Antiphospholipid Syndrome (APS)

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

Overview: What is Antiphospholipid Syndrome?

Antiphospholipid Syndrome KaleidoscopeAntiphospholipid Syndrome (APS) is also known as Hughes Syndrome. Bascially, it is an immune system abnormality that can cause the blood to overcoagulate (clot too much). Therefore it is often treated with anticoagulents (treatments that thin the blood.)

Antiphopholipid symptoms include anemia, blood clots, cognitive problems, eclampsia, heart attack, kidney problems, miscarriages, pulmonary embolism, thrombocytopenia, or stroke.

It is diagnosed by the presence of anticardiolipin, antiphosphatidlyserine, beta-2 glycoprotein I, or lupus anticoagulant antibodies plus symptom history, because it is possible to have such antibodies — or symptoms — without ever having or developing APS.

Infections can also cause a false-positive anticoagulent antibody test, so if it is positive, the test is often repeated three months later to confirm it. However, it is also possible for the antibodies to .(Also see Autoimmune Diseases)

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.

Scleroderma and Antiphospholipid Syndrome

Clinical relevance of antiphospholipid antibodies in systemic sclerosis (SSc): A systematic review & meta–analysis. A strong relationship exists between anticardiolipin antibodies and aß2GPI of IgG/IgM isotype and SSc; patients positive for these antibodies are more likely to suffer from pulmonary arterial hypertension, renal disease, thrombosis and digital infarction/ischaemia. Seminars in Arthritis and Rheumatism, 11/05/2016.

The risk of pulmonary embolism and deep venous thrombosis (VTE) in systemic sclerosis (SSc): A general population-based study. SSc patients are at a substantially increased risk of VTE and so increased monitoring for this potentially fatal outcome and its modifiable risk factors is warranted. PubMed, Arthritis Care & Research, 07/28/2015.

Antiphospholipid Antibody Levels High In Systemic Scleroderma (SSc) Patients. Patients with SSc commonly express antiphospholipid antibodies, although not all are affected by the antiphospholipid syndrome. Scleroderma News, 06/22/2015.

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)

(Case Report) 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. (Also see Types of Scleroderma: Sine)

Diagnosis of Antiphospholipid Syndrome

Comparison of different enzyme–linked immunosorbent assay methods for avidity determination of antiphospholipid antibodies. Both urea and sodium chloride may be used as chaotropic agents and reference values of avidity indices essential for interpretation of patients' results must be established individually for distinct assay conditions. PubMed, J Clin Lab Anal, 01/26/2017.

Antiphosphatidylserine antibodies(aPS) as diagnostic indicators of antiphospholipid syndrome (APS). Findings reveal a significant association between aPS and APS, especially when used to diagnosis clinical cases with other negative aPL tests and there is an independent association between aPS and primary APS. PubMed, Lupus, 2014 Sep 24.

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–ß2–glycoprotein I antibodies) criterion had to be met for the classification of APS. PubMed, J Autoimmun, 2014 Jan 22.

Symptoms of Antiphospholipid Syndrome

Antiphopholipid symptoms include anemia, blood clots, cognitive problems, eclampsia, heart attack, kidney problems, miscarriages, pulmonary embolism, thrombocytopenia, or stroke.

Antiphospholipid antibodies in patients with upper-extremity deep vein thrombosis (UEDVT). UEDVT could be the first clinical symptom of Antiphospholipid syndrome, and may be the first clinical manifestation of preceding the development of Systemic Lupus Erythematosus by several years. PubMed, Cent Eur J Immunol, 2015;40(3):307–310. (Also see Antibodies in Lupus)

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.

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)


Stillbirth: the impact of antiphospholipid syndrome (aPL)? Emerging evidence suggests that treatment with heparin agents and low dose aspirin to prevent fetal death is imperfect and new therapeutic approaches for patients with lupus anticoagulant or triple aPL positivity are needed. PubMed, Lupus, 10/03/2016.

Non–conventional antiphospholipid (APL) antibodies in patients with clinical obstetrical APS: prevalence and pregnancies treatment efficacy. In this study 68% of patients with clinical criteria for obstetrical antiphospholipid syndrome seronegative for conventional APL have non–conventional APL. Seminars in Arthritis and Rheumatism, 06/03/2016.

Musculoskeletal manifestations of the antiphospholipid syndrome (APS). Musculoskeletal complications are consistently reported in APS patients, not only causing morbidity and mortality, but also affecting their quality of life. PubMed, Lupus, 02/27/2016.

Non–stroke Central Neurologic Manifestations in Antiphospholipid Syndrome. Thrombotic manifestations of APS are well known, and various non–stroke neuro–psychiatric manifestations have also been consistently described, but their place in APS remains unclear. PubMed, Curr Rheumatol Rep, 2016 Feb;18(2):11.

Antiphospholipid Syndrome and Pregnancy. Antiphospholipid syndrome (APS) is an autoimmune disorder that is associated with pregnancy complications, including preeclampsia, thrombosis, autoimmune thrombocytopenia, fetal growth restriction, and fetal loss. Medscape, 04/15/2015. (Also see Pregnancy and Autoimmunity)

(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.


Rituximab usage in systemic lupus erythematosus (SLE)–associated antiphospholipid syndrome (APS): A single–center experience. In this single–center study there were no recurrent thrombotic events after the rituximab therapy, implicating further consideration of large–scale trials to evaluate its therapeutic role in SLE–associated APS patients. PubMed, Semin Arthritis Rheum, 2016 Aug;46(1):102-8. (Also see Treatments for Lupus)

Hydroxychloroquine (HCQ) as an anti–thrombotic in antiphospholipid syndrome. The mechanisms of action of HCQ, and clinical and experimental data in systemic lupus erythematosus (SLE) and APS are discussed. PubMed, Autoimmun Rev, 2015 Apr;14(4):358-62. (Also see Treatments for Lupus and Antimalarials)

FDA approves new treatment for varicose veins. The Food and Drug Administration has approved a new technique for treating varicose veins by sealing them with a clear liquid that turns into a solid adhesive. Washington Times, 02/20/2015.

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.

Causes and APS Research

Quality of life (QoL) in patients with (APS) antiphospholipid syndrome is related to disease burden and anticoagulant therapy. Health related QoL in primary APS was related to burden of the disease specifically at the vascular peripheral area and use of anticoagulants. PubMed, Int J Rheum Dis, 03/06/2017.

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)

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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