women & APS |
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written by the APS Foundation of America,
Inc. |
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A Patients Guide to APS |
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Doctor Hughes Book on a website |
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About thrombosis: thrombophilia: acquired thrombophilia: APS |
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Another article that supports an INR of
3.0 to 4.0 for APS patients. |
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American Venous Forum - Patients Section |
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Founded in 1988, the American Venous
Forum provides a serious academic colloquium to physicians
interested in the research, education, and clinical
investigation in the field of venous diseases. The Forum
membership includes more than 225 board-certified vascular
surgeons who have an accomplished record of interest and
contribution to the management of venous disease. The
mission of the American Venous Forum is to improve the care
of patients with venous and lymphatic disorders by providing
a forum dedicated to education and to the exchange of
information concerning basic and clinical research
pertaining to the venous and lymphatic systems. In past
years, the Forum has dedicated itself to academic pursuits
through vigorous educational meetings throughout the
country. Today, the Forum is a diverse organization that
includes a directory of experienced investigators and
clinicians, several of whom can also speak or assist in
research on a variety of venous-related topics. The Forum
also offers guidelines and protocols for the development of
research and clinical trials. With the guidance of the AVF
governing officers, the AVF will continue to bring medical
professionals and patients the latest venous health
information. The AVF By-Laws include more information on our
objectives, committees, meetings, and dues. |
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Anti-phospholipid antibody syndrome |
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The MayoFoundation for Medical Education
and Research provides the following web link addressing
antiphospholipid syndrome. |
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Anticardiolipin Test and the Antiphospholipid (Hughes)
Syndrome: 20 Years and Counting! |
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© 2004. The Journal of Rheumatology
Publishing Company Limited. |
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ANTIPHOSPHOLIPID ANTIBODIES |
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by Dr. Ron Ascherson |
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ANTIPHOSPHOLIPID ANTIBODY SYNDROME |
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(APS) is a term used to describe the
association between recurrent clinical events such as
thrombosis (arterial or venous), thrombocytopenia, or fetal
loss and the presence of a persistent antiphospholipid
antibody 1-3. Other clinical conditions associated with the
syndrome include stroke, transient ischemic attack, livedo
reticularis, migraine, epilepsy, and heart valve disease4.
The syndrome is termed "primary" if there is no accompanying
autoimmune disease and "secondary" if the patient also has
systemic lupus erythematosus (SLE) or an autoimmune
disorder1,2,5. Certain infectious diseases and drugs may
also result in the formation of antiphospholipid antibodies
which do not appear to be associated with clinical
complications and do not require therapy1,3,5. |
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Antiphospholipid Antibody Syndrome |
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Indian Pediatrics 2001; 38: 1413-1416
Promotes an INR greater than 3. |
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Antiphospholipid Antibody Syndrome |
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Antiphospholipid syndrome (APS) is
characterized by the following: venous or arterial
thrombosis--a condition where clots, called thrombi, form in
the blood vessels; recurrent miscarriages--the repeated loss
of the fetus in pregnancies; and thrombocytopenia--a low
number of blood platelets that can lead to bleeding, seen as
bruising and tiny red dots on the skin. Medical College of
Wisconsin. |
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Antiphospholipid Antibody Syndrome |
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Intelihealth Website |
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Antiphospholipid Antibody Syndrome (APS) |
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Neuroland |
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Antiphospholipid Antibody Syndromes (APS) |
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from the Rare Thrombotic Diseases
Consortium |
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Antiphospholipid or Hughes' syndrome |
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Supports an INR of 3.0 to 4.0 for APS
patients. |
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Antiphospholipid Syndrome |
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*Summary Points Antiphospholipid syndrome
is characterized by the presence of venous and/or arterial
thrombosis and/or pregnancy morbidity and the presence of
antiphospholipid antibodies. Long-term anticoagulation is
recommended for antiphospholipid syndrome patients with
recurrent vascular events. For antiphospholipid syndrome
patients with recurrent pregnancy events, aspirin plus
heparin is recommended during pregnacy. Promotes an INR of
>3. |
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|
Antiphospholipid Syndrome |
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|
Antiphospholipid syndrome (APS) is
characterized by the following: venous or arterial
thrombosis--a condition where clots, called thrombi, form in
the blood vessels; recurrent miscarriages--the repeated loss
of the fetus in pregnancies; and thrombocytopenia--a low
number of blood platelets that can lead to bleeding, seen as
bruising and tiny red dots on the skin. |
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Antiphospholipid syndrome |
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|
The antiphospholipid syndrome (APS) is
characterized by venous and/or arterial thrombosis,
recurrent pregnancy loss and the presence of
antiphospholipid antibodies. The antiphospholipid antibodies
(anticardiolipin, anti-bêta2GPI antibodies, lupus
anticoagulant) interacting with various coagulation
proteins, platelets or endothelial cells may contribute to
disease pathogenesis. Incidence remains unknown, however the
reported prevalence of antiphospholipid antibodies in the
general population is low (1-4.5%) and increases with age.
The main clinical manifestations associated with APS are
thromboses, pregnancy morbidity, thrombocytopenia,
neurological symptoms, livedo reticularis, hemolytic anemia.
The antiphospholipid antibodies have been detected in
approximately 1/3 of the patients with systemic lupus
erythematosus (SLE). High anticardiolipin antibodies titers,
lupus anticoagulant and especially anti-bêta2GPI antibodies
are important predictors of APS clinical manifestations in
SLE patients. The management of thrombosis includes
long-term, high-intensity warfarin therapy [International
Normalized Ratio (INR superior or equal to 3)]. For
pregnancy morbidity the recommended therapy is low-dose
aspirin (80 mg/day) plus subcutaneous unfractionated heparin
or low-molecular-weight heparin. |
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Antiphospholipid Syndrome |
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Information provided by the UMHS
Hemophilia and Coagulation Disorders Program, February 2003 |
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Antiphospholipid Syndrome |
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Copyright © 1996-2005 C. Stephen Foster
M.D. All Rights Reserved. |
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Antiphospholipid Syndrome |
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Last Updated: August 10, 2007 |
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Antiphospholipid Syndrome |
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Last modified Monday, August 10, 2007 |
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Antiphospholipid Syndrome |
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Virtual Medical Centre. Modified:
14/7/2006 |
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Antiphospholipid syndrome |
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An. Bras. Dermatol. vol.80 no.3 Rio de
Janeiro May/June 2005. Antiphospholipid syndrome is an
acquired multisystem disorder characterized by recurrent
thromboses in the arterial system, venous system, or both.
Antiphospholipid syndrome is classified into 2 groups:
primary and secondary. Secondary antiphospholipid syndrome
is often associated with systemic lupus erythematosus and
less frequently with infections, drugs and other diseases.
Serologic markers are antiphospholipid antibodies, lupus
anticoagulant and anticardiolipin. The primary diagnostic
criteria include arterial thrombosis or venous thrombosis
and recurrent fetal loss. About 41% of patients with lupus
anticoagulant have skin lesions as the first sign of
antiphospholipid syndrome. Cutaneous manifestations include
livedo reticularis, cutaneous ulceration and livedo
vasculitis. The mainstays of prophylaxis and treatment of
thrombosis are anticoagulant and antiplatelet agents. |
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Antiphospholipid Syndrome - Making the Diagnosis |
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SJS - September 2003 Levine JS, Branch
DW, Rauch J. The antiphospholipid syndrome. N Engl J Med.
2002;346(10):752-63. |
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Antiphospholipid Syndrome from eMedicine |
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Last Updated: December 5, 2004 Author:
Steven Carsons, MD, Chief, Division of Rheumatology,
Allergy, and Immunology, Professor of Medicine, Department
of Internal Medicine, Winthrop University Hospital, State
University of New York at Stony Brook |
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Antiphospholipid syndrome: an overview |
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CMAJ • June 24, 2003; 168 (13) © 2003
Canadian Medical Association or its licensors |
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APS Article on Medicine Net |
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Medical Author: William C. Shiel Jr., MD,
FACP, FACR Last Editorial Review: 9/18/2005 |
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APS Foundation of South Africa |
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APSSA is a foundation formed to promote
much needed awareness of the Antiphospholipid Syndrome (APS)
in South Africa. |
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Euro-Phospholipid on Line |
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Official Web-site of the "European Forum
on Antiphospholipid Antibodies" |
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Hughes' Syndrome |
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In 1983 and during the following two
years, the Lupus Research Unit at St Thomas published a
number of papers showing that certain blood proteins
(antiphospholipid antibodies) were associated with a
syndrome of clotting (thrombosis), recurrent miscarriages
and brain disease. Between 1983 and 1985, a comprehensive
clinical/laboratory profile was presented showing, for the
first time, a wide spectrum of clinical features including
the association with artery thrombosis (including major
organs such as kidney and liver), brain disease (strokes and
other features), skin rashes, low platelet counts, epilepsy
and migraine. |
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Management of Antiphospholipid Antibody Syndrome |
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Lim W, Crowther MA, Eikelboom JW.
Management of antiphospholipid antibody syndrome. A
systematic review. JAMA 2006; 295:1050–1057 |
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Managing antiphospholipid syndrome |
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Copyright Springhouse Corporation Mar
2004 |
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Medical Progress- Antiphospholipid Antibody Syndrome |
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PDF File from the New England Journal of
Medicine |
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New Treatments For Lupus Anticoagulants |
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|
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NORD - National Organization for Rare Disorders, Inc. |
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Copyright 1994, 1995, 1996, 2001, 2002 |
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Official Web-site of the "European Forum on Antiphospholipid
Antibodies" |
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Old-fashioned detective work |
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August 20, 2005 |
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Pathogenesis of the Antiphospholipid Syndrome |
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SINDROME da ANTICORPI ANTIFOSFOLIPIDI |
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This page is in Italian. |
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The "primary" antiphospholipid syndrome: major clinical and
serological features. |
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Medicine (Baltimore). 1989
Nov;68(6):366-74. |
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The Antiphospholipid Story |
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© 2003. The Journal of Rheumatology
Publishing Company Limited. |
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The Antiphospholipid Syndrome |
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Most patients with venous or arterial
thrombosis and APS do well with conventional warfarin
treatment (target INR 2.0 - 3.0). It is recommended that
patients with recurrent thrombosis despite conventional
doses of warfarin should maintain an INR of 3.0 - 4.0. This
recommendation is based on one descriptive study and
requires confirmation by randomised trials. The benefit of
adding aspirin in arterial disease is not clear, and is
likely to increase the risk of bleeding. |
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The Antiphospholipid Syndrome |
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US Pharm. 2008;33(1):HS-23-HS-30.
Conclusion: APS is an autoimmune disorder that presents
unique challenges in diagnosis and treatment. The revised
diagnosis can be simply stated as the presence of one
clinical and one laboratory criteria. In practice, the
heterogeneous presentation of both clinical and laboratory
findings complicates diagnosis. This disorder should always
be included in differential diagnosis of persons who have
coagulation defects, evidence of vascular thrombosis, and/or
history of recurrent miscarriages or fetal losses. Positive
laboratory testing should always be confirmed at least 12
weeks apart to verify persistence. The heterogeneous
presentation of APS also makes treatment challenging, and,
in particular, research clarifying optimal therapy remains
lacking. Currently, the mainstay of treatment is
anticoagulation in those persons who develop an acute
thrombotic event. Although indefinite duration of
anticoagulation therapy is recommended, the decision to
administer long-term anticoagulation certainly requires
judicious clinical evaluation and risk assessment, given the
potential hemorrhagic complications of anticoagulation
therapy. |
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The antiphospholipid syndrome (Hughes' syndrome) |
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Last updated 01.08.2005 Written by Dr MY
Karim, lecturer in immunology, St Thomas' Hospital and Dr
GRV Hughes, consultant physician and rheumatologist, St
Thomas' Hospital |
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The antiphospholipid syndrome. |
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Natl Med J India. 2003
Nov-Dec;16(6):311-6. Promotes an INR of 3-4. |
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The expanding spectrum of renal diseases associated with
antiphospholipid syndrome. |
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Am J Kidney Dis. 2003 Jun;41(6):1205-11.
The cases reported here represent a new aspect of the
expanding spectrum of renal diseases encountered in
association with APS. |
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The Spectrum of the Antiphospholipid Syndrome: A Matter of
Perspective |
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© 2001. The Journal of Rheumatology
Publishing Company Limited. |
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Unusual Manifestations of the Antiphospholipid Syndrome |
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Reported by Ronald A. AshersonI
MD,FACP,MD(Hon)FRCP,FCP(SA)FACR,and Ricard Cervera2 MD,PhD |
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Warfarin in Antiphospholipid Syndrome — Time to Explore New
Horizons |
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The Journal of Rheumatology Feb. 2005 |
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Top of Page |
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APS - Cardiology & Pulmonology Related
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Antiphospholipid antibodies and hypertension. |
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Lupus. 2004;13(10):769-72. Hypertension
is a common manifestation of antiphospholipid syndrome
(APS). Antiphospholipid antibodies (aPL) have been described
in patients with hypertension secondary to renal artery
stenosis (RAS). Twenty-six patients with RAS and 25 patients
with severe essential hypertension (diastolic blood pressure
> 110 mmHg or > or = 3 hypertensive drugs) were studied and
compared to 61 age- and sex-matched healthy subjects. Serum
samples were tested for lupus anticoagulant (LA),
anticardiolipin (aCL) IgG and IgM, antiprothrombin (aPT) IgG
and IgM, anti-beta2glycoprotein 1 (abeta2GP1) IgG and IgM.
aPL were negative in all patients with RAS. Two patients
with essential hypertension had positive aPL (8%) (LA in one
patient confirmed in a second assay and abeta2GP1-IgG in the
other patient confirmed one year later together with aCL IgG
positivity). Among healthy subjects, one case (1.6%) was
found to be positive for LA, aCL IgM, abeta2GP1 IgM, aPT
IgG, aPT IgM. In conclusion, the association between RAS and
aPL seems to be casual rather than an expression of an
elective thrombotic localization ofAPS. The positive finding
of aPL in 8% of patients with essential hypertension, a
frequency higher than that of the control population,
deserves further studies in larger series to better explore
the relationship between aPL and hypertension. |
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Antiphospholipid Antibodies Tied to Mitral Regurgitation in
SLE |
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Arthritis Rheum 2006;54:3918-3925. |
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Antiphospholipid antibodies: a prognostic factor in
sarcoidosis? |
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CHEST, April, 1994. |
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Antiphospholipid antibody syndrome as the cause of clinical
rapidly progressing vasculopathy |
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Dtsch Med Wochenschr. 2000 May
12;125(19):589-93. |
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Antiphospholipid Syndrome and Perioperative Hemostatic
Management of Cardiac Valvular Surgery |
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Mayo Clin Proc. 2000;75:971-976 © 2000
Mayo Foundation for Medical Education and Research.
Hemostatic aspects of antiphospholipid syndrome (APS)
present unique challenges to clinicians and laboratory
personnel alike, particularly in the perioperative period.
These challenges are especially evident in patients
requiring cardiac valve replacement surgery. However, the
literature outlining the optimal approach in such patients
is limited. We present the case of a 25-year-old woman with
severe aortic regurgitation as a result of APS with
particular reference to the precautions necessary during
perioperative care. Particularly important are the
prevention of thrombotic or hemorrhagic complications,
management of associated thrombocytopenia, and laboratory
methods of perioperative anticoagulation monitoring in the
setting of prolonged clotting times. |
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Antiphospholipid syndrome and vascular disease |
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Cas Lek Cesk. 1999 Apr 26;138(9):276-9. |
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Antiphospholipid syndrome presenting as cardiac failure |
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Q J Med 2001; 94: 504-506 |
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Antiphospholipid syndrome with anti-prothrombin
autoantibodies in a patient with an axial-flow left
ventricular assist device. |
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J Heart Lung Transplant. 2005
Aug;24(8):1133-6. Autoantibodies to prothrombin, first
described almost 50 years ago, are paradoxically associated
with thrombosis. Described is an unusual case of fatal
hypercoagulability in a patient with multiple arterial and
venous thromboembolic complications despite intense
anticoagulation while being bridged to transplantation with
a left ventricular assist device. Serum analysis revealed
the presence of prothrombin autoantibodies and high titers
of anti-nuclear antibodies, and autopsy revealed pulmonary
arteriolar vasculitis. These findings suggest an autoimmune
basis for the presence of anti-prothrombin antibodies and
the hypercoagulable state observed in the present case. |
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Assessment of Cardiac Structure and Left Atrial Appendage
Functions in Primary Antiphospholipid Syndrome |
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Stroke. 2005;36:592. © 2005 American
Heart Association, Inc. Conclusions— It was concluded that
disease process in PAPS frequently involved cardiac valves
especially mitral valve but spared LAA function. LAA
function was normal, but intracardiac thrombus was present
in 5 patients and 1 of them was located in LAA. MR in PAPS
patients seems to impair LAA function. |
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Association of antibodies against phospholipids with heart
valve disease in systemic lupus erythematosus. |
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Lancet. 1990 Jun 30;335(8705):1541-4. |
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Cardiac dysfunction in SLE and antiphospholipid syndrome
patients |
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Ann Rheum Dis. Published Online First: 1
November 2006. doi:10.1136/ard.2005.044073 © 2006 by BMJ
Publishing Group Ltd & European League Against Rheumatism.
Conclusion: Abnormal echocardiographic findings were
detected frequently in asymptomatic patients with SLE or
PAPS. Although SLE patients were younger, LV systolic
function was more impaired in SLE compared to PAPS patients
while LV and RV diastolic function, as reflected by LVIVRT
and E/A ratios, were significantly more impaired in APS
patients. |
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Cardiac involvement in the antiphospholipid syndrome. |
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|
Lupus. 2005;14(9):691-6. Antiphospholipid
syndrome (APS) is a systemic autoimmune disease, associated
with a hypercoagulable state and fetal loss and with other
clinical manifestations including cardiac involvement.
Cardiac manifestations of APS are valve abnormalities (valve
thickening and vegetations), occlusive arterial disease
(atherosclerosis and myocardial infarction), intracardiac
emboli, ventricular dysfunction, and pulmonary hypertension.
Antiphospholipid antibodies (aPLs) may have a role in the
accelerated atherosclerotic arterial disease observed in
APS, related to their ability to induce endothelial
activation. aPLs have been incriminated in the pathogenesis
of heart valve lesions in APS patients. Markers of
endothelial cell activation are up-regulated with prominent
deposition of aPL in heart valves, suggesting aPL deposition
initiates an inflammatory process that recruits complement
leading to the valve lesion. Autoantibody-mediated
endothelial cell activation probably plays a role in
sustaining a proadhesive, proinflammatory, and procoagulant
phenotype. The heterogeneity of APS clinical manifestations
is likely linked to the varied effects that aPL can induce
on endothelial cells and to the different functions that
endothelial cells display depending on the anatomic
localization. |
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Cardiac manifestations of the antiphospholipid syndrome. |
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Am Heart J. 1992 Nov;124(5):1331-8. The
antiphospholipid syndrome has been associated with multiple
cardiac abnormalities. The earliest reports were of valvular
disease, including verrucous endocarditis, as well as
valvular thickening and insufficiency. Subsequently,
antiphospholipid antibodies were implicated in coronary
artery disease manifested by premature myocardial infarction
and coronary artery bypass graft occlusion. In addition,
there have been rare reports of intracardiac thrombi and
diffuse cardiomyopathy in association with antiphospholipid
antibodies. In this review, we discuss the nature and
prevalence of the cardiac manifestations of the
antiphospholipid antibody syndrome as well as some of the
proposed pathophysiologic mechanisms. We also provide
examples from our own experience. The expanding spectrum of
cardiac disease associated with antiphospholipid antibodies
suggests an important role for these antibodies in certain
types of cardiac pathology. |
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Cardiac Valvulopathy in the Antiphospholipid Syndrome |
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Source: Clinical Reviews in Allergy and
Immunology, Volume 23, Number 3, December 2002, pp.
341-348(8) Abstract: The Libman and Sacks non bacterial
endocarditis was reported in 1924 in patients with SLE. Its
relation to the anti cardiolpin syndrome has only been
described as recently as the last decade. In this paper we
review the deposition of theses antibodies on the valve with
complement components initiating the deformation of the
valve. The valvulopathy in APS is quite common and may lead
to valve replacement. In addition, a diversity of
manifestations are detailed. More awareness should be drawn
to this new complication of APS. |
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Cardiovascular Pathology - Libman-Sacks endocarditis |
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Here are flat, pale tan, spreading
vegetations over the mitral valve surface and even on the
chordae tendineae. This patient has systemic lupus
erythematosus. Thus, these vegetations that can be on any
valve or even on endocardial surfaces are consistent with
Libman-Sacks endocarditis. These vegetations appear in about
4% of SLE patients and rarely cause problems because they
are not large and rarely embolize. Note also the thickened,
shortened, and fused chordae tendineae that represent remote
rheumatic heart disease. |
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Classification Criteria for Antiphospholipid Syndrome: The
Case for Cardiac Valvular Disease |
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|
© 2004. The Journal of Rheumatology
Publishing Company Limited. |
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|
Clinical study of anti-phospholipid antibody in patients
with sarcoidosis |
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|
Nihon Kyobu Shikkan Gakkai Zasshi. 1994
Jan;32(1):3-8. |
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|
Heart Valve Involvement (Libman-Sacks Endocarditis) in the
Antiphospholipid Syndrome |
|
|
Circulation. 1996;93:1579-1587. © 1996
American Heart Association, Inc. The antiphospholipid
syndrome (APS) is defined by the presence of anti-phospholipid
antibodies (aPLs) and venous or arterial thrombosis,
recurrent pregnancy loss, or thrombocytopenia. The syndrome
can be either primary or secondary to an underlying
condition, most commonly systemic lupus erythematosus (SLE).
Echocardiographic studies have disclosed heart valve
abnormalities in about a third of patients with primary APS.
SLE patients with aPLs have a higher prevalence of valvular
involvement than those without these antibodies. Valvular
lesions associated with aPLs occur as valve masses
(nonbacterial vegetations) or thickening. These two
morphological alterations can be combined and are thought to
reflect the same pathological process. Both can be
associated with valve dysfunction, although such association
is much more common with the latter alteration. The
predominant functional abnormality is regurgitation;
stenosis is rare. The mitral valve is mainly affected,
followed by the aortic valve. Valvular involvement usually
does not cause clinical valvular heart disease. The presence
of aPLs seems to further increase the risk for
thromboembolic complications, mainly cerebrovascular, posed
by valve lesions. Superadded bacterial endocarditis is rare
but may be difficult to distinguish from pseudoinfective
endocarditis. The current therapeutic guidelines are those
for APS in general. Secondary antithrombotic prevention with
long-term, high-intensity oral anticoagulation is advised.
The efficacy of aspirin, either alone or in combination, is
yet to be assessed. Corticosteroids are not beneficial and
may even facilitate valve damage. Immunosuppressive agents
should only be used for the treatment of an underlying
condition. Current data suggest a role for aPLs in the
pathogenesis of valvular lesions. aPLs may promote the
formation of valve thrombi. These antibodies may also act by
another mechanism, as indicated by the finding of
subendothelial deposits of immunoglobulins, including anti-cardiolipin
antibodies, and of colocalized complement components in
deformed valves from patients with APS. |
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Heart Valve Involvement (Libman-Sacks Endocarditis) in the
Antiphospholipid Syndrome |
|
|
(Circulation. 1996;93:1579-1587.) © 1996
American Heart Association, Inc. Conclusion: Data provided
by clinical and immunopathological studies support an
association between aPLs and heart valve lesions. They also
imply that aPLs play a pathogenetic role in endocardial
damage. Basic and randomized, prospective, controlled
clinical studies are needed to further define the role of
aPLs in cardiac valve disease, elucidate its natural
history, and establish optimal treatment and prevention of
the disease and its potential clinical sequelae. |
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Hypertension as the presenting feature of the
antiphospholipid syndrome. |
|
|
Lupus. 2002;11(4):253-6. The
antiphospholipid or Hughes syndrome is the association
between antiphospholipid antibodies (aPL), venous and
arterial thromboses and pregnancy morbidity.
Antiphospholipid syndrome (APS) commonly coexists with
autoimmune diseases usually systemic lupus erythematosus (SLE),
when it is known as secondary APS. When present in isolation
it is known as primary APS (PAPS). Although the kidney may
be affected in APS, its involvement is perhaps not as well
described as that of other organs. Thrombotic
microangiopathy (TMA) affecting the kidney has been reported
as a manifestation in both primary and secondary APS. This
report describes hypertension related to underlying renal
TMA as a presenting symptom of APS. |
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|
Libman-Sacks
endocarditis and cerebral embolization in antiphospholipid
syndrome. |
|
|
Eur J Echocardiogr. 2008 Jan;9(1):192-3.
In antiphospholipid syndrome (APS), there is a high
prevalence of valvular heart disease which leads to
increased risk of thrombo-embolic events, in particular,
cerebrovascular events. We present a patient with cerebral
infarction, previous deep-vein thrombosis, and miscarriages
with positive lupus anticoagulant and anticardiolipin
antibodies. Echocardiographic examination revealed mitral
valve leaflet thickening and verrucous vegetations
consistent with Libman-Sacks endocarditis, which is commonly
associated with APS. In patients with combined Libman-Sacks
endocarditis and antiphospholipid antibodies,
anticoagulation therapy with warfarin is indicated due to
high risk of valvular thrombus formation and subsequent
embolization. |
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|
Libman-sacks endocarditis and primary antiphospholipid
syndrome. |
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J Heart Valve Dis. 2005 Sep;14(5):700-2.
Cardiac involvement is a not uncommon complication in
patients with antiphospholipid syndrome (APS). Herein, the
case is reported of cardiac failure in a female patient with
Libman-Sacks endocarditis and with primary APS diagnosed
eight years previously. Aggressive anticoagulation therapy
and medical treatment for the cardiac failure over a
12-month period resulted in a partial regression of the
severe mitral regurgitation. Close clinical and
echocardiographic surveillance during the follow up of
patients with APS and heart valve disease is mandatory.
Optimal treatment, including adequate aggressive
anticoagulation therapy and specific treatment for heart
failure, may play a pivotal role in reducing the severity of
valve dysfunction in these patients. |
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LIBMAN-SACKS ENDOCARDITIS IN A PREGNANT WOMAN WITH ACUTE
RESPIRATORY DISTRESS SYNDROME |
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Obstetrics & Gynecology 1999;93:819-821 ©
1999 by The American College of Obstetricians and
Gynecologists. Conclusion: With rapid decompensation of
acute respiratory distress syndrome in pregnancy, despite
aggressive medical therapy, complicating processes must be
considered, especially with antiphospholipid antibodies,
which can be associated with sterile heart vegetations and
subsequent fatal thromboembolism. |
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Myocardial infarction caused by cardiac microvasculopathy in
a patient with the primary antiphospholipid syndrome. |
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Ann Intern Med. 1992 Jun 15;116(12 Pt
1):974-6. Antiphospholipid antibodies occur in various
clinical states, including the primary antiphospholipid
syndrome. Clinical features in these conditions appear to be
caused by vasculopathy associated with the presence of these
antibodies. We report the case of a patient with primary
antiphospholipid syndrome who experienced cardiac necrosis
secondary to myocardial microvasculopathy in the absence of
vasculitis. This case demonstrates unequivocally that
noninflammatory myocardial microvasculopathy occurs in the
primary antiphospholipid syndrome per se without any
clinical or immunologic signs of systemic lupus
erythematosus or other disease process. The histopathologic
findings in the skin and myocardial biopsies showed a
noninflammatory vasculopathy characterized by bland thrombi
and lack of infiltration of the vessel wall by inflammatory
cells. Ultrastructural examination of the myocardial biopsy
confirmed the vascular thrombosis and endothelial activation
and showed no deposits in basement membranes. The patient
survived after appropriate treatment. Evidence presented
here supports the concept that the vasculopathy in the
antiphospholipid syndrome is distinct from other types of
vascular occlusions seen in systemic lupus erythematosus. We
suggest that myocardial biopsy can be crucial in showing an
underlying myocardial ischemic process despite "normal"
findings on coronary angiography. Results of the biopsy
hastened the decision to use potentially lifesaving
plasmapheresis and anticoagulation therapy in this patient. |
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Overt congestive heart failure with mitral and aortic
regurgitation due to antiphospholipid syndrome in a patient
with systemic lupus erythematosus |
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A 51-year-old woman with overt congestive
heart failure with pleural and pericardial effusion was
treated with furosemide and nifedipine, leading to
improvement in her condition and a decrease in effusions. An
echocardiography demonstrated mitral and aortic
regurgitation with mitral valve prolapse, which caused the
congestive heart failure. Since leukocytopenia and
lymphocytopenia with arthralgia could be observed,
serological investigations were performed. She was diagnosed
as having systemic lupus erythematosus (SLE) with
antiphospholipid syndrome, and started on a treatment of
prednisolone and aspirin. Based on the treatment, the
pleural and pericardial effusion went into complete
remission, indicating that the serositis related to SLE had
overlapped the heart failure. Since there was no evidence of
any other diseases that could be responsible for the
valvular lesions, we concluded that they were due to
antiphospholipid syndrome. The administration of
prednisolone had no significant effect on valvular
morphology or function as demonstrated by echocardiography.
When patients with valvular disease are seen, a valvulopathy
related to antiphospholipid syndrome should be considered as
part of the differential diagnosis. |
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Peripheral vascular disease in antiphospholipid syndrome. |
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Thromb Res. 2004;114(5-6):509-19.
Atherosclerosis has been considered an inflammatory disease
based on the finding that atherosclerotic lesion contains
activated T lymphocytes reacting with oxidized low-density
lipoproteins (oxLDL) and heat shock proteins (HSP); it also
contains autoantigens like beta2GPI, a target of antibodies
occurring in an immune-mediated thrombophilia called
antiphospholipid syndrome (APS). Further support to this
hypothesis comes from the cross-reactivity, which occurs
between antiphospholipid antibodies (aPL) and antibodies to
oxLDL. Animal experiments have shown that aPL are associated
with atheroma. In addition, accelerated atherosclerosis has
been detected in patients with a prototype systemic
autoimmune disease, such as systemic lupus erythematosus
(SLE). However, the association of APS or aPL with
atherosclerosis is a matter of debate due to the small
numbers of patients studied, and the fact that traditional
risk factors for atherosclerosis coexist. The prevalence of
APS ranges from 1.7% to 6%, and that of aPL reaches to 14%
among patients with peripheral vascular disease defined on
the basis of clinical outcomes. On the other hand, the
prevalence of asymptomatic atherosclerosis, defined in terms
of plaques in ultrasonography, reaches to 15% of patients
with APS compared to 9% of SLE patients and 3% of normal
controls. Among SLE patients with aPL, the prevalence of
plaques ranges from 6% in premenopausal women to 31% in
unselected patients. Less than 10% of APS patients express
premature atherosclerosis in the absence of other risk
factors. Which APS patient will develop atherosclerosis is
unpredictable. |
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Prevalence of antiphospholipid antibodies in systemic
sclerosis and association with primitive pulmonary arterial
hypertension and endothelial injury. |
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Clin Exp Rheumatol. 2005
Mar-Apr;23(2):199-204. CONCLUSION: We found that the
prevalence of antiphospholipid antibodies in SSc patients
was low. However, aCL antibodies were associated with PAH
and endothelial injury. |
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Severe valvular regurgitation and antiphospholipid
antibodies in systemic lupus erythematosus: A prospective,
long-term, followup study |
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Arthritis Rheum. 2005 Jun 2;53(3):460-467
CONCLUSION: In SLE patients, the presence of high levels of
IgG anticardiolipin antibodies is associated with the
development of severe valvular regurgitation and with a high
incidence of thromboembolic events and the need for valvular
surgery. |
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Severe, non-infectious mitral valve endocarditis after
mitral valve reconstruction in a 32-year old female with
primary antiphospholipid syndrome |
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Z Kardiol. 2004 Jul;93(7):546-54.
CONCLUSION: Secondary cardiac valve operations on patients
with primary antiphospholipid syndrome may be successfully
performed within a multidisciplinary approach. Oral
anticoagulation remains the treatment of choice to prevent
further thromboembolic events. |
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Sticky Blood May Underlie Early Atherosclerosis In Men |
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DALLAS, TX -- April 20, 1998 |
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The Intrarenal Vascular Lesions Associated with Primary
Antiphospholipid Syndrome |
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J Am Soc Nephrol 10:507-518, 1999 © 1999
American Society of Nephrology |
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Top of Page |
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APS - Catastrophic Antiphospholipid Syndrome
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An acute multiorgan thrombotic disorder associated with
antiphospholipid antibodies; two `catastrophic' cases. |
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Ann Rheum Dis 1997;56:568-570 ( September
). Over the past decade the antiphospholipid syndrome (APS)
was defined by the presence of antiphospholipid antibodies
(aPL) and clinical manifestations including thrombosis,
recurrent fetal loss, thrombocytopenia, chorea, livedo
reticularis, heart valve lesions, and renal involvement.1
Asherson et al first drew attention to a catastrophic
variant of APS (CAPS) that is characterised by multiple
widespread vascular occlusions, leading to multiple organ
failure and often death.2 We describe two non-systemic lupus
erythematosus (SLE) patients with a strikingly similar
clinical presentation of CAPS and emphasise the difficulties
in differentiating CAPS from other thrombotic angiopathies. |
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CAPS REGISTRY |
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Last updated: 18 September 2004 |
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Catastrophic antiphospholipid antibody syndrome |
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Pediatric Nephrology, Volume 20, Number 7
/ July, 2005, Pages 998-999. Antiphospholipid antibody
syndrome (APS) is characterized by recurrent thrombosis with
the presence of circulating antiphospholipid antibodies. A
diagnosis of APS requires the presence of at least one
clinical and one laboratory criteria (detection of aCL IgG
or IgM antibodies or the presence of lupus anticoagulant on
two or more consecutive occasions 6 weeks apart). A severe,
rapidly progressive form characterized by clinical
involvement of at least three different organ systems with
histopathological evidence of small and large vessel
occlusion is termed catastrophic antiphospholipid syndrome.
Early recognition of APS is crucial since aggressive
management can result in a favorable outcome. |
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Catastrophic Antiphospholipid Syndrome (CAPS) |
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Juan Javier Lichauco, M.D., Jayashree
Sinha, M.D.,, and Peter Barland, M.D. February 2002 |
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Catastrophic antiphospholipid syndrome in a 14-year-old
child. |
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Pediatr Nephrol. 2005 Apr;20(4):519-21.
Epub 2005 Feb 17. Antiphospholipid syndrome (APS) is an
autoimmune disease. Less than 1% of patients with APS
present with life-threatening catastrophic APS (CAPS). We
report here a case of CAPS in a young girl with cardiac,
gastrointestinal and renal involvement. Although the
management was complicated, the outcome was better than
expected. We suggest that CAPS be included in the
differential diagnosis of acute renal failure in children
with multi-organ involvement and prolonged
phospholipid-dependent coagulation time and promptly treated
with immunomodulating agents and anticoagulants. |
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Catastrophic antiphospholipid syndrome in cancer. |
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Haematologia (Budap). 2000; 30(4):303-11.
Antiphospholipid syndrome is characterized by the presence
of antiphospholipid antibodies resulting in arterial and
venous thromboembolism. Apart from primary cases, this
syndrome is often associated with autoimmune diseases.
Around 50 cases of catastrophic antiphospholipid antibody
syndrome have been reported as yet. Authors describe the
first case of catastrophic antiphospholipid syndrome
associated with gastric cancer. Apart from presenting the
clinical case, authors also discuss the possible
pathomechanism of this associated disorder including the
role of immunological factors, as well as antiphospholipid
antibodies. |
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Catastrophic antiphospholipid syndrome presenting as dilated
cardiomyopathy with bilateral branch retinal artery
thrombosis. |
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Int J Clin Pract. 2000 Oct;54(8):550-1.
Cardiac manifestation of antiphospholipid syndrome (APS) is
mainly in the form of left-sided valvular insufficiency,
intra-cardiac thrombi or coronary artery occlusion. Dilated
cardiomyopathy is a rare but important cardiac manifestation
of APS, and responds well to adequate anticoagulation and
steroids. We describe a case in which APS presented with
dilated cardiomyopathy and bilateral retinal artery
thrombosis. |
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Catastrophic antiphospholipid syndrome: A rare cause of
disseminated microvascular thrombotic injury – a case report
with pathological and molecular correlative studies |
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Pathology International, Volume 55 Issue
3 Page 144 - March 2005. Catastrophic antiphospholipid
syndrome (CAPS) is a severe and rare variant of
antiphospholipid syndrome (APS) characterized by acute
multiorgan failure due to small vessel thrombi in patients
with positive antiphospholipid antibodies. We report a fatal
case of catastrophic antiphospholipid syndrome in a young
woman with a history of polymyositis and Hodgkin lymphoma.
The patient was admitted to hospital because of severe foot
pain following several weeks of skin ulcerations. Doppler
ultrasonography showed evidence of arterial ischemia of the
both lower extremities. Despite anticoagulation,
immunosuppression, plasmapheresis and antibiotic therapy,
she developed cutaneous gangrene, retroperitoneal hematoma,
ileus, and acute respiratory and renal failure that resulted
in death. Autopsy showed multifocal vascular injury and
microthrombi with associated hemorrhages and infarcts in
multiple organs. The patient had normal levels of functional
protein C and protein S and a normal level of plasma
homocysteine. Tests for common thromophilic gene mutations
including prothrombin 20210, factor V Leiden 1691, and
methylene tetrahydrofolate reductase 677 were negative. To
our knowledge, this is the first CAPS patient with molecular
studies for genetic prothrombotic mutations. Our report
showed that there was no association between the development
of CAPS and inherited thromophilia. |
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Catastrophic secondary antiphospholipid syndrome with
peripheral nervous system involvement: a case report. |
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Acta Med Okayama. 2004 Apr;58(2):107-10.
A 34-year-old woman was admitted to our emergency room with
a high fever, abdominal pain, dyspnea and confusion. High
fever and abdominal pain had first occured after a cystocele
operation 5 months earlier. Later, congestive heart failure
with mural thrombus formation, peripheral polyneuropathy and
ischemic cerebrovascular accident were identified in
clinical follow-ups, and multiple arterial and venous
thromboses were seen on cranial and abdominal magnetic
resonance imaging angiography. The patient's symptoms
improved with anticoagulant treatment. Antiphospholipid
syndrome with elevated serum anticardiolipin IgG levels was
diagnosed, and ischemic peripheral polyneuropathy with
axonal degeneration was determined by sural nerve biopsy. In
antiphospholipid syndrome, elevated anticardiolipin
antibodies appear to be the most common acquired blood
protein defect causing thrombosis. Disseminated vascular
thrombosis in catastrophic antiphospholipid syndrome can
result in multiorgan failure with increased morbidity and
mortality. It rarely occurs secondary to various infections
as in the case of our patient, who suffered postoperative
intraabdominal infection. It is important to note that
peripheral nervous system involvement is rare in
antiphospholipid syndrome. |
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Imaging findings in the rare catastrophic variant of the
primary antiphospholipid syndrome |
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European Radiology, Volume 12, Number 3 /
March, 2002, Pages 545-548. We report imaging findings in a
case of the rare catastrophic variant of antiphospholipid
syndrome (CAPS) characterized by widespread microvascular
occlusions, which may lead to multiple organ failure. We
present a case of a 66-year-old woman with bone marrow
necrosis, acute acalculous cholecystitis (AAC), focal liver
necrosis, subtle patchy splenic infarctions, and bilateral
adrenal infarction. The demonstration of multiple
microvascular organ involvement (three or more) is crucial
for the diagnosis of the catastrophic variant of APS. This
can be performed radiologically intra-vitam. Imaging can
even reveal subclinical microinfarctions, which are often
only diagnosed at autopsy. |
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Laboratory studies on pathophysiology of the catastrophic
antiphospholipid syndrome. |
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Autoimmun Rev, December 1, 2006; 6(2):
68-71. The 'catastrophic' variant of the antiphospholipid
syndrome (APS) is characterized by a diffuse thrombotic
microvasculopathy. In contrast to the classical APS, single
venous or arterial medium-to-large blood vessel occlusions
are uncommon. The mechanisms of catastrophic APS are not
clearly understood. In addition, there are no studies on
pathophysiologic mechanisms of catastrophic APS. The
clinical manifestations of catastrophic APS probably depend
on (a) the organs affected by the thrombotic events and
extent of the thrombosis and (b) manifestations of the
systemic inflammatory response syndrome which are presumed
to be due to excessive cytokine release from affected and
necrotic tissues. The evident relationship between APS and
infection may enable us to explain the development of
catastrophic APS using the sepsis model. This is because
catastrophic APS is characterized by multiple microvascular
thrombotic events, of rapid onset, and causing multiorgan
failure, a picture suggestive of septic shock, in which,
there is a massive, acute inflammatory response. |
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Long term outcome of catastrophic antiphospholipid syndrome
survivors |
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Annals of the Rheumatic Diseases
2003;62:530-533. Conclusion: Sixty six per cent of patients
who survive an initial catastrophic APS event remained
symptom free with anticoagulation during an average follow
up of 67.2 months. Twenty six per cent of the survivors
developed further APS related events and the mortality rate
of these patients was about 25%. |
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Registry Improves Understanding of Catastrophic
Antiphospholipid Syndrome |
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(2006) 2, 81-89 - Nature Clinical
Practice Rheumatology. The most important clinical feature
of CAPS to recognize is that, despite the high risk early on
of this potentially devastating syndrome, the long-term
prognosis of those patients who do survive the initial onset
appears to be excellent. |
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Relapsing Catastrophic Antiphospholipid Syndrome: Report of
Three Cases |
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doi:10.1016/j.semarthrit.2007.08.001
Conclusions: Relapses occur very infrequently in patients
with the CAPS. The presence of MHA is common in these
patients, suggesting that an association between MHA and
relapses of CAPS could be present and that a “continuum”
between various MHAs might exist, as recently suggested. |
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Top of Page |
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APS - Hematology & Rheumatology Related
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A cross-sectional study of clinical thrombotic risk factors
and preventive treatments in antiphospholipid syndrome |
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Rheumatology 2002; 41: 924-929 © 2002
British Society for Rheumatology. Conclusion. While
traditional risk factors were similar between groups,
pregnancy and surgical procedures increased the risk of
thrombosis. Hypertension and smoking were associated with
arterial events. Possessing a combination of risk factors
may increase the occurrence of arterial thrombosis but not
venous thrombosis. Use of aspirin and/or hydroxychloroquine
may be protective against thrombosis in asymptomatic
aPL-positive individuals. |
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Acquired Thrombophilia Antiphospholipid Antibody Syndrome
Category 2 Immune Problems |
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Acquired thrombophilia, antiphospholipid
antibodies (APAs) and the antiphospholipid antibody syndrome
(APS) are autoimmune conditions characterized by the
presence of certain clinical features and levels of
circulating APAs. |
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Annexin and APS: the clot thickens |
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Blood, 1 June 2006, Vol. 107, No. 11, pp.
4195-4196. |
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Antibody Associated with Autoimmune Disorders Found to
"Cluster" in Families |
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Finding May be First Step in Preventing
Premature Stroke, Heart Attack and Miscarriage |
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Antibody Resource Page |
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Anticardiolipin Antibodies (ACA), IgA, IgG, IgM |
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Anticardiolipin Antibodies (ACA): Antigenic Targets and
Pathophysiology |
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Anticardiolipin Test and the Antiphospholipid (Hughes)
Syndrome: 20 Years and Counting! |
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© 2004. The Journal of Rheumatology
Publishing Company Limited. |
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Antinuclear Antibody |
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Although access to this page is not
restricted, the information found here is intended for use
by medical providers. Patients should address specific
medical concerns with their physicians. |
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Antiphospholipid antibodies and venous thromboembolism |
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Volume 86, Issue 10, pp. 3685-3691,
11/15/1995 Copyright © 1995 by The American Society of
Hematology |
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Antiphospholipid antibodies by Dr. Stephan Moll (Q/A) |
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Q1: "My hemo just ran five new tests on
me, looking for another cause of clotting. It looks as if
the beta-2-glycoprotein test is an indicator of APS (=
antiphospholipid antibody syndrome). My IgG and IgA are both
normal, but my IgM is 34 (should be |
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Antiphospholipid antibodies in alcoholic liver disease are
influenced by histological damage but not by alcohol
consumption. |
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Lupus. 2000;9(6):451-5. |
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Antiphospholipid Antibodies Not Predictive of Future
Thrombo-Occlusive Events After Ischaemic Stroke |
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JAMA 2004;291:576-584. |
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Antiphospholipid antibody syndrome and polymyalgia
rheumatica/giant cell arteritis |
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Rheumatology 2000; 39: 565-567 © 2000
British Society for Rheumatology |
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Antiphospholipid Antibody Syndrome. |
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Curr Treat Options Cardiovasc Med. 2003
Apr;5(2):127-136. Antiphospholipid antibody syndrome (APS)
is a recently defined autoimmune disorder characterized by
recurrent vascular thromboses or recurrent pregnancy
morbidity; these features are linked to the presence in
blood of autoantibodies against negatively charged
phospholipids or phospholipid-binding proteins. Thrombosis
can occur in any tissue, in veins, arteries, or the
microvasculature. Pregnancy morbidity in APS includes
miscarriages or premature birth. Criteria that define the
major clinical and laboratory features of APS were published
in 1999. In patients with antiphospholipid antibodies and
prior thrombosis or pregnancy morbidity, there is a high
risk of recurrence that persists as long as antiphospholipid
antibodies occur in blood. This risk for recurrence of
thrombosis or pregnancy morbidity is greatly reduced by
preventive anticoagulant therapy. Patients presenting with
thrombosis in APS are initially managed in much the same way
as are patients with vascular thrombosis owing to other
causes. However, in patients with APS, high-intensity
anticoagulation is usually needed to prevent recurrences of
thrombosis. Thrombosis in APS is often multifactorial, as
with non-APS thrombosis. Therefore, in all patients with
APS, other reversible risk factors for thrombosis should be
sought. The pregnancy outcome of women with APS who have had
prior miscarriages is greatly improved by treatment during
pregnancy with a combination of heparin and low-dose
aspirin. |
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Antiphospholipid Antibody Syndrome: Lupus Anticoagulants and
Anticardiolipin Antibodies |
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Antiphospholipid antibody testing: which are most useful for
diagnosis? |
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Rheum Dis Clin North Am. 2006
Aug;32(3):455-63. Laboratory diagnosis of APS relies on the
demonstration of a positive test for aPL. In clinical
practice, the gold standard tests are those that detect
beta2GPI-dependent aCL or LA. The question on the use of
anti-beta2GPI asa routine diagnostic test remains
unanswered, and testing for these antibodies should be only
performed in very selected cases and not as an alternative
to aCL or LA testing. Clinical utility and standardization
are still lacking for other aPL specificities; therefore,
their application as routine diagnostic tools is not
recommended. |
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Antiphospholipid syndrome and recurrent thrombosis in
children. |
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Arthritis Rheum, November 30, 2006;
55(6): 850-855. CONCLUSION: APS in children has unique
features. SLE may develop in a significant percentage of
girls presenting with APS. Hereditary thrombophilia did not
predict recurrent thrombosis, whereas the preventive impact
of anticoagulant treatment following the first thrombotic
event was noteworthy. |
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Antiphospholipid Syndrome and Vascular Ischemic (Occlusive)
Diseases: An Overview |
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Yonsei Med J 48(6):901 - 926, 2007. DOI
10.3349/ymj.2007.48.6.901. CONCLUSION The nature of APS, as
well as the evolving symptomatology of SLE, contributed to
the recognition that anticoagulating approaches, rather than
steroids or immunosuppressive drugs, significantly improved
the outcome in a substantial number of patients with APS. We
may also conclude that CNS disease in SLE is significantly
associated with aPLs. Cerebral ischaemia due to vessel
occlusion is considered the most important cause of CNS
diseases in SLE, and aPLs play an important role in their
pathogenesis. There is a strong association between aPLs and
CVD, headache, cognitive dysfunction, and seizures, thus
supporting the importance of occlusive vasculopathies in
neuro-psychiatric lupus. Hence, testing for aPLs should be
recommended not only for patients with autoimmune diseases
and neuropsychiatric syndromes but also for younger patients
(age < 40 years) without an underlying autoimmune disease
who develop ischemic cerebral events and may have
‘non-classic’ MS, transverse myelitis, or atypical seizures.
During brain MRI, such young individuals with multiple
hyperintensity lesions without other known causes should
also undergo testing for aPLs. All patients with a target
INR > 3.0 and cerebral ischaemia should undergo
anticoagulation therapy to prevent recurrences. All patients
with thrombosis associated with aPLs should undergo
long-term (life-long) warfarin therapy. Unfortunately,
low-dose aspirin alone does not prevent recurrent
thrombosis. Additionally, oral anticoagulation carries an
increased risk of serious haemorrhage; however, this risk,
if well controlled, might be lowered to acceptable levels.
Steroids and immunosuppressive drugs in aPLpositive patients
and thrombosis may be justified only in life-threatening
situations when episodes of thrombosis occur despite
adequate anticoagulation treatment. In aPL-positive patients
without previous thrombosis, low-dose aspirin (75 mg/day)
indefinitely as a thromboprophylactic measure is
recommended. In neuropsychiatric appearances different from
CVD (e.g., headache, seizures), anticoagulation therapy
should be considered for patients with more severe disease
and unsatisfactory responses to traditional treatments for
headache or conventional anti-epileptic drugs. |
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Antiphospholipid Syndrome from Pediatrics/Rheumatology |
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Authored by Barry L Myones, MD, Director
of Research, Pediatric Rheumatology Center, Texas Children's
Hospital at Houston; Associate Professor, Departments of
Pediatrics & Immunology, Pediatric Rheumatology Section,
Baylor College of Medicine Adjust dose to maintain an INR in
the range of 2.5-3.5. |
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Antiphospholipid syndrome in patients with systemic lupus
erythematosus treated by autologous hematopoietic stem cell
transplantation. |
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Blood. 2005 Oct 15;106(8):2700-9. Epub
2005 May 3. |
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Antiphospholipid syndrome with only
antiphosphatidylethanolamine antibodies: report of 20 cases |
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Rev Med Interne. 2002 Apr;23(4):357-63.
PURPOSE: The association of antiphosphatidylethanolamine
antibodies (aPE) as the only antiphospholipid antibody with
antiphospholipid syndrome (APS) is discussed. The aPE was
described as the sole antibody in many cases suggesting APS.
aPE was not included in the Sapporo criteria for the
classification of APS. METHODS: We investigated the clinical
features of 20 patients with aPE only; 17 patients had
symptoms potentially related to APS (group 1) and three had
other manifestations (group 2). RESULTS: There were 15 women
and five men, mean age was 35 +/- 12 years at the beginning.
In group 1 (n = 17), ten patients presented arterial
thrombosis, nine venous thrombosis (five had both), and six
microvascular thrombosis (livedo reticularis, lacunar
pathology). The aPE positivity was persistent in 13
patients. A subgroup of four patients (three women)
presented arteriosclerosis with peripheral arteriopathy
which started before 45 years of age. They had another
atherosclerosis risk factor associated with aPE persistence.
In group 2 (n = 3), there was no thrombotic event, one
demyelinating pathology, one microvascular pathology, and
one arterial dysplasia. The aPE positivity was never
confirmed. Finally, 13 patients presented an APS with aPE
only, confirmed at least 8 weeks later. CONCLUSIONS: Our
study points out that testing for aPE would be of interest
for patients when symptoms were potentially related to APS,
particularly when other antiphospholipid antibodies were
negative. This description questions the enlargement of the
APS biological criteria defined in Sapporo. The role of aPE
in atherosclerosis is considered. |
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Antiphospholipid syndrome without antiphospholipid
antibodies at the time of the thrombotic event: transient
'seronegative' antiphospholipid syndrome? |
|
|
Clin Exp Rheumatol. 1997
Sep-Oct;15(5):541-4. The antiphospholipid syndrome (APS) is
characterized by the presence of venous and arterial
thrombosis, recurrent fetal losses and thrombocytopenia,
associated with the presence of antiphospholipid antibodies
(aPL). This syndrome may be "primary" or may be associated
with other diseases, mainly systemic lupus erythematosus
(SLE). However, some patients present the clinical picture
of this syndrome but without evidence of aPL in their serum.
The term "seronegative" APS has been proposed to categorize
these patients. Here with we present two patients with
seronegativity for aPL at the time of a thrombotic event,
but in whom these antibodies were detected 2 and 7 months
later. |
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Antiphospholipid Syndrome: A Coagulation Disorder in Women |
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|
Posted 01/24/1997 Antiphospholipid (aPL)
syndrome, or APS,--a cluster of conditions that includes
arterial or venous thromboses and thrombocytopenia, as well
as recurrent fetal loss associated with elevation of aPL
antibody--has been reported to occur 2-5 times more
frequently in women than men. Strong familial associations
lead to the suspicion that aPL positivity, estimated to be
present in 2% of the population, is a heritable trait in
some cases. Currently, 2 major categories of the illness are
recognized--primary and secondary. Secondary APS may be
associated with autoimmune disease, malignancy, infectious
disease, or drug-induced states. Two assays, one for lupus
anticoagulant antibodies and the other for anticardiolipin
(aCL) antibodies, are recognized to be the gold standards
for serologic diagnosis of the disease. Despite extensive
attempts at international standardization of aCL test
results, no consensus exists for a value beyond which the
test is considered positive. Interestingly, a "dose-effect"
relationship for aCL antibody titers has been noted--higher
titers of the antibody correlate with increased numbers of
thrombotic events. An experimental assay for antibody
against beta 2-glycoprotein 1 (beta-2-GP1), a
phospholipid-binding protein, may become the most important
assay for aPL. Skin findings in APS include livedo
reticularis, ulceration, gangrene, or purpura, and, when
present, may be the key to diagnosis of this sometimes
insidious syndrome. Anticoagulation, usually with warfarin,
is the mainstay of therapy, although steroids,
immunosuppressive agents, hydroxychloroquine sulfate, and
plasmapheresis may all be beneficial adjunctive therapy. |
| |
|
Antiphospholipid syndrome: Clinical and immunologic
manifestations and patterns of disease expression in a
cohort of 1,000 patients |
|
|
APS may affect any organ of the body and
display a broad spectrum of manifestations. An association
with SLE, the patient's sex, and the patient's age at
disease onset can modify the disease expression and define
specific subsets of APS. |
| |
|
Antiphospholipid thrombosis: clinical course after the first
thrombotic event in 70 patients. |
|
|
Ann Intern Med. 1992 Aug 15;117(4):303-8.
CONCLUSIONS: Recurrent thrombosis is a potentially serious
problem for patients with lupus anticoagulant or
anticardiolipin antibodies or both. The site of the first
event (arterial or venous) tended to predict the site of
subsequent events. Intermediate- to high-intensity warfarin
therapy may confer better antithrombotic protection than
low- to intermediate-intensity warfarin therapy or aspirin
therapy. Further studies are needed to define more precisely
the rethrombosis rate and optimal type, intensity, and
duration of antithrombotic therapy. |
| |
|
Antiphospholipid, anti-beta 2-glycoprotein-I and
anti-oxidized-low- density-lipoprotein antibodies in
antiphospholipid syndrome |
|
|
QJM, Vol 91, Issue 9 619-626, Copyright ©
1998 by Oxford University Press |
| |
|
Archives of Rheumatology |
|
|
The On-line Archives of Rheumatology
publishes original papers dealing with the clinical
manifestations, laboratory investigations and the treatment
of rheumatic diseases. |
| |
|
Chronic fatigue syndrome and/or Fibromyalgia as a variation
of Antiphospholipid antibody syndrome: an explanatory model
and approach to laboratory diagnosis |
|
|
D. Berg, L. H. Berg, J. Couvaras and H.
Harrison (Received 22 June 1999; accepted 2 July 1999) |
| |
|
Comparison of the primary and secondary antiphospholipid
syndrome: a European Multicenter Study of 114 patients. |
|
|
Am J Med. 1994 Jan;96(1):3-9. |
| |
|
Factor XIII in Primary Antiphospholipid Syndrome |
|
|
PAUL R.J. AMES, LUIGI IANNACCONE, JOSE
DELGADO ALVES, ANNAMARIA MARGARITA, LUIS R. LOPEZ, and
VINCENZO BRANCACCIO J Rheumatol 2005;32:1058-62 Conclusion.
Enhanced FXIII activity may contribute to atherothrombosis
in primary APS via increased fibrin/fibrinogen
cross-linking. This pathway is not exclusive to primary APS,
being present also in thrombotic controls, but the presence
of IgG aPL may favor a higher degree of FXIIIa activation in
the primary APS group. |
| |
|
Family testing for genetic abnormalities |
|
|
Last Updated: 2/15/2004 |
| |
|
Further
evidence of false negative screening for lupus
anticoagulants |
|
|
Thrombosis Research. Volume 121, Issue 4,
2008, Pages 477-484. Conclusions: LA activity can be
demonstrated by assessment of screen and confirm data
irrespective of screening test elevation above a reference
range. Other workers have demonstrated this phenomenon in
APTT using different study designs and it may be that
standard interpretation criteria warrant re-assessment. |
| |
|
General Management of the Patient with a Positive
Antiphospholipid Antibody Test: What Evidence Is Available
For You and Your Physician To Consider? |
|
|
Written by: Gale A McCarty, MD, FACP,
FACR You are an individual and your particular case may
involve some features that separate you from the study
patients. What is most likely to have the right balance of
“helpful vs. harmful” effects on you is the major concern of
your physician to prevent the effects of aPL antibodies in
contributing to blood clots and cell or organ damage in you.
Let’s look at the major approaches used to manage your
positive aPL test. |
| |
|
General Management of Thromboses (Blood Clot) II—What
Evidence is Available For You and Your Physician To
Consider? |
|
|
Written by: Gale McCarty, MD, FACP, FACR.
As always, the physician treating the individual APS patient
has to balance the wisdom of these guidelines (which are
general guidelines and not mandates applicable to ALL
patients) with the needs of his/her patient to find the best
answer. |
| |
|
Genetic study on Familial Antiphospholipid Syndrome. |
|
|
Antiphospholipid syndrome (APS) is
familial in 10% of cases. |
| |
|
HYDROXYCHLOROQUINE - EVERYTHING OLD IS NEW AGAIN! |
|
|
By: Gale McCarty, MD, FACR, FACP.
Hydroxychloroquine (HCQ, or its trade name-Plaquenil) has a
long and honored history of use in systemic lupus
erythematosus (SLE) as a general medication to decrease
activity of the immune system and decrease symptoms. For
years it has been approved for use by the FDA for lupus and
rheumatoid arthritis, and has been used most frequently for
skin and joint manifestations. It is considered a mainstay
of therapy for any patient with SLE by many lupus experts
and rheumatologists. It has many mechanisms of action, some
related to decrease in the activity of the immune system,
and some related to effects on blood clotting mechanisms.
HCQ belongs to the class of drugs call anti-malarials, which
includes Chloroquine and Atabrine. (This does not mean that
anyone thinks that SLE or APS is caused by the agent that
causes malaria-like most discoveries in medicine, it was the
chance observation that patients with some autoimmune
diseases who got anti-malarial drugs to prevent malaria when
traveling to likely areas of infection noted their symptoms
improved on HCQ). One of the most complete and excellent
reviews of all the literature on the anti-malarials to which
all patients and their physicians are directed is Dr. Dan
Wallace’s Chapter 59 in the Wallace-Hahn Dubois’ Lupus
Erythematosus textbook. Another excellent review on APS
therapy in general has been published by Dr. Robert Roubey. |
| |
|
Hydroxychloroquine Reverses Thrombogenic Properties of
Antiphospholipid Antibodies in Mice |
|
|
Circulation. 1997;96:4380-4384.
Conclusions Hydroxychloroquine significantly diminished both
thrombus size and total time of thrombus formation in mice
previously injected with IgG-APS. |
| |
|
Hypercoagulable State in Patients With Antiphospholipid
Syndrome Is Related to High Induced Tissue Factor Expression
on Monocytes and to Low Free Protein S |
|
|
(Arteriosclerosis, Thrombosis, and
Vascular Biology. 1996;16:1319-1326.) © 1996 American Heart
Association, Inc. |
| |
|
Intravenous immunoglobulin therapy of antiphospholipid
syndrome |
|
|
Rheumatology 2000; 39: 421-426 © 2000
British Society for Rheumatology |
| |
|
Laboratory diagnosis and management challenges in the
antiphospholipid syndrome. |
|
|
Lupus. 2006;15(3):172-8. The
antiphospholipid syndrome (APS) is characterized by
recurrent arterial and/or venous thrombosis and pregnancy
morbidity manifested by early or late losses. Laboratory
diagnosis ofAPS relies on the demonstration of a positive
test for antiphospholipid antibodies (aPL). In clinical
practice, the gold standard tests are those that detect
anticardiolipin antibodies (aCL) and/or the lupus
anticoagulant (LA). Although other specificities for aPL
have been described their clinical utility and
standardization has still to be established. Persistence of
aPL positive tests must be demonstrated, and other causes
and underlying factors considered. Although it is
universally recognized that the routine screening tests (aCL
and/or LA) might miss some cases, careful differential
diagnosis and repeat testing are mandatory before the
diagnosis of 'seronegative APS' can be made. Correct
identification of patients with APS is important, because
prophylactic anticoagulant therapy can prevent thrombosis
from recurring, and treatment of affected women during
pregnancy can improve fetal and maternal outcome. |
| |
|
Livedo Reticularis in Antiphospholipid Antibody Syndrome |
|
|
Posted on: Tuesday, 14 February 2006,
03:03 CST |
| |
|
LJP 1082: a toleragen for Hughes syndrome |
|
|
Lupus, Vol. 13, No. 5, 335-338 (2004).
DOI: 10.1191/0961203304lu1022oa. © 2004 SAGE Publications |
| |
|
Lupus anticoagulant and INR by Dr. Stephan Moll (Q/A) |
|
|
Example: Q1: "Could you please explain
more about the effect of lupus anticoagulants on the INR
tests? My hematologist has not heard of the difficulties of
the two interacting with each other. Are there any
references you could provide so I can give them to my doc? I
have not been able to maintain a consistent INR level; it is
either at 1.8 or 3.8, very rarely does it come in between
2-3." |
| |
|
Lupus Anticoagulant Assays: Questions Answered and to Be
Answered |
|
|
Arch Pathol Lab Med—Vol 131, June 2007.
Conclusions: Although there are published criteria for
confirming the presence of a lupus anticoagulant, there is
no consensus on assay methods for lupus anticoagulant. |
| |
|
Lupus Anticoagulant: Testing While on Anticoagulant Therapy:
Can It Be Done? |
|
|
Written by: Thomas L Ortel, MD, PhD. In
conclusion, it is optimal to test for a lupus anticoagulant
when the patient is on no anticoagulant therapy. All of the
test results can be interpreted more easily in that setting.
Sometimes this can be difficult to arrange, however, and
testing needs to be performed while the patient is still
taking anticoagulants. In this situation, the doctor needs
to work carefully with the laboratory, to understand how the
tests are being performed and to make sure that the results
are interpreted correctly. |
| |
|
Lupus anticoagulants |
|
|
Update Date: 1/26/2005 Updated by: Rita
Nanda, MD., Department of Hematology/Oncology, University of
Chicago Medical Center, Chicago, IL. Review provided by
VeriMed Healthcare Network. |
| |
|
Lupus anticoagulants are stronger risk factors for
thrombosis than anticardiolipin antibodies in the
antiphospholipid syndrome: a systematic review of the
literature |
|
|
Blood, 1 March 2003, Vol. 101, No. 5, pp.
1827-1832 |
| |
|
Mechanisms of Disease: antiphospholipid antibodies—from
clinical association to pathologic mechanism |
|
|
Nature Clinical Practice Rheumatology.
Published online: 19 February 2008. doi:10.1038/ncprheum0740
Received 6 September 2007. Accepted 4 December 2007.
Conclusion and future directions: APS is still seen as a
rather obscure disease despite extensive research; this view
is mainly the result of the unreliability of the current
assays for detecting the presence of antiphospholipid
antibodies. The consequences are a poor correlation between
serological markers and clinical manifestations, and a lack
of clarity about the pathogenetic mechanism causing the
syndrome. As mentioned above, we found that the presence of
LA-inducing anti-beta2 GPI antibodies (with an affinity for
domain I) correlates almost uniformly with the occurrence of
thrombosis.21, 23 Our next aim is to investigate whether the
presence of anti-domain I antibodies correlates with
recurrent fetal loss. Research focused specifically on
anti-domain I antibodies will hopefully provide information
about the mechanism that causes APS. We have found that
these antibodies cause increased (factor V
Leiden-independent) APC resistance, increased resistance
against the anticoagulant properties of annexin A5 and
increased levels of activated von Willebrand factor (Figure
3).39, 42, 46 It is anticipated that this population is also
involved in disease-causing mechanisms other than thrombosis
in APS. It seems strange that new roles for a protein with
no previous clear function come to light in the presence of
an autoantibody. Perhaps native beta2 GPI already possesses
these functions, but requires activation (such as a
conformational change) before it can perform them. Native
beta2 GPI could potentially already exhibit these actions at
sites of cellular damage.47 Under apoptotic conditions, the
antigen would be concentrated on the anionic phospholipids
of the exposed cellular membranes and inhibit the unwanted
consequences of the exposure of negatively-charged surfaces
to components of the circulating bloodstream. In the
presence of anti-beta2 GPI antibodies, the active
conformation of the beta2 GPI protein is stabilized and its
function is no longer limited to sites of damage. The
consequence would be an imbalance in the hemostatic system,
resulting in thrombosis at any vascular site in the body. In
the future, more insight is needed into whether there are
more antibody subpopulations that can cause the (specific)
clinical manifestations that are observed in APS. Future
investigations into the pathology should be performed with
antibody populations with precisely defined epitopes. |
| |
|
Monitoring Warfarin Therapy in Patients with Lupus
Anticoagulants |
|
|
Recommended therapeutic international
normalized ratios (INRs) for oral anticoagulation in
patients with lupus anticoagulants who sustain a
thromboembolic event are controversial. Patients with lupus
anticoagulants often have a prolonged prothrombin time,
which may complicate management of anticoagulant therapy. |
| |
|
Multiple antiphospholipid tests do not increase the
diagnostic yield in antiphospholipid syndrome |
|
|
The British Journal of Rheumatology, Vol
37, 1229-1232, Copyright © 1998 by British Society for
Rheumatology |
| |
|
New approaches to prevention of thrombosis in the
antiphospholipid syndrome: hopes, trials, and tribulations. |
|
|
Arthritis Rheum. 2003 Nov;48(11):3004-8. |
| |
|
Ovarian Vein Thrombosis May Occur in Antiphospholipid
Syndrome, May Be Underdiagnosed |
|
|
Arthritis Rheum 2004 Jan;50:1:183-6.
"Ovarian vein thrombosis in the antiphospholipid syndrome" |
| |
|
Pathogenesis of the antiphospholipid syndrome: An additional
example of the mosaic of autoimmunity |
|
|
Journal of Autoimmunity. Volume 30,
Issues 1-2, February-March 2008, Pages 99-103. Autoimmunity:
From the Mosaic to the Kaleidoscope. The antiphospholipid
syndrome (APS) is a systemic autoimmune disease
characterized by an adaptive immune response against
self-PL-binding proteins ending in the production of
specific autoantibodies. Antiphospholipid antibodies (aPL;
and in particular anti-β2 glycoprotein I antibodies) are
formal diagnostic markers and pathogenic antibodies.
Although APS may be considered as an autoantibody-mediated
disease, there is now evidence that aPL are necessary but
not sufficient to trigger some of the clinical
manifestations of the syndrome. For example, additional
factors, such as mediators of the innate immunity are now
recognized to play a key role as second hits able to induce
the thrombotic events in the presence of the autoantibodies.
The APS scenario is also supplemented by the influence of
genetically determined factors. Finally, environmental
agents – in particular infectious ones – were reported to
act as triggers for the production of autoantibodies
cross-reacting with PL-binding proteins as well as
inflammatory stimuli that potentiate the aPL thrombogenic
effect. Altogether these findings do support the concept of
a mosaic of factors that participate to the pathogenesis of
the syndrome at different levels. |
| |
|
Patient information: Blood clotting problems due to
antibodies (antiphospholipid antibody syndrome) |
|
|
Peter H Schur, MD Harvard Medical School
UpToDate performs a continuous review of over 330 journals
and other resources. Updates are added as important new
information is published. The literature review for version
13.2 is current through April 2005; this topic was last
changed on July 6, 2004. The next version of UpToDate (13.3)
will be released in October 2005. |
| |
|
Patients with antiphospholipid antibodies and venous
thrombosis should receive long term anticoagulant treatment |
|
|
Annals of the Rheumatic Diseases, 1993,
Vol 52, 689-692 |
| |
|
Platelet-Endothelial Interactions: Sepsis, HIT, and
Antiphospholipid Syndrome |
|
|
Hematology 2003 © 2003 The American
Society of Hematology |
| |
|
Possible mechanisms of action of the antiphospholipid
binding antibodies. |
|
|
Clin Exp Rheumatol. 1989 Sep-Oct;7 Suppl
3:S85-9. If antibodies directed against the phospholipid
itself play any part in the pathogenesis of the thrombotic
events, the very ubiquitous nature of the antigen dictates
that no single pathogenetic mechanism can be implicated. The
majority of in vivo studies recently performed have failed
to show that antiphospholipid (aPL) antibodies bind cultured
endothelial cells, or that they affect prostacyclin
production by these cells. Previously postulated actions
showing defective fibrinolysis and impairment of coagulation
inhibitors (protein C, thrombomodulin and antithrombin III)
have yet to be substantiated. Studies have thus far failed
to demonstrate any significant binding of aPL antibodies to
intact platelets, although binding to disrupted platelets
has been documented. |
| |
|
Predicting Thrombosis Risk in Individuals with
Antiphospholipid Antibodies |
|
|
Written by: Thomas L Ortel, MD, PhD
“...several studies have shown that the presence of a lupus
anticoagulant in the blood is associated with a higher risk
for a clot than the presence of an anticardiolipin
antibody." |
| |
|
Primary antiphospholipid syndrome and panniculitis. |
|
|
On-line Arch Rheumatol 1998; 2: 4
published on June 7, 1998 |
| |
|
RESULTS OF A RANDOMIZED, PLACEBO CONTROLLED, DOUBLE BLIND
PHASE 1/2 CLINICAL TRIAL (RCT) TO ASSESS THE SAFETY AND
TOLERABILITY OF LJP 1082 IN PATIENTS WITH ANTIPHOSPHOLIPID
SYNDROME |
|
|
Arash Horizon, Michael H. Weisman, Daniel
J. Wallace, Joan T. Merrill, Matthew D. Linnik, Keith A.
Cockerill, Richard Furie. Conclusion: LJP 1082 appeared to
be well tolerated in patients with antibodies to beta-2-GPI
following a single intravenous dose up to 200 mg. Although
the single-dose trial design was not intended to measure B
cell tolerance, the observed binding of LJP 1082 to target
antibodies was consistent with its intended pharmacological
activity. The results of this trial support the further
development of a B cell toleragen to treat Antiphospholipid
Syndrome. |
| |
|
SERONEGATIVE ANTIPHOSPHOLIPID ANTIBODY SYNDROME (SNAPS)…AND
SNAPPING TO IT!! |
|
|
By: Gale McCarty, MD, FACR, FACP. “You
don’t have the syndrome because your tests are low level or
negative…” or “You have livedo, a heart valve problem, and
thrombocytopenia, but these aren’t listed as criteria for
diagnosis” are comments made frequently by healthcare
providers from many specialties to patients with clinical
features suggesting the Antiphospholipid Antibody Syndrome
(APS). |
| |
|
Significance of Persistent Antiphospholipid Antibodies in
the Elderly |
|
|
First Release July 1 2006; J Rheumatol
2006;33:1559–62. Conclusion. Interpretation of a positive
determination of APL is difficult in the elderly; persistent
LAC may be the most valuable biological marker of APS in the
elderly. |
| |
|
Significance of Persistent Antiphospholipid Antibodies in
the Elderly |
|
|
Conclusion. Interpretation of a positive
determination of APL is difficult in the elderly; persistent
LAC may be the most valuable biological marker of APS in the
elderly. (First Release July 1 2006; J Rheumatol
2006;33:1559–62) |
| |
|
Small vessel thrombosis without major thrombotic events in
systemic lupus erythematosus patients with antiphospholipid
syndrome. |
|
|
Wien Klin Wochenschr. 2000 Aug
25;112(15-16):707-10. Antiphospholipid syndrome has been
defined by the presence of antiphospholipid antibodies or
lupus anticoagulant in association with certain clinical
events, including recurrent arterial or venous thromboses
and recurrent fetal loss. It comprises two separate clinical
entities: simple, characterized by large vessel occlusions,
and catastrophic, with multiple occlusive events
predominantly affecting small vessels. Three patients with
systemic lupus erythematosus and permanently increased IgG
anticardiolipin antibody levels are being described. Only
postmortem histopathological examination revealed
microangiopathic thrombotic changes in different organs,
which were clinically silent in early stages of the disease
and misinterpreted later in its course because of a peculiar
clinical picture. All patients presented features of
catastrophic antiphospholipid syndrome in the final stage of
the disease. |
| |
|
Testing for and Clinical Significance of Anticardiolipin
Antibodies |
|
|
Clinical and Diagnostic Laboratory
Immunology, November 1999, p. 775-782, Vol. 6, No. 6
1071-412X/99/$04.00+0. The aCL assay is only one of the
methods used to detect aPL, and the test should be
administered with the LA and anti-B2GPI assays. The aCL
assay is reasonably sensitive but not at all specific, with
additional significant variation among laboratories and
among commercial kits; therefore, clinicians should treat
the clinical state and not an incidentally found antibody.
Although there is an association between antibody titer and
risk of thrombosis, this is not a ground for ignoring or not
reporting weakly positive results. False-positive results
that are difficult to interpret are particularly likely to
occur when there are other causes of thrombosis such as
atherosclerosis in the elderly; therefore, screening widely
should not be encouraged. The predictive value of testing
with the aCL ELISA for APS can be improved to an extent by
concurrent LA testing, but often the appropriate duration
and intensity of treatment after a first manifestation
cannot be determined by these two tests. The anti-B2GPI
ELISA offers an improved predictive value, but there is a
need for an optimization or evaluation procedure to
manipulate and assess the effects of the different variables
on test performance. We suggest that laboratories interested
in aPL evaluate the assay, both in an in-house form using an
optimal buffer and a large quantity of B2GPI and in a
commercial form when thoroughly tested and externally
validated kits become available. |
| |
|
Testing for and Clinical Significance of Anticardiolipin
Antibodies |
|
|
Clinical and Diagnostic Laboratory
Immunology, November 1999, p. 775-782, Vol. 6, No. 6 |
| |
|
The antiphospholipid antibody syndrome in the emergency
department setting--livedo reticularis and recurrent venous
thrombosis. |
|
|
Ann Emerg Med. 1992 Feb;21(2):207-11. We
present the case of a 26-year-old man with an exacerbation
of apparent chronic asthma with chronic peripheral vascular
disease due to recurrent venous thrombosis. Localized livedo
reticularis, new cutaneous infarctions, severe venous
insufficiency, thrombocytopenia, renal failure, and cerebral
supratentorial dysfunction were noted. During hospital
admission, antibodies to phospholipids in high titer were
present by three different testing methods. Renal biopsy
demonstrated significant renal vasculature abnormalities
characteristic of hemolytic endovasculopathy, and magnetic
resonance imaging showed multiple cerebral infarctions. This
case exemplifies the spectrum of presentations and
management of the primary antiphospholipid antibody
syndrome. The clue to its presence in this patient was the
livedo reticularis rash, a cutaneous marker for this
syndrome that was evident in the emergency department. |
| |
|
The Management of Thrombosis in the
Antiphospholipid-Antibody Syndrome |
|
|
N. Engl. J. Med. 332:993-997 April 1995.
Conclusions The risk of recurrent thrombosis in patients
with the antiphospholipid-antibody syndrome is high.
Long-term anticoagulation therapy in which the international
normalized ratio is maintained at or above 3 is advisable in
these patients. |
| |
|
The Presence of Multiple Prothrombotic Risk Factors Is
Associated with a Higher Risk of Thrombosis in Individuals
with Anticardiolipin Antibodies |
|
|
J Rheumatol 2003;30:2385-91. Conclusion.
In individuals with positive aCL-IgG, we observed an
association between the number of prothrombotic risk factors
and history of thrombotic events. |
| |
|
Thrombocytopenia in the Antiphospholipid Syndrome |
|
|
SJS Aug 2003 |
| |
|
Thrombosis and the Antiphospholipid Syndrome |
|
|
Hematology 2005 © 2005 The American
Society of Hematology. Summary: Even with the most complete
datasets, it is still important for the physician to develop
a therapeutic plan appropriate for the individual patient,
based on clinical presentation, co-morbid conditions, and
other variables. With uncommon disorders and limited
datasets, such as with the antiphospholipid syndrome,
decision-making becomes even more difficult. Table 3
presents a strategy that the author uses when evaluating and
developing a treatment plan for a patient with
antiphospholipid syndrome and thrombosis, based on the
available studies summarized in this article. Critical areas
for future research include identifying which patients with
antiphospholipid antibodies are at highest risk for
thrombotic complications, developing new antithrombotic
agents that are effective and safe, and investigating novel
approaches to eliminate the autoantibody and, hopefully, the
increased prothrombotic state. |
| |
|
Thrombotic microangiopathic haemolytic anaemia and
antiphospholipid antibodies |
|
|
Annals of the Rheumatic Diseases
2004;63:730-736 © 2004 by BMJ Publishing Group Ltd &
European League Against Rheumatism |
| |
|
Validation of the Sapporo criteria for antiphospholipid
syndrome. |
|
|
Arthritis Rheum. 2000 Feb;43(2):440-3.
Some patients with false-negative results were true
seronegative cases. CONCLUSION: The Sapporo criteria for APS
compare favorably with the American College of Rheumatology
criteria for SLE and are usable for clinical studies. |
| |
|
Warfarin in Antiphospholipid Syndrome — Time to Explore New
Horizons |
|
|
© 2005. The Journal of Rheumatology
Publishing Company Limited. |
| |
|
Top of Page |
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APS - Lupus Related
|
|
Antiphospholipid antibodies and cerebral lupus. |
|
|
Ann N Y Acad Sci. 1997; 823:270-8 (ISSN:
0077-8923) Antiphospholipid antibodies probably play a minor
role in cerebral lupus. They are associated primarily with
stroke and transient ischemic attacks, which occur only in a
minority of patients with central nervous system
complications of SLE. However, ready demonstration of
functional effects of antiphospholipid antibodies in the
coagulation system as well as persuasive evidence that they
can induce thrombosis and pregnancy loss in experimental
mice lend credence to the belief that many autoantibodies
play a direct role in disease pathogenesis. Hence the role
of the many autoantibodies associated with CNS lupus is a
legitimate field of inquiry. This review outlines the
history of antiphospholipid antibodies, discusses the
controversy concerning antiphospholipid antibody
specificity, summarizes recent experimental data on their
functional effects in the coagulation system, and describes
animal models of the antiphospholipid syndrome in which
antibodies with cardiolipin-binding activity have been shown
to play a direct role in thrombosis and pregnancy loss. |
| |
|
ANTIPHOSPHOLIPID ANTIBODIES AND SYSTEMIC LUPUS ERYTHEMATOSUS |
|
|
Michelle Petri, M.D., M.P.H. Associate
Professor of Medicine The Johns Hopkins University School of
Medicine Baltimore, MD |
| |
|
Antiphospholipid Antibodies: Anticardiolipin Antibodies and
the Lupus Anticoagulant in Systemic Lupus Erythematosus |
|
|
Michelle Petri, M.D., M.P.H. Associate
Professor of Medicine, The Johns Hopkins University School
of Medicine Baltimore, MD |
| |
|
Antiphospholipid syndrome in systemic lupus erythematosus:
is the whole greater than the sum of its parts? |
|
|
Rheum Dis Clin North Am. 2005
May;31(2):255-72 |
| |
|
Antiphospholipid Syndrome With Catastrophic Bleeding and
Recurrent Ischemic Strokes as Initial Presentation of
Systemic Lupus Erythematosus. |
|
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J Pediatr Hematol Oncol. 2005
Jul;27(7):403-407 |
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Antiphospholipids & SLE: A Clinical Overview |
|
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Dr. Graham R.V. Hughes, MD FRCP |
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Arterial disease in lupus and secondary antiphospholipid
syndrome: association with anti-beta2-glycoprotein I
antibodies but not with antibodies against oxidized
low-density lipoprotein |
|
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The British Journal of Rheumatology, Vol
37, 883-888, Copyright © 1998 by British Society for
Rheumatology |
| |
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Association between antiphospholipid antibodies and epilepsy
in patients with systemic lupus erythematosus. |
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Arthritis Rheum. 1994 Apr;37(4):568-71. |
| |
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Association between microscopic brain damage as indicated by
magnetization transfer imaging and anticardiolipin
antibodies in neuropsychiatric lupus |
|
|
Arthritis Research & Therapy 2006, 8:R38
doi:10.1186/ar1892. The pathogenetic role of anticardiolipin
antibodies (aCLs) in patients with neuropsychiatric systemic
lupus erythematosus (NPSLE) without cerebral infarcts
remains elusive. Magnetization transfer imaging (MTI) has
proved to be a sensitive tool for detecting diffuse
microscopic brain damage in NPSLE patients. In this study we
examined the correlation between grey and white matter
magnetization transfer ratio (MTR) parameters and the
presence of IgM and IgG aCLs and lupus anticoagulant in 18
patients with systemic lupus erythematosus and a history of
NPSLE but without cerebral infarcts on conventional magnetic
resonance imaging. Lower grey matter mean MTR (P < 0.05),
white matter mean MTR (P < 0.05), white matter peak location
(P < 0.05) and grey matter peak location (trend toward
statistical significance) were observed in IgM aCL-positive
patients than in IgM aCL-negative patients. No significant
differences were found in MTR histogram parameters with
respect to IgG aCL and lupus anticoagulant status, nor with
respect to anti-dsDNA or anti-ENA (extractable nuclear
antigen) status. This is the first report of an association
between the presence of aCLs and cerebral damage in grey and
white matter in NPSLE. Our findings suggest that aCLs are
associated with diffuse brain involvement in NPSLE patients. |
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Association of mannose-binding lectin gene polymorphisms
with antiphospholipid syndrome, cardiovascular disease and
chronic damage in patients with systemic lupus erythematosus |
|
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Rheumatology Advance Access originally
published online on June 26, 2006 Rheumatology 2007
46(1):76-80; doi:10.1093/rheumatology/kel199. Conclusion:
Although the prevalence of cardiovascular disease in our SLE
patients carrying MBL-deficient genotypes was 3.3 times
higher than in patients with non-deficient genotypes, only
APS was independently associated with cardiovascular events.
This suggests that the higher frequency of thrombotic events
in SLE patients carrying MBL-deficient genotypes might be
related to coexisting APS. |
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Autoantibodies to human prothrombin and clinical
manifestations in 207 patients with systemic lupus
erythematosus. |
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J Rheumatol. 1998 Jun;25(6):1104-8. |
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Bone infarcts in a woman with systemic lupus erythematosus
and antiphospholipid antibody syndrome |
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CMAJ • February 14, 2006; 174 (4).
doi:10.1503/cmaj.050801. © 2006 CMA Media Inc. or its
licensors |
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Clinical and laboratory diagnosis of heart disease in
systemic lupus erythematosus |
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Arq Bras Cardiol. 1997 Feb;68(2):79-83.
CONCLUSION: Myocardial disease was the most frequent heart
involvement in active SLE, and we did not found any
association between SLE heart disease and positive
anticardiolipin antibodies. |
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High Impact of Antiphospholipid Syndrome on Irreversible
Organ Damage and Survival of Patients With Systemic Lupus
Erythematosus |
|
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Arch Intern Med. 2004;164:77-82.
CONCLUSIONS: Antiphospholipid syndrome with thrombotic
manifestations is a major predictor of irreversible organ
damage and death in patients with SLE. |
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I Have the Lupus Anticoagulant, But I Don’t Have Lupus? |
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By: Thomas L. Ortel, MD, PhD “...although
the ‘lupus’ anticoagulant was first described in several
patients with lupus, most patients with lupus anticoagulants
actually don’t have any of the other clinical manifestations
of lupus" |
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Long-Term Follow-Up in 128 Patients With Primary
Antiphospholipid Syndrome: Do They Develop Lupus? |
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Medicine. 84(4):225-230, July 2005.
Gomez-Puerta, Jose A. MD; Martin, Helena MD; Amigo,
Mary-Carmen MD; Aguirre, Maria A. MD; Camps, Maria T. MD;
Cuadrado, Maria J. MD, PhD; Hughes, Graham R. V. MD, FRCP;
Khamashta, Munther A. MD, FRCP, PhD. The current study
confirms that progression from primary APS to SLE or
lupus-like disease is unusual, even after a long follow-up.
Only 3 patients developed anti-dsDNA antibodies. The
presence of a positive Coombs test might be a marker for the
development of SLE in patients with primary APS. |
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Lupus and Antiphospholipid Syndrome (APS) |
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This article was published on Thursday 18
November, 2004. |
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Lupus and Thrombosis |
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|
September 2006. In summary, lupus
patients are at significantly increased risk for premature
atherosclerosis and thrombosis, which is a multifactorial
process. A risk-stratified approach to thrombosis risk
assessment (i.e., lupus disease activity/severity,
traditional and lupus-related as well as acquired and
genetic thrombotic risk factors, and aPL profile) is
important in the management of lupus patients; however,
there are no evidence-based recommendations yet for the
primary thrombosis prevention. Current and future clinical
lupus trials hopefully will further advance primary
thrombosis prevention strategies. |
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Non-invasive cardiac evaluation in patients with systemic
lupus erythematosus. |
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|
J Med. 2001;32(3-4):195-206. The aim of
this study was non-invasive assessment of cardiac function
in patients with systemic lupus erythematosus (SLE). In a
group of 36 patients with SLE transthoracic
echocardiography, standard ECG and the 24-hour ECG Holter
monitoring were performed and the results were compared to a
control group of 35 healthy volunteers. Significantly lower
mean values of heart rate variability (HRV), the presence of
late ventricular potentials and tendency to tachycardia were
detected in SLE patients when compared to the control group.
On echocardiography examination valvular lesions were found
in 15 SLE patients but only in 5 of them were insignificant
mitral or aortic regurgitant jets observed. Echocardiography
did not reveal abnormalities in cardiac dimensions and left
ventricle systolic function. Abnormal indexes of left
ventricular filling were found in 3 patients. All SLE
patients with antiphospholipid antibodies had some
cardiovascular manifestation. |
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Systemic Lupus Erythematosus and Antiphospholipid Antibodies |
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Mountain States Regional Genetic Services
Network Systemic Lupus Erythematosus and Antiphospholipid
Antibodies
http://www.obgyn.net/pb/articles/systemic_lupus_pg.htm 2002 |
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Systemic Lupus Erythematosus and Antiphospholipid
Antibodies: One Cause of Neurologic Dysfunction |
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|
by Alan Lash, MD. This is an original
article prepared for the Bay Area Lupus Foundation |
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Systemic lupus erythematosus in a multiethnic US cohort
(LUMINA): XXV. Smoking, older age, disease activity, lupus
anticoagulant, and glucocorticoid dose as risk factors for
the occurrence of venous thrombosis in lupus patients. |
|
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Arthritis Rheum. 2005 Jul;52(7):2060-8
CONCLUSION: Venous thrombotic events occur early in the
course of SLE. Our data confirm the association between LAC
and venous thrombotic events. Smoking, shorter disease
duration, older age, disease activity over time, and higher
mean daily glucocorticoid dose were identified as additional
risk factors for the development of this vascular
complication. These findings may have implications for the
management of patients with SLE. |
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Top of Page |
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APS - Lupus Research & Trials Information
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12th International Congress on Anti-Phospholipid Antibodies |
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18-20 April 2007 in Florence, Italy |
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A Pilot Study of a Cognitive Rehabilitation Intervention
Program in Systemic Lupus Erythematosus (SLE) Patients |
|
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Study of a novel group educational
program for lupus patients to improve their ability to
perform everyday tasks that have become difficult due to
cognitive dysfunction. Sponsor: Mary Kirkland Center for
Lupus Research. Principal Investigator: Melanie J. Harrison,
MD, MS. HSS IRB #: 22076 |
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A Study of the Genetics of Antiphospholipid Antibody
Syndrome |
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Written by: Melissa Hall |
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Antiphospholipid Antibody Syndrome (APS) |
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We know very little about why people
develop APS. The goal of this research study is to identify
genetic or environmental factors that may increase
susceptibility to APS. We are looking for families where an
individual has APS and one or more family members also have
APS. We are also looking for families where an individual
has APS and one or more family members has an autoimmune
disease such as lupus, diabetes, rheumatoid arthritis,
multiple sclerosis, or similar disorders. |
| |
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Antiphospholipid syndrome in patients with systemic lupus
erythematosus treated by autologous hematopoietic stem cell
transplantation |
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Blood, 15 October 2005, Vol. 106, No. 8,
pp. 2700-2709. Prepublished online as a Blood First Edition
Paper on May 3, 2005; DOI 10.1182/blood-2005-01-0330. |
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Antiphospholipid Syndrome Studies/Research |
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One fast click to see the current studies
dealing with APS. |
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APLASA Study: Determining the Role of Aspirin for Thrombosis
Prophylaxis in Antiphospholipid Syndrome |
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An international multi-center,
randomized, placebo (sugar tablet) controlled study designed
to study whether low-dose aspirin can prevent blood clots in
asymptomatic aPL-positive individuals (those who only have
the antibodies but have never had a clot). Sponsors: Bayer
Pharmaceuticals, Arthritis Foundation (New York Chapter).
Principal Investigator: Doruk Erkan, MD. HSS
Co-investigators: Melanie J. Harrison MD, MS, Michael D.
Lockshin, MD, Lisa Sammaritano, MD, and Margaret Peterson,
PhD. HSS IRB #: 20068 |
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APLASA-P Study: Determining the Role of Aspirin in the
Management of Antiphospholipid Syndrome Patients who Present
Only with Pregnancy Morbidity |
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An international multi-center,
observational study designed to study whether low-dose
aspirin can prevent blood clots in APS patients who have
only had miscarriages but no blood clots in other vessels.
Sponsors: Bayer Pharmaceuticals, Arthritis Foundation, New
York Chapter. Principal Investigator: Doruk Erkan, MD. HSS
Co-investigators: Melanie J. Harrison MD, MS, Michael D.
Lockshin, MD, Lisa Sammaritano, MD, Margaret Peterson, PhD.
HSS IRB #: 20069 |
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APSCORE: National Antiphospholipid Syndrome Collaborative
Registry |
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National registry that collects clinical
and laboratory information on patients with antiphospholipid
antibodies and/or syndrome. Sponsor: National Institute of
Arthritis and Musculoskeletal and Skin Diseases (NIAMS)
Principal Investigator: Robert Roubey, MD (University of
North Carolina at Chapel Hill) HSS Investigators: Michael D.
Lockshin, MD and Lisa Sammaritano, MD. HSS Co-investigator:
Doruk Erkan, MD. HSS IRB #: 20067 |
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CAPS REGISTRY |
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REGISTRY OF THE "EUROPEAN FORUM ON
ANTIPHOSPHOLIPID ANTIBODIES" FOR PATIENTS WITH THE
"CATASTROPHIC" ANTIPHOSPHOLIPID SYNDROME |
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CDC Hemostatis and Thrombosis Center Pilot Site Locations |
|
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The Centers for Disease Control and
Prevention supports a network of specialized health-care
centers to prevent and reduce complications experienced by
persons with certain hereditary blood disorders. Currently,
CDC has provided funds to eight “pilot” hemostatis and
thrombosis centers to find out how to best provide treatment
and preventative care to people with thrombosis or
thrombophilia. These centers have multi-disciplinary teams
of health-care specialists, state-of-the art clinical
research programs, and provide outreach and education
programs for patients. |
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Clinical Trials Information for Patients |
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|
In medical research, a clinical trial is
an organized study conducted in people with a disease or
condition to answer specific questions about a new treatment
or a new way of using a known treatment. Each study tries to
increase medical knowledge and to find new and better ways
to help patients. Besides studying new drugs, clinical
trials study new combinations of drugs already used in
treatment, new ways of giving treatment, and how changes in
lifestyle can help patients or prevent diseases from
occurring. Other clinical trials compare the best known
standard therapy with a newer therapy to see if one produces
more cures and causes fewer side effects than the other. |
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Clinical Trials.gov |
|
|
ClinicalTrials.gov provides regularly
updated information about federally and privately supported
clinical research in human volunteers. ClinicalTrials.gov
gives you information about a trial's purpose, who may
participate, locations, and phone numbers for more details.
Before searching, you may want to learn more about clinical
trials. |
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Clinical Trials: Strokes |
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|
Research will evaluate whether study
medication will affect breathing, relax tightness in the
muscles, and allow the wrist, fingers, and elbow to be
straightened. Study will last for about 32 weeks; if you
participate you will be asked to make nine visits to the
study center, where you will have injections into your arm
and hand. Research site located in Pittsburgh, Penn. |
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Cognitive Function in Lupus and Primary Antiphospholipid
Syndrome Patients over Time |
|
|
An ongoing, observational investigation
of lupus patients designed to identify cognitive dysfunction
and factors associated with it. Sponsors: Arthritis
Foundation (New York Chapter and National Office), Mary
Kirkland Center for Lupus Research, NIAMS. Principal
Investigator: Melanie J. Harrison, MD, MS RCRC
Co-investigators: Michael D. Lockshin, MD and Doruk Erkan,
MD. HSS IRB #: 97054 |
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CRISP (Computer Retrieval of Information on Scientific
Projects) |
|
|
CRISP (Computer Retrieval of Information
on Scientific Projects) is a searchable database of
federally funded biomedical research projects conducted at
universities, hospitals, and other research institutions.
Maintained by the Office of Extramural Research at the
National Institutes of Health (NIH), CRISP includes projects
funded by the NIH as well as the Substance Abuse and Mental
Health Services Administration (SAMHSA), Health Resources
and Services Administration (HRSA), Food and Drug
Administration (FDA), Centers for Disease Control and
Prevention (CDC), Agency for Healthcare Research and Quality
(AHRQ), and Office of Assistant Secretary of Health (OASH).
Currently, 27 projects listed on CRISP involve APS. To read
about these studies, click on the link below. In the 'enter
search terms' box, enter 'antiphospholipid syndrome' and
click the 'and' button below the box. Then click 'Submit
Query.' |
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Dexamethasone Treatment for Congenital Heart Block (CHB) in
Newborns With Lupus |
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Purpose: Some newborns are born with
congenital heart block (CHB), a condition occurring in
babies with neonatal lupus. The first part of the study will
test the effectiveness of fluorinated steroids, including
dexamethasone, in improving the heart function and general
health of newborns who have auto-antibody-associated CHB.
The second part of this study will use ultrasound and heart
monitoring to observe high-risk pregnant women and their
fetuses during the third trimester of pregnancy. |
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Effect of antimalarials on thrombosis and survival in
patients with systemic lupus erythematosus |
|
|
Lupus, Volume 15, Number 9, September
2006, pp. 577-583(7). Our study shows a protective effect of
antimalarials against thrombosis and an increased survival
of SLE patients taking these drugs. These data support the
routine use of antimalarials in all patients with SLE. |
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Genetics of antiphospholipid antibody syndrome (APS): Study
#1 |
|
|
We know very little about what causes
antiphospholipid antibody syndrome (APS). Sometimes several
family members have APS. Other times, one person has APS and
other relatives have a different autoimmune disease like
lupus or diabetes. Because APS sometimes runs in families,
we think that certain genes may cause APS. The purpose of
this study is to find the genes that cause APS. |
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Genetics of Antiphospholipid Antibody Syndrome Study 5806:
Brochure |
|
|
We are looking for families where an
individual has APS and one or more family members also have
APS. We are also looking for families where an individual
has APS and one or more family members has an autoimmune
disease such as lupus, diabetes, rheumatoid arthritis,
multiple sclerosis, or similar disorders. If your family has
one or more members with APS or another autoimmune disorder,
your family may be eligible to join the study. |
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Genetics of Antiphospholipid Syndrome Study |
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|
Written by: Thomas L Ortel, MD PhD. We
are currently recruiting patients with APS for a study
investigating the genetics of the disorder. The “proband”
(typically the patient) must have symptoms associated with
the disorder (such as blood clots, or recurrent
miscarriages) and the presence of the antibodies associated
with the syndrome (for example, the lupus anticoagulant, or
anticardiolipin antibodies). The “proband” also needs to
have at least one or more family members affected with APS,
or one or more family members affected with another
autoimmune disorder (for example, lupus, rheumatoid
arthritis, multiple sclerosis, myasthenia gravis, etc.). We
currently have participants enrolled from more than sixty
families, and are looking for more! |
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HUMAN GENOME SCIENCES ANNOUNCES FULL PRESENTATION OF RESULTS
OF PHASE 2 CLINICAL TRIAL OF LYMPHOSTAT-B™ IN SYSTEMIC LUPUS
ERYTHEMATOSUS |
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|
Human Genome Sciences, Inc. announced
that a Phase 2 clinical trial demonstrated that
LymphoStat-B™ (belimumab) significantly reduced disease
activity in patients with serologically active systemic
lupus erythematosus (SLE), exhibited clinically relevant
bioactivity, and was safe and well tolerated. Two oral
presentations reported the complete study results today in
Amsterdam at the Annual European Congress of Rheumatology
(EULAR 2006). |
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Identifying Unexplored Domains of Quality of Life in Lupus
Patients |
|
|
Study to identify facets of daily life
that have been impacted upon by lupus that are not routinely
evaluated in studies of quality of life in adults with
lupus. Sponsors: Mary Kirkland Center for Lupus Research.
Principal Investigator: Melanie J. Harrison, MD, MS. HSS IRB
#: 21067 |
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Information for Potential Participants of the Genetics of
Venous Thrombosis Study |
|
|
Why is this study being done? The purpose
of this study is to determine inherited causes of venous
thromboembolism(includes both deep vein thrombosis and
pulmonary embolism) by studying families in whom two or more
family members have had either deep vein thrombosis or
pulmonary embolism. |
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|
LCTC: Lupus Clinical Trials Consortium HSS Clinical Lupus
Registry |
|
|
A clinical registry of systemic lupus
erythematosus (SLE) patients that is created by the
Department of Rheumatology at HSS for LCTC multicenter
clinical lupus studies. Sponsor: Lupus Clinical Trials
Consortium. Clinical Director: Doruk Erkan, MD. Center
Director: Michael D. Lockshin, MD. HSS co-investigator:
Melanie J. Harrison, MD. HSS IRB #: 24023 |
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Longitudinal Study of the Clinical and Haematological Cause
of Women With Antiphospholipid Antibodies. |
|
|
This study is currently recruiting
patients. Verified by Imperial College London September 2005 |
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Lupus
Clinical Trials |
|
|
Our goal is to get as many people
enrolled in lupus clinical trials as possible, so that the
lupus community can finally get some answers on prevention,
treatments, and a cure. |
| |
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Lupus Research Institute |
|
|
Recognizing that most major medical
breakthroughs come from unexpected directions, the LRI
fosters and supports only the highest ranked new science to
prevent, treat and cure lupus. Millions in private sector
funding support more than 50 scientists pursuing basic and
clinical research studies at leading medical institutions
around the country. |
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Lupus
Research Institute - Chicago |
|
|
The LRI Chicago shares the mission of its
lead organization, the Lupus Research Institute, in
championing only new science — the truly innovative research
that will prevent, treat and cure lupus. |
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Multiple Autoimmune Disease Genetics Consortium (MADGC) |
|
|
MADGC is a group of leading genetic
researchers who have joined efforts to identify and
understand the genes that autoimmune diseases have in
common. This research may ultimately give doctors valuable
knowledge about the basic causes of autoimmune disease and
allow them to better diagnose and treat patients. |
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NIH Launches Clinical Studies Nationwide to Investigate Rare
Diseases |
|
|
$71 Million Effort to Address Neglected
Conditions. The National Institutes of Health (NIH)
announced today it is launching the first clinical studies
of its Rare Diseases Clinical Research Network (RDCRN). More
than 20 studies are expected to open in the next few months
at about 50 sites across the United States and in several
other countries including the United Kingdom, Japan, and
Brazil. |
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NIH Office of Rare Diseases |
|
|
Online resources and databases on
completed current, or planned rare disease studies. If you
enter the Web sites listed below, you will leave the ORD Web
site. Please return to our Web site to find more information
on rare diseases, patient support groups, and genetic
testing laboratories and clinics. |
| |
|
Pilot and Feasibility Study of Modafinil Treatment to
Improve Cognitive Efficiency in SLE Patients |
|
|
This study is being conducted in order to
determine if the FDA-approved drug Modafinil can improve
cognitive function in patients with systemic lupus
erythematosus (SLE). Sponsor: Mary Kirkland Center for Lupus
Research. Principal Investigator: Melanie J. Harrison, MD,
MS. Co-investigators: Michael D. Lockshin, MD. HSS IRB #:
25085 |
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Predictors of Pregnancy Outcome in Systemic Lupus
Erythematosus (SLE) and Antiphospholipid Syndrome (APS) |
|
|
This study is currently recruiting
patients. Verified by Hospital for Special Surgery, New York
September 2005 |
| |
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Pregnancy Complications & APS |
|
|
Antiphospholipid antibody syndrome (APS)
is a medical problem that is associated with health risks
for the pregnant mother and her baby. Initial selection
criteria for the study include women between 18 and 44 years
of age, diagnosis of antiphospholipid antibody syndrome and
able to safely undergo treatment with heparin. Research site
located in Chicago, Ill. |
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PreventionGenetics |
|
|
PreventionGenetics performs DNA testing
and DNA banking for health care and biomedical research. The
mission of PreventionGenetics is to prevent disease and
disability through genetic testing. PreventionGenetics is
dedicated to the highest standards of genetic Ethical
Principles. |
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Quality of Life in Pediatric SLE |
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Study to determine what characteristics
of childhood lupus are associated with decreased quality of
life. Sponsors: Hospital for Special Surgery, Department of
Pediatrics Principal Investigator: Lakshimi Moorthy, MD and
Thomas Lehman, MD. Co-investigators: Melanie J. Harrison,
MD, MS, Margaret Peterson, PhD. |
| |
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Rare Diseases Clinical Research Network |
|
|
The Rare Diseases Clinical Research
Network was created to facilitate collaboration among
experts in many different types of rare diseases. Our goal
is to contribute to the research and treatment of rare
diseases by working together to identify biomarkers for
disease risk, disease severity and activity, and clinical
outcome, while also encouraging development of new
approaches to diagnosis, prevention, and treatment. |
| |
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Rare Thrombotic Diseases Consortium |
|
|
The Duke Hemostasis & Thrombosis Center
is a multidisciplinary program devoted to improving the care
of patients with bleeding and clotting disorders through
innovative basic and clinical research efforts. The Center
was formed in 2001, when it was selected by the Centers for
Disease Control & Prevention to be one of eight pilot
Hemostasis & Thrombosis Centers in the United States .
Leadership of the program includes faculty in Hematology,
Maternal-Fetal Medicine, Pediatric Hematology, and
Cardiology. Ongoing clinical research trials are
investigating the optimal 'dose' of platelets for
replacement therapy, the role of inherited hypercoagulable
states in intrauterine growth restriction, and therapeutic
strategies for patients with end-stage renal disease and
recurrent vascular access occlusions. |
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Rheumatology Clinical Research Center (RCRC) |
|
|
The RCRC at the Hospital for Special
Surgery under the Division of Rheumatology is comprised of
rheumatologists with strong interests and expertise in
epidemiological, health services, and outcomes research in
various rheumatic diseases. The goal of the RCRC is to
design and execute scientifically sound clinical studies
that are important to clinical rheumatology and the care of
patients. |
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|
Steroids and Antiphospholipid Syndrome- Related Pregnancy
Loss |
|
|
This study is not yet open for patient
recruitment. Verified by Imperial College London September
2005 |
| |
|
Stroke Trials Directory |
|
|
Welcome to the Stroke Trials Directory, a
continuously updated registry of randomized clinical trials.
This project is a joint effort of the Internet Stroke Center
at Washington University School of Medicine, the American
Stroke Association and the National Institute of
Neurological Disorders and Stroke. Please visit our
frequently asked questions page for more information about
this site. |
| |
|
The
Alliance for Lupus Research |
|
|
|
| |
|
The Lupus
Multiplex Registry and Repository (LMRR) |
|
|
The Lupus Multiplex Registry and
Repository (LMRR) is: A long-term research project operating
in conjunction with the Lupus Genetic Studies; Sponsored by
the National Institute of Arthritis and Musculoskeletal and
Skin Diseases (NIAMS, a branch of the National Institutes of
Health or NIH); The only research resource of multiplex
lupus families in the world that scientists in the USA can
apply to use in their own lupus research; A collection of
DE-IDENTIFIED materials from families with 2 or more members
diagnosed with SLE including: Clinical Data, Serum, Plasma &
DNA. |
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|
The State of Lupus Research |
|
|
|
| |
|
Thrombosis
Interest Group of Canada (T.I.G.C) |
|
|
The Thrombosis Interest Group of Canada
consists of a group of 40 specialists in fields related to
thrombosis who collaborate to write evidence-based or
consensus-based clinical guides on the investigation,
management, and diagnosis of thrombotic disorders. |
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Top of Page |
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APS - Nephrology Related
|
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Acute renal failure due to bilateral renal artery thrombosis
associated with primary antiphospholipid syndrome |
|
|
Nephrology Dialysis Transplantation, Vol
13, Issue 10 2645-2647, Copyright © 1998 by Oxford
University Press |
| |
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Acute renal failure in a renal transplant donor due to
primary antiphospholipid syndrome. |
|
|
Am J Nephrol. 2001 Jan-Feb;21(1):55-7.
Primary antiphospholipid antibody (APA) syndrome, a common
prothrombotic disorder, has been known in dialysis patients
and renal transplant recipients. We report a case of primary
APA syndrome presenting as a posttransplant complication in
a renal transplant donor. A renal donor presented with
acute, painless anuria due to renal artery thrombosis 6
years following renal transplant surgery, subsequent
thrombosis of jugular catheter and arteriovenous fistula
occurred, despite anticoagulation treatment, due to primary
APA syndrome. This incident represents the most catastrophic
complication reported in a renal donor due to primary APA
syndrome. The validity of a prothrombotic assay in an organ
donor workup to detect predilection to hypercoagulable
disorders and to prevent such complications is open to
question. The actual significance of APA in the blood is
unclear; hence, the presence of APA in a potential renal
donor would pose an ethical and practical dilemma. |
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Antiphospholipid antibody syndrome and posttransplant renal
thrombosis. |
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Transplant Proc. 1999
Feb-Mar;31(1-2):230-3. |
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Antiphospholipid syndrome and renal artery stenosis |
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Q J Med 2000; 93: 127-129 © 2000
Association of Physicians. |
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Antiphospholipid syndrome and renal artery stenosis |
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Q J Med 2000; 93: 127-129 © 2000
Association of Physicians |
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Antiphospholipid syndrome nephropathy in patients with
systemic lupus erythematosus and antiphospholipid
antibodies: prevalence, clinical associations, and long-term
outcome. |
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Arthritis Rheum. 2004 Aug;50(8):2569-79.
OBJECTIVE: To evaluate the prevalence, clinical
associations, and outcome of antiphospholipid syndrome (APS)
nephropathy in patients with systemic lupus erythematosus
(SLE) and antiphospholipid antibodies (aPL) and in SLE
patients without aPL. METHODS: Kidney biopsy specimens
obtained from 81 patients with aPL (18 of whom had APS) and
70 patients without aPL were retrospectively examined for
the presence of APS nephropathy. Clinical and serologic data
obtained at the time of kidney biopsy and during a mean
followup of 7 years were recorded. In cases for which serial
kidney biopsy specimens were available, the evolution of APS
nephropathy was examined. RESULTS: APS nephropathy existed
in 39.5% of patients with aPL, compared with only 4.3% of
patients without aPL. APS nephropathy was associated with
both lupus anticoagulant and anticardiolipin antibodies.
Among aPL-positive SLE patients, APS nephropathy was found
in two-thirds of those with APS and in one-third of those
without APS. A strong association between APS nephropathy
and the presence of arterial thrombosis and livedo
reticularis was noted. Patients with APS nephropathy had a
higher frequency of hypertension and elevated serum
creatinine levels at the time of kidney biopsy but did not
have a higher frequency of renal insufficiency, end-stage
renal disease, or death at the end of followup. Serial
kidney biopsy specimens were available from 11 patients and
showed progression of APS nephropathy lesions. During
followup, manifestations of APS (especially arterial
thromboses) developed more frequently in the SLE/non-APS
patients with APS nephropathy than in those without APS
nephropathy. CONCLUSION: Among patients with SLE, APS
nephropathy occurs almost exclusively in those with aPL,
suggesting an important role of aPL in the pathogenesis of
APS nephropathy. Patients with APS nephropathy develop
hypertension, raised serum creatinine levels, and
progression of histologic lesions, all of which are
associated with a poor renal outcome. Manifestations of APS
also tend to develop in these patients. APS nephropathy
should be included in the APS classification criteria, and
the use of appropriate anticoagulant therapy should be
tested. |
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Is it possible to diagnose primary anti-phospholipid
syndrome (PAPS) on the basis of renal thrombotic
microangiopathy (PAPS nephropathy) in the absence of other
thrombotic process? |
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Ren Fail. 2003 Nov;25(6):1043-9. |
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Nephropathy and hypertension as manifestations in a 13-y-old
girl with primary antiphospholipid syndrome. |
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Acta Paediatr. 1998 Aug;87(8):903-7. |
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Renal artery stenosis in hypertensive patients with
antiphospholipid (Hughes) syndrome: outcome following
anticoagulation. |
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Rheumatology (Oxford). 2005
Mar;44(3):372-7. Epub 2004 Nov 30. |
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Renal artery stenosis in the antiphospholipid (Hughes)
syndrome and hypertension. |
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Ann Rheum Dis. 2003 Oct;62(10):999-1002.
CONCLUSION: A significantly increased prevalence of RAS
(26%) was found in patients with APS and hypertension,
compared with relatively young ( |
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Renal infarction in a severely hypertensive patient with
lupus erythematosus and antiphospholipid antibodies. |
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Nephron. 1996;72(2):298-301. |
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Renal infarction in antiphospholipid syndrome |
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Conclusion: High index of suspicion is
required in the appropriate clinical setting with
unexplained asymmetrical functioning of kidney. |
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Renal thrombotic microangiopathy in patients with systemic
lupus erythematosus and the antiphospholipid syndrome. |
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Am J Kidney Dis. 1992 Aug;20(2):150-8. |
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Renal thrombotic microangiopathy in patients with systemic
lupus erythematosus and the antiphospholipid syndrome. |
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Am J Kidney Dis. 1992 Aug;20(2):150-8.
Current studies indicate that a thrombotic microangiopathy (TMA)
identifies patients with systemic lupus erythematosus (SLE)
who are at high risk of progressing to end-stage renal
disease. We have observed two patients with SLE and one
patient with a primary antiphospholipid syndrome (APS) who
developed acute renal insufficiency with thrombocytopenia.
Renal biopsies showed a TMA characterized by thrombi or by
cellular and mucoid intimal hyperplasia of small arteries
and arterioles. No arterial or arteriolar immune-complex
deposits were detected by immunofluorescent or electron
microscopy. Biopsies from one SLE patient and the APS
patient showed no immune-complex glomerular disease. Both
had serum antiphospholipid antibodies (aPL). aPL were not
detected in the serum of the other SLE patient who had an
active lupus nephritis. Acute renal failure and
thrombocytopenia resolved in each case following treatment
by plasmapheresis or prednisone and heparin. None of the
patients were initially treated with cytotoxic drugs. As
more knowledge is gained, the accurate identification of
renal vascular lesions in SLE or related diseases could
influence renal prognosis and choice of therapy. The cases
reported here provide further evidence that a TMA can cause
acute renal failure independent of lupus nephritis. TMA
should be distinguished from other forms of renal vascular
disease, particularly a noninflammatory lupus
microangiopathy, which is probably mediated by
subendothelial immune-complex deposits. The absence of
immunoglobulin deposits in vessels involved by a TMA
indicates that microvascular thrombosis is promoted by
mechanisms other than those usually attributed to
immune-complex disease. Phospholipid reactive antibodies may
be pathogenetic in some cases. |
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Renovascular Hypertension Observed in a Patient With
Antiphospholipid-Antibody Syndrome |
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Japanese Circulation Journal Vol. 64
(2000) , No. 7 541-543 |
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Study of renal histopathological correlation with
anticardiolipin antibody status in patients with systemic
lupus erythematosus |
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Menon RN, Bichile LS. Study of renal
histopathological correlation with anticardiolipin antibody
status in patients with systemic lupus erythematosus. Indian
J Nephrol 2007;17:53-60. Conclusions: Hypocomplementemia is
the only probable marker of renal morbidity in the
laboratory follow-up of the SLE patients. aCL positivity
does contribute to greater incidence of interstitial
fibrosis, tubular atrophy and capillary wall thickening in
biopsy specimens. APSN is an independent entity that must be
specifically sought for on histological assessment. |
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The Intrarenal Vascular Lesions Associated with Primary
Antiphospholipid Syndrome |
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J Am Soc Nephrol 10:507-518, 1999 © 1999
American Society of Nephrology. This study allows us to
approach the nephropathy of PAPS as an entity in its own
right, characterized by small-vessel vaso-occlusive
intrarenal pathology that likely evolves by repeated flares
leading to a morphologic picture suggestive of its diagnosis
even in the absence of history. This picture combines the
lesions of TMA, FIH of the arteries and arterioles, and FCA.
The clinical hallmark of this nephropathy is hypertension,
only variably associated with renal insufficiency, protein-uria,
or hematuria. Conversely, the recognition of such lesions on
a biopsy performed for diagnostic purposes should lead to
work-up for possible PAPS. However, we need to understand
better the morbidity associated with this nephropathy to be
able to define the indications for and modalities of
treatment. |
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