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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

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

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.

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. PubMed, Semin Arthritis Rheum, 2017 Apr;46(5):615-624. (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

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)

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.

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. (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

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-α, 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)

Length of exposure to antiphospholipid antibodies (aPL), rather than age, is a risk factor for thrombosis: a retrospective single–centre observational study. Unlike age, lack of thromboprophylaxis, prolonged aPL exposure and lupus anticoagulant may increase the risk of thrombosis in aPL carriers. PubMed, Rheumatol Int, 11/10/2017.

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)

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.

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

Treatments

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)

Catastrophic antiphospholipid syndrome: an update. Treatment strategy is based on the combination of anticoagulation, glucocorticoids, plasma exchange and/or intravenous immunoglobulins. PubMed, Panminerva Med, 2017 Sep;59(3):254-268. (Also see Therapeutic Plasma Exchange)

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)

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.

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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