What is antiphospholipid syndrome?

Abstract

The description of antiphospholipid syndrome (APS) is vascular thrombosis and/or pregnancy morbidity occurring in persons with antiphospholipid antibodies (aPL). However, because of an unclear etiopathogenesis and the heterogeneous nature of aPL and of aPL-related clinical manifestations, a single, unambiguous definition of APS does not exist. In this paper, we describe a structured approach to APS, discuss the controversies, and offer descriptions of APS that include: an autoimmune systemic disease with a spectrum of (mostly thrombotic) clinical manifestations, an overdiagnosed disease when aPL is present but has no proven direct causative role in the clinical manifestations, and a disease of controversies that requires clarification of etiology and mechanisms and risk-stratified controlled clinical studies.

Antiphospholipid syndrome: interpretation of laboratory data

“…Most APS patients with only IgG aCL positivity that are diagnosed with APS have a titre below 40 GPL and are affected by pregnancy morbidity [4]. It may well be that obstetric and vascular APS are different clinical entities and the mechanism(s) of placental injury is distinct from those that determine vascular thrombosis [5,6]. If these results are confirmed, patients with thromboembolic events and only low titre aCL should be carefully reassessed for other causes of thrombosis.…”

Case studies in anticoagulation management

“…[1,5,17]. For patients like MD, elevated and unstable INR results and challenging warfarin dosing are expected with antiphospholipid syndrome patients [51][52][53][54][55][56][57][58][59][60]. Case reports and one clinical trial have used oral vitamin K doses ranging from 80-500 lg/day [46][47][48][49][50]61].…”

Antiphospholipid antibodies in rheumatoid arthritis: Identifying the dominoes

“…Observational studies suggest that patients with aPL and no prior thrombosis do not warrant primary antithrombotic prophylaxis [37]. In the multicenter, randomized, double-blind, placebocontrolled Antiphospholipid Antibody Acetylsalicylic Acid (APLASA) study of 98 patients, Erkan et al [36•] evaluated the effi cacy of 81 mg/d of aspirin in preventing a primary thrombotic occurrence in 48 persistently aPL-positive (LA or aCL IgG/IgM/IgA isotype-positive) adults without a prior history of thrombotic or pregnancy events.…”

“…Erkan and Lockshin [37] reported on the ability of hydroxychloroquine to inhibit platelet aggregation, decrease aCL titers, decrease thrombotic events in SLE patients, and decrease thrombus size in aPL-injected mice. The effi cacy of hydroxychloroquine in preventing thrombosis has not been described in an aPL-positive RA patient.…”