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Antiphospholipid Syndrome (APS)

Author: Shelley Ensz. Scleroderma is highly variable. See Types of Scleroderma. Read Disclaimer
Overview
APS and Heart Disease
Scleroderma and APS
Diagnosis
Symptoms
Complications
Treatments
Causes and Research
Support Resources
Personal Stories

What is Antiphospholipid Syndrome?

Antiphospholipid Syndrome is also known as Hughes Syndrome. (Also see: Autoimmune Diseases)
The antiphospholipid syndrome (Hughes' syndrome) NetDoctor.

Antiphospholipid Syndrome and Heart Disease

The autoimmunity conundrum: clotting or inflammation. Vigilance is required to establish if there is an underlying rheumatological condition in a patient who presents with presumptive infective endocarditis in the absence of risk factors. Treatment for systemic lupus erythematosus and primary APS (Antiphospholipid syndrome) are distinct. PubMed, Int J Rheum Dis. 2010 Oct;13(4):e62-6.

Scleroderma and Antiphospholipid Syndrome

Systemic sclerosis (SSc) increases the risks of deep vein thrombosis (DVT) and pulmonary thromboembolism (PTE): a nationwide cohort study. SSc patients exhibited a significantly higher risk of developing DVT and PTE compared with the general population and thus multidisciplinary teams should guide the assessment, treatment and holistic care of SSc patients. PubMed, Rheumatology (Oxford). 2014 Apr 8.
The association of antiphospholipid antibodies (APLA) with cardiopulmonary manifestations of systemic sclerosis. APLA is associated with pulmonary arterial hypertension (PH), interstitial lung disease, Raynaud's phenomenon and digital ulcers, so endothelial abnormalities and small vessel thrombosis may be important in the pathogenesis of these disease features. PubMed, Clin Exp Rheumatol, 2014 Feb 24. (Also see: Pulmonary Involvement, Raynaud's and Digital Ulcers)
Systemic sclerosis sine scleroderma associated with antiphospholipid syndrome (APS). Cases described in medical literature from 1966-2009 associate the presence of antiphospholipid antibodies with the presence of APS and systemic sclerosis (SS). We describe the first case of a patient with APS associated with systemic sclerosis sine scleroderma. Rheumatol Int. 2012 Oct;32(10):3265-8. (Also see: Types of Scleroderma: Sine)

Diagnosis of Antiphospholipid Syndrome

Diagnosis and classification of the antiphospholipid syndrome (APS). At least one clinical (vascular thrombosis or pregnancy morbidity) and one laboratory (anticardiolipin antibodies, lupus anticoagulant or anti-β2-glycoprotein I antibodies) criterion had to be met for the classification of APS. PubMed, J Autoimmun. 2014 Jan 22.

Symptoms of Antiphospholipid Syndrome

Renal involvement in antiphospholipid syndrome (APS). This article reviews the range of renal abnormalities associated with APS, and their diagnosis and treatment options. PubMed, Curr Rheumatol Rep, 2014 Feb;16(2):397.
Cognitive dysfunction in antiphospholipid antibody (aPL)-negative systemic lupus erythematosus (SLE) versus aPL-positive non-SLE patients. Both aPL-negative SLE and aPL-positive non-SLE patients, without other overt neuropsychiatric disease, demonstrated high levels of cognitive impairment. PubMed, Clin Exp Rheumatol, 09/10/2013. (Also see: Systemic Lupus Erythematosus)
The Prevalence of Iron Deficiency Anemia (IDA) in Primary Antiphospholipid Syndrome (PAPS). PAPS patients have a higher incidence of IDA and Iron deficient erythropoiesis (IDE) compared to healthy controls. This can be attributed to inadequate ingestion of folic acid and vitamin C. Turkish Journal of Rheumatology. (Also see: Anemia of Chronic Disease)
Antiphospholipid syndrome in obstetrics. The clinical hallmarks of this syndrome are thrombosis and poor obstetric outcomes, including miscarriages, fetal loss and severe pre-eclampsia. Best Pract Res Clin Obstet Gynaecol. 2011 Nov 10.
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

Pregnancy outcomes in patients with systemic autoimmunity. Results confirm that a strict follow-up and targeted treatments significantly improve pregnancy outcomes in autoimmune patients with primary antiphospholipid syndrome (PAPS), systemic lupus erythematosus (SLE) and isolated autoantibodies in the absence of a definite autoimmune disease. The pregnancy outcome in patients with APS/SLE remains unsatisfactory. Canti V, Autoimmunity. 2011 Aug 30 (Also see: Lupus, and Pregnancy and Autoimmunity)
Risk factors for pregnancy failure in patients with anti-phospholipid syndrome treated with conventional therapies: a multicentre, case-control study. APS patients diagnosed on the basis of a single positive test and/or history of pregnancy morbidity alone were generally found to have successful pregnancies. Conclusion. It would seem from these findings that the risk of pregnancy failure in APS women planning to conceive can be stratified on the basis of some specific clinical and laboratory features. Ruffatti A. Rheumatology (Oxford). 2011 Jun 7.
Pulmonary thromboembolism as the initial manifestation in a child with antiphospholipid syndrome in the emergency department. Antiphospholipid syndrome (APS) should also be kept as a possibility in children presenting for the first time with pulmonary thromboembolism in the emergency department. Bhat MA. Pediatr Emerg Care. 2011 Mar;27(3):205-7.
(Case Report) Sudden sensorineural hearing loss in a patient with primary antiphospholipid syndrome. J Laryngol Otol. (PubMed). (Also see: Ear Involvement)
Livedo Racemosa: A Striking Dermatological Sign for the Antiphospholipid Syndrome (APS). J Rheumatol. Editorial.

Treatments

Perioperative management of antiphospholipid antibody(aPL)-positive patients. There are limited data on which to base recommendations and periods without anticoagulation should be kept to an absolute minimum for patients with a history of thrombosis. PubMed, Curr Rheumatol Rep. 2014 Jul;16(7):426.
Efficacy of aspirin for the primary prevention of thrombosis in patients with antiphospholipid antibodies (aPL). This meta-analysis shows that the risk of first thrombotic event is significantly decreased by low dose aspirin among asymptomatic aPL individuals, patients with systemic lupus erythematosus or obstetrical antiphospholipid syndrome. PubMed, Autoimmun Rev, 2013 Nov 2.
Use of new oral anticoagulants in antiphospholipid syndrome. The use of warfarin is problematic, particularly in patients with antiphospholipid syndrome (APS), where prospective clinical studies of new oral anticoagulants (NOAC) are the way forward. PubMed, Curr Rheumatol Rep, 2013 Jun;15(6):331.
Hydroxychloroquine Use Is Associated with Lower Odds of Persistently Positive Antiphospholipid Antibodies and/or Lupus Anticoagulant in Systemic Lupus Erythematosus. This is the first study to show that hydroxychloroquine (plaquenil, HCQ) use is associated with lower odds of having persistently positive lupus anticoagulant (LAC) and/or antiphospholipid antibodies. (aPL). The Journal of Rheumatology, Vol. 40, No. 1 30-33, August 1, 2012. (Also see: Lupus Treatments, and Antimalarials)
Intensity and Duration of Anticoagulation Therapy in Antiphospholipid Syndrome. A recent consensus document recommends warfarin to an INR range of 2.0 to 3.0 for patients with a first venous thromboembolic event. Higher anticoagulation intensity is recommended for patients presenting with arterial events. Semin Thromb Hemost, 2012 Mar 30.

Causes and APS Research

Cigarette smoking, antiphospholipid antibodies (aPL) and vascular events (VE) in Systemic Lupus Erythematosus (SLE). Among ever regular smokers who were aPL positive, there was a strikingly high frequency of former VE and the underlying mechanisms and temporality between smoking, aPL and VE need further investigations. PubMed, Ann Rheum Dis, 2014 Apr 1. (Also see: Antibodies in Systemic Lupus Erythematosus)
Gene profiling reveals specific molecular pathways in the pathogenesis of atherosclerosis and cardiovascular disease in antiphospholipid syndrome (APS), systemic lupus erythematosus (SLE) and antiphospholipid syndrome with lupus (APS-SLE). Specific gene signatures explain the pro-atherosclerotic and pro-thrombotic alterations in these highly related autoimmune diseases. PubMed, Ann Rheum Dis, 2014 Mar 11. (Also see: Systemic Lupus Erythematosus)
New Tests to Detect Antiphospholipid Antibodies: Anti-Domain I Beta-2-Glycoprotein-I Antibodies. Anti–DI antibodies are strongly positively correlated with thrombotic and pregnancy manifestations, enabling identification of patients at higher risk of clinical events. Springer Link, January 2014.
Distinct antibody profile: a clue to primary antiphospholipid syndrome (PAPS) evolving into systemic lupus erythematosus (SLE)? The detection of a distinct subgroup of lupus-associated autoantibody in PAPS patients seems to be a hint to overt SLE disease, particularly in those patients with young age at diagnosis. PubMed, Clin Rheumatol, 2014 Jan 14. (Also see: Systemic Lupus Erythematosus)
Vaccine model of antiphospholipid syndrome induced by tetanus vaccine. Successful induction of antiphospholipid syndrome (APS) in two different non-autoimmune prone mouse strains, was achieved by tetanus toxoid hyperimmunization. PubMed. Lupus. 2012;21(2):195-202.

Support Organizations

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

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... .
 
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