Autoimmune Diseases

Antiphospholipid Syndrome (APS)

Overview: What is Antiphospholipid Syndrome?

Antiphospholipid 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

Prevalence of antiphospholipid antibodies in patients with overt myocardial dysfunction in systemic lupus erythematosus. A case-control study. This study did not find any significant association of anti-phospholipid antibodies with overt myocardial dysfunction in patients of SLE. PubMed, Lupus, 2020 Aug 4;961203320947784. (Also see Symptoms and Complications of Lupus)

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

Systemic Sclerosis (SSc) Is an Independent Risk Factor for Ischemic Heart Disease (IHD), Especially in Patients Carrying Certain Antiphospholipid Antibodies. Patients with SSc are at higher risk for developing IHD with an additional risk for the latter in those positive for anti-cardiolipin or anti-beta2GPI. PubMed, Eur J Intern Med, 06/30/2020. (Also see Cardiac (Heart) Involvement)

Prevalence and Clinical Associations of Antiphospholipid Antibodies (aPL) in Systemic Sclerosis (SSc): New Data From a French Cross–Sectional Study, Systematic Review, and Meta–Analysis. In our SSc population, aPL positivity was associated with venous thrombosis and miscarriage and these data provide additional insights into the role of aPL in the vasculopathy observed in SSc. PubMed, Front Immunol, 2018 Nov 2;9:2457.

Prevalence and Clinical Associations of Antiphospholipid Antibodies in Systemic Sclerosis: New Data From a French Cross-Sectional Study, Systematic Review, and Meta-Analysis. These data provide additional insights into the role of aPL in the vasculopathy observed in SSc. PubMed, Front Immunol, 2018 Nov 2;9:2457. (Also see Causes of Pulmonary Hypertension, 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

Prevalence of Antiphospholipid Antibodies (aPL) Negativisation in Patients with Antiphospholipid Syndrome: A Long-Term Follow-Up Multicentre Study. aPL negativisation occurred in approximately 9% of patients and negativisation occurred most often in patients who were previously found to be positive for only one aPL. PubMed, Thromb Haemost, 10/06/2019.

Clinical profiles and risk assessment in patients with antiphospholipid antibodies (aPL). Further studies, particularly prospective randomized controlled trials, are highly warranted to establish an effective and tolerable treatment regimen for high risk aPL carriers. PubMed, Expert Rev Clin Immunol, 11/01/2018.

Galectin-9 is an easy to measure biomarker for the interferon signature in systemic lupus erythematosus (SLE) and antiphospholipid syndrome (APS). Galectin-9 is a novel, easy to measure hence clinically applicable biomarker to detect the IFN signature in patients with systemic autoimmune diseases such as SLE and APS. PubMed, Ann Rheum Dis, 09/05/2018. (Also see Diagnosis of Lupus)

Symptoms of Antiphospholipid Syndrome

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

Prevention of recurrent miscarriage (RM) in women with antiphospholipid syndrome (APS): A systematic review and network meta-analysis. Our findings support the use of low-dose aspirin plus heparin as the first-line treatment for prevention of RM in women with APS, and support the efficacy of hydroxychloroquine, IVIG, and prednisone when added to current treatment regimens. PubMed, Lupus, 2020 Oct 20;961203320967097.

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. (Also see Antibodies in Lupus)

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

Clinical Insights into Diffuse Alveolar Hemorrhage (DAH) in Antiphospholipid Syndrome (APS). DAH is a rare but devastating manifestation of APS patients with or without other systemic autoimmune diseases. PubMed, Curr Rheumatol Rep, 2019 Sep 6;21(10):56.

Anti-D4GDI antibodies activate platelets in vitro: a possible link with thrombocytopenia in primary antiphospholipid syndrome. We show here that antibodies anti-D4GDI are present in the sera of PAPS patients and can prime platelet activation, explaining, the pro-thrombotic state and the thrombocytopenia of PAPS patients. PubMed, Arthritis Res Ther, 2019 Jul 1;21(1):161.

Obstetric outcomes in patients with primary thrombotic and obstetric antiphospholipid syndrome and its relation to the antiphospholipid antibody profile. Patients with primary antiphospholipid syndrome, especially those with obstetric antiphospholipid syndrome and triple antibody positivity, are at higher risk for adverse pregnancy outcomes. PubMed, Lupus, 2019 May 27:961203319852155.

Two subgroups in systemic lupus erythematosus (SLE) with features of antiphospholipid (APS) or Sjögren's syndrome (SS) differ in molecular signatures and treatment perspectives. Stratifying SLE patients based on an autoantibody profile could be a way forward to understand underlying pathophysiology and to improve selection of patients for clinical trials of targeted treatments. PubMed, Arthritis Res Ther, 2019 Feb 18;21(1):62. (Also see Lupus in Overlap and Sjögren's Syndrome)

Apoptosis in patients with primary antiphospholipid antibody syndrome (pAPS). There was a trend toward elevated plasma levels of sTRAIL, IL-10 and TNF-a, but no evidence for dysregulated apoptosis in pAPS patients. PubMed, Int J Rheum Dis, 02/06/2019.

Clinical Phenotype of a First Unprovoked Acute Pulmonary Embolism (PE) Associated with Antiphospholipid Antibody Syndrome (APS). Patients with first unprovoked PE with hemoptysis and have a low pulmonary embolism severity index, should be suspected of having APS, and undergo laboratory testing for positive antiphospholipid antibodies. PubMed, Tuberc Respir Dis (Seoul), 2019 Jan;82(1):53-61.

Intrauterine fetal deaths (IUFD) related to antiphospholipid syndrome (APS): a descriptive study of 65 women. IUFD was most often the inaugural sign of APS and of the APS classification criteria, IUFD, preeclampsia, and thromboses were common in this cohort. PubMed, Arthritis Res Ther, 2018 Nov 6;20(1):249.

Antiphospholipid syndrome (APS) in obstetrics. This article will focus on the common manifestations of the pregnancy-related complications of APS. PubMed, Lupus, 2018 Oct;27(1_suppl):28-31. (Also see Pregnancy and Autoimmunity)

Case Report: Antiphospholipid syndrome (APS) in a patient with rheumatoid arthritis (RA). We report a case of APS manifesting as cutaneous ulceration and necrosis in a patient with severe RA. PubMed, Cutis. (Also see Symptoms and Complications of Rheumatoid Arthritis)

(Case Report) Sudden sensorineural hearing loss in a patient with primary antiphospholipid syndrome. J Laryngol Otol. (PubMed). (Also see Ear Involvement)

Treatments

A trial of antithrombotic therapy in patients with refractory migraine and antiphospholipid antibodies: A retrospective study of 75 patients. There was a high rate of symptomatic response to antithrombotic therapy in this context. PubMed, Lupus, 2021 Jan 5;961203320983913.

Treatment of Vascular Thrombosis in Antiphospholipid Syndrome: An Update. Early use of a combined triple therapy such as anticoagulation, plasma exchange, and steroids with either or not addition of immunoglobulins is important to reduce mortality. PubMed, Hamostaseologie, 2020 Feb;40(1):31-37. (Also see Therapeutic Plasma Exchange)

Treatment Failures of Direct Oral Anticoagulants (DOACs). Our analysis illustrates that DOACs may fail in the setting of Food and Drug Administration and non-Food and Drug Administration- approved indications. PubMed, Am J Ther, 10/09/2019.

HIBISCUS: Hydroxychloroquine for the secondary prevention of thrombotic and obstetrical events in primary antiphospholipid syndrome. Hydroxychloroquine (HCQ) appears as an additional therapy, as it possesses immunomodulatory and anti-thrombotic various effects. PubMed, Autoimmun Rev, 2018 Dec;17(12):1153-1168. (Also see Treatments for Lupus and Antimalarials)

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.

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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