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.

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.

written by the APS Foundation of America, Inc.

Circulation. 2005;112:e39-e44. © 2005 American Heart Association, Inc. Caron P. Misita, PharmD; Stephan Moll, MD

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

Promtes an INR in the range of 2.5-3.5.

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.

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.

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.

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.

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."

Written by: Thomas L. Ortel, MD, PhD

Written by: Thomas L. Ortel, MD, PhD

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.

Postgraduate Medical Journal 2003;79:81-83 © 2003 Fellowship of Postgraduate Medicine. Promotes an INR of greater than 3.0.

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.

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."

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.

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.

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).

Written by: Silvia Pierangeli

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.

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.

The Journal of Rheumatology 2003; 30:9 The true history of Antiphospholipid Antibody Syndrome, dating back to 1906.