Antiphospholipid Syndrome: An Overview

Extract

This paper reviews antiphospholipid syndrome (APS), also known as Hughes syndrome, which is a potentially life-threatening autoimmune disorder where the body produces antibodies directed toward phospholipids and phospholipid-binding proteins. Diagnosis of this syndrome relies on both clinical and laboratory criteria. Laboratory testing used for diagnosing APS includes coagulation assays for the detection of lupus anticoagulant (LA) and enzyme-linked immunosorbent assay (ELISA) for antiphospholipid antibody (APL) detection.

APS, which was first described by Graham Hughes in 1983,¹ is an auto-immune disorder that is characterized by the presence of APL, arterial and/or venous thrombosis, and repeated pregnancy loss. The syndrome is categorized as primary or secondary based on whether it occurs alone (primary APS) or in the presence of other diseases (secondary APS), primarily autoimmune disorders such as systemic lupus erythematosus.² Patients with APS progress seldom to the catastrophic form of this disorder which is characterized by multiple organ infarcts transcending to multiple organ failure within days. The syndrome is more commonly noted in young to middle-aged adults and exhibits a female predominance.³ APL can occur in connective tissue disorders, infectious disease states such as syphilis and AIDS, and may be drug induced. They can also occur incidentally in healthy individuals and as a result, APL are considered clinically significant only when present in APS.⁴