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Antiphospholipid Syndrome (APS) or Hughes Syndrome

This page was written by Shelley Ensz, and has not yet been medically edited. See Disclaimer.

What is APS?
APS and Heart Disease
Scleroderma and APS
Symptoms of APS
Complications of APS

Treatments for APS
Causes of APS and Research
Support Organizations for APS
Personal Stories about APS
Media Stories about APS

What is Antiphospholipid Syndrome?

The Antiphospholipid Story. Before the concept of an antiphospholipid syndrome originated, lupus patients with venous occlusions and particularly those with arterial occlusions were treated mainly with corticosteroids and immunosuppressives. In addition, patients with primary APS were often diagnosed as lupus and met classification criteria for this disease. This could have been considered reasonable were it not for the unnecessary steroid treatment they received instead of merely anticoagulant and/or platelet antiaggregant treatment. Donato Alagcon-Segovia, MD, MS, PhD. J Rheumatol. Vol 30: No. 9. Sept 2003. (Also see: Lupus)

Catastrophic Antiphospholipid Syndrome: Treatment, Prognosis, and the Risk of Relapse. The "catastrophic" variant of the antiphospholipid syndrome (APS) is characterized by multiple vascular occlusive events, usually affecting small vessels and developing over a short period of time. (PubMed) S. Bucciarelli. Clin Rev Allergy Immunol.Dec 2 2008.

The antiphospholipid syndrome (Hughes' syndrome) NetDoctor.

Antiphospholipid Syndrome and Heart Disease

Antiphospholipid antibodies and risk of myocardial infarction and ischaemic stroke in young women in the RATIO study: a case-control study. Our results suggest that lupus anticoagulant is a major risk factor for arterial thrombotic events in young women, and the presence of other cardiovascular risk factors increases the risk even further. Rolf T Urbanus PhD. The Lancet Neurology. November 2009.

The cardiac manifestations of antiphospholipid syndrome and their echocardiographic recognition. Cardiac involvement occurs frequently. Leaflet thickening and vegetations are detected quite often echocardiographically, but hemodynamically significant stenotic and/or regurgitant valvular disease is uncommon. Silbiger JJ. (PubMed) J Am Soc Echocardiogr. 2009 Oct;22(10):1100-8.

Scleroderma and Antiphospholipid Syndrome

Clinical significance of antiphospholipid antibodies in Indian scleroderma patients. In patients with systemic sclerosis (SSc), antiphospholipid antibodies (aPL) have been reported to be associated with more severe manifestations including digital infarct, gangrene and pulmonary hypertension. But these findings are not consistent in all studies; moreover, there are no data available from Indian subcontinent. Gupta R. Rheumatol Int. 2009 Aug 23. (PubMed).

Antiphospholipid antibody syndrome and autoimmune diseases. Evidence is growing that antiphospholipid antibodies may have a pathogenic role in pulmonary hypertension and accelerated atherosclerosis of autoimmune diseases. Ostrowski RA. Hematol Oncol Clin North Am. 2008 Feb;22(1):53-65, vi. (PubMed)

Thrombosis in Systemic Lupus Erythematosus (SLE) and Other Autoimmune Diseases of Recent Onset. Patients with autoimmune diseases, particularly SLE, are at an increased risk of thrombosis. J. Romero -Diaz. J Rheumatol First Release Nov 15 2008. (Also see: Lupus)

Symptoms of Antiphospholipid Syndrome

Antiphospholipid antibodies can come and go in an individual. There are many ways to measure these antibodies, and different methods may not always give the same result. The presence of these antibodies may also increase the future risk of deep venous thrombosis (thrombophlebitis), stroke, gangrene, and heart attack. Lupus Foundation of America. (Also see: Scleroderma Cardiac Involvement)

Complications of Antiphospholipid Syndrome

Pregnancy outcome in different clinical phenotypes of antiphospholipid syndrome (APS). Women with thrombotic APS have higher rates of pregnancy complications than those with obstetric APS. (SageJournals) K. Bramham. Lupus, Vol. 19, No. 1, 58-64 (2010).

Autoimmune Disorder Linked to Stroke, Heart Attack in Women Smoking, birth control pills raise risk higher, research finds. Women under 50 with a certain form of the autoimmune condition called antiphospholipid syndrome (APS) are at greatly increased risk for heart attack and stroke, and that risk is even higher when these women smoke or take birth control pills, new research shows. Robert Preidt (HealthDay News) MSN. 09/28/09.

Antiphospholipid Antibody Syndrome and Pregnancy. Recognized for its associated thrombosis in the 1950s, antiphospholipid syndrome (APS) and lupus anticoagulant (LAC) are associated with pregnancy complications that include fetal loss, fetal growth restriction, preeclampsia, thrombosis, and autoimmune thrombocytopenia. Teresa Berg. MD. eMedicine. 12/01/09.

Catastrophic Antiphospholipid Syndrome (CAPS) and Sepsis. A Common Link? Catastrophic APS resembles severe sepsis in its acute presentation. Iinfections are the best known triggers of CAPS. This emphasizes the need for early diagnosis and aggressive antibiotic treatment as soon as infection is suspected in both classic APS and CAPS patients. The Journal of Rheumatology. Editorial. VOL 34: NO. 5 MAY 2007.

Systemic antiphospholipid syndrome and atherosclerosis (AT). AT is one of the main features of systemic APS and offer opportunities for new treatment strategies. Jara JA. Clin Rev Allergy Immunol. 2007 Apr;32(2):172-7. (PubMed)

Sudden sensorineural hearing loss in a patient with primary antiphospholipid syndrome. Consensus exists on the effectiveness of anticoagulant agents in aiding a favourable outcome of sudden sensorineural hearing loss in patients with antiphospholipid syndrome. Kang KT. J Laryngol Otol. 2008 Feb;122(2):204-6. (PubMed). (Also see: Ear Involvement)

Livedo Racemosa: A Striking Dermatological Sign for the Antiphospholipid Syndrome (APS). Livedo racemosa is the most common dermatologic manifestation of APS, frequently associated with cerebrovascular events, arterial thrombosis, and pregnancy morbidity, and considered an independent, additive, thrombotic risk factor. J Rheumatol 2006 December;33:2379. Editorial.

Improving Management of Pregnancy in Antiphospholipid Antibody-Positive Women. One or more of the manifestations of aPL - early abortion, fetal death, and fetal growth restriction - occur in as many as 20% of women carrying the lupus anticoagulant (LAC) and/or anticardiolipin antibodies (aCL)1. J Rheumatol 2006 November;33:1925. Editorial.

Treatments for Antiphospholipid Syndrome

Recent trends in the management of antiphospholipid syndrome (Hughes syndrome). At present, patients with thrombosis are treated with long-term oral anticoagulation as standard. The approach for women with obstetric manifestations is based on the use of aspirin plus heparin. However, the knowledge of new pathogenic mechanisms might identify novel therapeutic targets and, therefore, may improve the management of these patients. Espinosa G. Drugs Today (Barc). 2010 Jan;46(1):39-47. (PubMed).

Comparison between single antiplatelet therapy and combination of antiplatelet and anticoagulation therapy for secondary prevention in ischemic stroke patients with antiphospholipid syndrome. The recent study did not show any difference in effectiveness for secondary prevention between single antiplatelet (aspirin) and single anticoagulant (warfarin) therapy. Our results indicate that combination therapy may be more effective in APS-related ischemic stroke. Okuma H. Int J Med Sci. 2009 Dec 5;7(1):15-8. (PubMed)

Causes of Antiphospholipid Syndrome and APS Research

The Calcium-Sensing Receptor (CaSR) is a Target of Autoantibodies in Patients with Autoimmune Polyendocrine Syndrome Type 1 (APS1). The CaSR is an autoantigen in APS1, but detection of antibodies against the receptor appears to be influenced by the assay system used. The Journal of Clinical Endocrinology & Metabolism Vol. 92, No. 6 2107-2114.

Rare condition provides information about common illnesses. By working on APS-1 (Antiphospholipid Syndrome), one of the rarest autoimmune diseases, scientists hope that new knowledge about this rare disease can provide new insight into the entire phenomenon of autoimmunity. Nettavis for Universitetet i Bergen - lørdag 6. januar 2007.

Support Organizations for Antiphospholipid Syndrome

APS Foundation of America, Inc. APS is an acronym for Antiphospholipid Antibody Syndrome. APS is also called APLS or APLA in the United States and Hughes Syndrome or Sticky Blood in the UK. APFSA.

Personal Stories about Antiphospholipid Syndrome

Hailee Vale: MCTD but Now Undiagnosed I still do not have any proper answers. I have my own theories as to what went wrong and I am waiting to see a rheumatologist...

Papa V: Lupus, Antiphospholipid Syndrome, Myasthenia Gravis, and Scleroderma About thirty years passed, and then—BAM !—I got a heart blockage, stroke, liver biopsy due to elevated enzymes, skin rash, high blood pressure, very high ANA... .

Media Stories about Antiphospholipid Syndrome

Miscarriages May Be A Symptom Of Greater Health Risk. Autoimmune Diseases, such as lupus or thyroid conditions, are associated with RPL (Recurrent Pregnancy Loss) and infertility. If you have a history of autoimmune disease and unexplained RPL, ask about these potentially appropriate tests: ACA/APA, LAC (Lupus Anti-coagulant). ElitesTV. 07/19/05. (Also see: Lupus, and Thyroid Disease)