Primary antiphospholipid syndrome evolving into systemic lupus erythematosus

Abstract

A young woman had a history of spontaneous venous thromboembolic disease which recurred on several occasions after cessation of treatment with oral anticoagulants. The presence of antiphospholipid antibodies (lupus anticoagulant and a high titre of IgG class anticardiolipin antibodies) in the absence of other clinical and serological features of sys temic lupus erythematosus (SLE) confirmed a diagnosis of primary antiphospholipid syn drome (PAPS). Antinuclear antibodies (ANA) were positive (1 :1280; speckled pattern). Twelve years after the first thrombotic episode she fulfilled criteria for the classification of SLE (antinuclear antibodies, platelet count < 100 x 109/l, anti-dsDNA antibodies, Coombs' positive haemolytic anaemia). She suffered a myocardial infarction while adequately anti coagulated and developed polyarthritis and immune complex-mediated nephritis over the next 3 years. This case history supports suggestions made by others that a strongly positive ANA test in a patient diagnosed with PAPS may be a harbinger for the development of SLE. Such evolution can take place over more than 10 years.