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Primary antiphospholipid syndrome evolving into systemic lupus erythematosus
RHWM Derksen
Department of Internal Medicine F02.126 (Section Clinical Immunology and Infectious Diseases, University Hospital, PO Box 855000
FHJ Gmelig-Meijling
IDepartment of Immunology, University Hospital, PO Box 85500
PhG de Groot
Department of Haematology, University Hospital, PO Box 85500, 3508GA Utrecht, The Netherlands
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.
Key Words: anticardiolipin antibodies • lupus anticoagulant • antiphospholipid syndrome • sys temic lupus erythematosus
Lupus, Vol. 5, No. 1,
77-80 (1996)
DOI: 10.1177/096120339600500115
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