Antiphospholipid Syndrome (APS)

Associated Conditions: MAIN MENU

Autoimmune Diseases:
Antiphospholipid Syndrome (APS) or Hughes Syndrome

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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 ALARCÓN-SEGOVIA, MD, MS, PhD. J Rheumatol. VOLUME 30: NO. 9 SEPTEMBER 2003. (Also see: Lupus)

Medical Edge: Accurate diagnosis relies on second test. Antiphospholipid syndrome (APS) can be diagnosed with a blood test repeated 6-12 weeks apart to compare results. PostBulletin 02/08/08.

NIH Launches Clinical Studies Nationwide to Investigate Rare Diseases. 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. National Institutes of Health News. 05/05/06. (Also see: Vasculitis)

Antiphospholipid Antibodies Syndrome (APS):From Benchtop to Bedside. APS is defined as a clinical entity where recurrent arterial or venous thrombotic events and fetal losses are associated with antiphospholipid antibodies (aPL), namely lupus anticoagulant (LA) or anticardiolipin antibodies (aCL). R. Levy. SP0036 EULAR 2003.

The antiphospholipid syndrome (Hughes' syndrome) NetDoctor.

Peripheral vascular disease in antiphospholipid syndrome. Less than 10% of APS patients express premature atherosclerosis in the absence of other risk factors. Which APS patient will develop atherosclerosis is unpredictable. PubMed. Thromb Res. 2004;114(5-6):509-19.

Antiphospholipid Syndrome and Heart Disease

Cardiac involvement in the antiphospholipid syndrome. Antiphospholipid syndrome (APS) is a systemic autoimmune disease, associated with a hypercoagulable state and fetal loss and with other clinical manifestations including cardiac involvement. PubMed. Lupus. 2005;14(9):691-6.

Systemic autoimmune diseases form a diverse group which includes: systemic lupus erythematosus (SLE), mixed connective tissue disease (MCTD), scleroderma, dermato-polymyositis, Wegener's granulomatosis, Sjogren syndrome. Clinical evidence of cardiac abnormalities has been observed, in as many as 58% of cases with positive echocardiographic findings. The frequency and extent of cardiac pathology positively correlated with the detection of antiphospholipid antibodies. No such relationship was observed in patients with the presence of very high titers of antinuclear antibodies. PubMed. Pol Arch Med Wewn. 2003 Apr;109(4):375-81.

Scleroderma and Antiphospholipid Syndrome

Antiphospholipid Antibodies (APLA) in Systemic Sclerosis. APLA are commonly found in SS without the clinical manifestations of antiphospholipid syndrome. APLA are correlated with PH ,and may be considered as markers of vascular causes of pulmonary manifestations in SS. S. Tanaseanu. SAT0244 EULAR 2006. (Also see: Pulmonary Hypertension)

Prevalence of antiphospholipid antibodies in systemic sclerosis and association with primitive pulmonary arterial hypertension and endothelial injury. We found that the prevalence of antiphospholipid antibodies in SSc patients was low. However, aCL antibodies were associated with PAH and endothelial injury. PubMed. Clin Exp Rheumatol. 2005 Mar-Apr;23(2):199-204. (Also see: Pulmonary Hypertension)

Low Prevalence of Antiphospholipid Antibodies in Systemic Sclerosis (SSc) and Association with Pulmonary Arterial Hypertension. APL antibodies have a low prevalence in SSc with few thromboembolic events but are significantly associated with pulmonary arterial hypertension. N. Assous. FRI0298 EULAR 2004. (Also see: Pulmonary Hypertension)

Antiphospholipid antibody in localised scleroderma. These results suggest that aCL (antibodies against cardiolipin) and LAC (lupus anticoagulant) are the major autoantibodies in patients with generalised morphoea. PubMed. Ann Rheum Dis. 2003 Aug;62(8):771-4. (Also see Antibodies and Generalized Morphea)

Identification of masqueraders of autoimmune disease in the office. There are several rheumatologic and autoimmune disorders that can masquerade as allergic disease. These conditions include rheumatoid and juvenile arthritis, Sjogren's syndrome, systemic lupus erythematosus, Behcet's and antiphospholipid syndromes, systemic sclerosis, vasculitis, sarcoidosis, chronic fatigue syndrome, and fibromyalgia. PubMed. Allergy Asthma Proc. 2003 Nov-Dec;24(6):421-9. (Also see: Rheumatoid Arthritis, Sjogren's Syndrome, Lupus, Behcet's, Systemic Sclerosis (Scleroderma), Vasculitis, Sarcoiditis, Fibromyalgia and CFS)

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)

"Listen to the Patient" Anticoagulation Is Critical in the Antiphospholipid (Hughes) Syndrome. Affecting, almost uniquely, both veins and arteries, the clinical features range through deep vein thrombosis (DVT), chronic leg ulcers, recurrent miscarriages, headache, heart attacks, renal vein and artery thrombosis, to pulmonary embolism and even pulmonary hypertension. The Journal of Rheumatology VOLUME 30: NO. 4 APRIL 2003. (Also see: Leg Ulcers, Cardiac, Pulmonary Hypertension)

Complications of Antiphospholipid Syndrome

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.

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.

Antiphospholipid syndrome and pregnancy. Early treatment combined with close maternal-fetal surveillance was associated with a 90% chance of a live birth rate. However, prematurity, preeclampsia and intrauterine growth restriction were common. PubMed. Ginecol Obstet Mex. 2006 Jul;74(7):367-75.

The systemic nature of the antiphospholipid syndrome. Antiphospholipid syndrome (APS, Hughes' syndrome) is a systemic autoimmune disorder characterized by arterial and/or venous thrombosis and recurrent foetal loss, accompanied by mild to moderate thrombocytopaenia and elevated titres of antiphospholipid antibodies: lupus anticoagulant and/or anticardiolipin antibodies. PubMed. Scand J Rheumatol. 2004;33(6):365-72.

Antiphospholipid antibodies in patients with sensorineural hearing loss. Our data suggest that antibodies against beta(2)-glycoprotein seem to coincidence with an acute event, such as sudden sensorineural hearing loss, whereas antibodies against phosphatidylserine IgG are detectable in the prolonged sequel, such as in patients with progressive sensorineural hearing loss and normal tension glaucoma. PubMed. Eur Arch Otorhinolaryngol. 2005 Feb 25. (Also see: Ear Involvement, and Eye Involvement)

The antiphospholipid antibody syndrome (APLS) is multisystem, autoimmune disease, which is characterized by: thrombosis, obstetrics complications and thrombocytopenia. The two most clinically significant antiphospholipid antibodies (APLa) that are associated with recurrent pregnancy loss and thrombosis are anticardiolipin antibodies (ACL) and lupus anticoagulant (LA). Maternal treatment and careful monitoring of fetal well-being are mandatory in the management of these high-risk pregnancies. PubMed. Akush Ginekol (Sofiia). 2004;43(1):36-42.

Classification Criteria for Antiphospholipid Syndrome: The Case for Cardiac Valvular Disease. Cardiac valve disease should now be included in classification criteria for APS. However, its pathogenesis may not be primarily thrombotic, nor should its initial treatment be limited to anticoagulation. Editorial. J Rheumatol. Volume 31: No. 12 December 2004.

Features Associated with Epilepsy in the Antiphospholipid Syndrome (APS). Epilepsy is common in APS and most of the risk seems to be linked to vascular disease as manifested by extensive central nervous system (CNS) involvement, valvulopathy, and livedo reticularis and to the presence of systemic lupus erythematosus (SLE). These factors, however, explain only part of the increased occurrence of epilepsy in APS and other causes such as direct immune interaction in the brain should be investigated. J Rheumatol. VOLUME 31: NO. 7 JULY 2004;31:1344-8. (Also see: Lupus)

Antiphospholipid Antibodies Not Predictive of Future Thrombo-Occlusive Events After Ischaemic Stroke. The presence of either anticardiolipin antibodies (aCL) or lupus anticoagulant antibodies (LA) at the time of an ischaemic stroke does not predict an increased risk for thrombo-occlusive events during the following 2 years, according to data from the Antiphospholipid Antibodies and Stroke Study (APASS). Doctor's Guide. 02/05/04 JAMA 2004;291:576-584.

Ovarian Vein Thrombosis May Occur in Antiphospholipid Syndrome, May Be Underdiagnosed. Based on these cases that show that OVT can occur in patients with APS, with or without pregnancy, the authors conclude that "given that pelvic pain or tenderness is far from constant, this complication may be underdiagnosed." Doctor's Guide. 02/03/04 Arthritis Rheum 2004 Jan;50:1:183-6.

Antiphospholipid antibody syndrome complicated by Grave's disease. There is a close relationship between the cutaneous manifestation of antiphospholipid antibody syndrome and the activities of Grave's disease and a possible link of antiphospholipid antibody syndrome with Grave's disease was suggested both by the etiology of the disease as well as the disease activity. PubMed. J Dermatol. 2002 Dec;29(12):776-80. (Also see: Grave's Disease)

Features Associated with Epilepsy in the Antiphospholipid Syndrome. Epilepsy is common in APS and most of the risk seems to be linked to vascular disease as manifested by extensive CNS involvement, valvulopathy, and livedo reticularis and to the presence of SLE. These factors, however, explain only part of the increased occurrence of epilepsy in APS and other causes such as direct immune interaction in the brain should be investigated. J Rheumatol. VOLUME 31: NO. 7 JULY 2004;31:1344-8. (Also see:Lupus)

The Presence of Multiple Prothrombotic Risk Factors Is Associated with a Higher Risk of Thrombosis in Individuals with Anticardiolipin Antibodies. In individuals with positive aCL-IgG, we observed an association between the number of prothrombotic risk factors and history of thrombotic events. J Rheumatol. Volume 30: No. 11 November 2003;30:2385-91.

Systemic autoimmune diseases form a diverse group which includes: systemic lupus erythematosus (SLE), mixed connective tissue disease (MCTD), scleroderma, dermato-polymyositis, Wegener's granulomatosis, Sjogren syndrome. The frequency and extent of cardiac pathology positively correlated with the detection of antiphospholipid antibodies. No such relationship was observed in patients with the presence of very high titers of antinuclear antibodies (anti-dsDNA). PubMed. Pol Arch Med Wewn. 2003 Apr;109(4):375-81. (Also see: Cardiac Involvement)

Treatments for Antiphospholipid Syndrome

Warfarin in Antiphospholipid Syndrome — Time to Explore New Horizons. Despite the effectiveness of warfarin in secondary thrombosis prevention in APS, warfarin use is cumbersome for both patients and physicians due to significant bleeding complications and the need for frequent blood monitoring. J. Rheumatol. Vol. 32 No. 2 February 2005. Editorial.

Protective Effects of Heparin in Preventing Miscarriages in Lupus Patients. The investigators found that heparin works not as an anticoagulant but instead by blocking activation of the complement pathway, a series of inflammatory proteins that the investigative team previously found to play an essential role in pregnancy loss and placental injury. newswise 10-18-04. (Also see: Lupus)

A comparison of two intensities of warfarin for the prevention of recurrent thrombosis in patients with the antiphospholipid antibody syndrome. High-intensity warfarin was not superior to moderate-intensity warfarin for thromboprophylaxis in patients with antiphospholipid antibodies and previous thrombosis. PubMed. N Engl J Med. 2003 Sep 18;349(12):1133-8.

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.

Autoantibodies Against C-Reactive Protein: Clinical Associations in Systemic Lupus Erythematosus and Primary Antiphospholipid Syndrome. We observed that the presence of these antibodies was associated with lupus nephritis and with clinical features of the APS in patients with lupus and non-lupus patients. J Rheumatol 2006;33:1980-6. (Also see: Lupus and Antibodies)

B cell biology, apoptosis, and autoantibodies to phospholipids. In some diseases, including anti-phospholipid syndrome, autoantibodies can be directly pathogenic. Understanding how these autoantibodies are formed represents an important avenue towards understanding the pathogenesis of systemic autoimmune disease itself. PubMed. Thromb Res. 2004;114(5-6):307-19.

Anticardiolipin Test and the Antiphospholipid (Hughes) Syndrome: 20 Years and Counting! It has been 20 years since The Lancet published the first report on the anticardiolipin (aCL) test that identified a group of patients subject to recurrent arterial and/or venous thromboses and recurrent pregnancy losses (RPL). It spawned a wave of interest in what came to be known as the antiphospholipid syndrome (APS). Editorial. J Rheumatol. Volume 31: No. 11 November 2004.

Birth Order and Maternal Microchimerism in Pediatric SLE. More than half of the patients with SLE were not firstborn which is in support of the hypothesis of increased risk for SLE (although this cohort only contained 1 SLE sib-pair). There was a high percentage of firstborn patients with antiphospholipid abs in the first cohort group and there is a chance that their mothers may also have had antiphospholipid abs thus conferring risk for fetal loss. Christine B. Bernal. ACR Conference Oct. 2003. (Also see: Fetal Cells and Scleroderma and Lupus)

The antiphospholipid syndrome and atherosclerosis: clue to pathogenesis. Disturbance of blood vessel homeostasis in this disorder may increase risk for atherosclerosis by mechanisms that are direct (through antibody targeting of blood vessel-regulating proteins) or indirect (via inflammatory mechanisms that have recently been implicated in autoantibody-mediated thrombosis). PubMed. Curr Rheumatol Rep. 2003 Oct;5(5):401-6.

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)