THE PRIMARY ANTIPHOSPHOLIPID SYNDROME - ANTIPHOSPHOLIPID ANTIBODY PATTERN AND CLINICAL-FEATURES OF A SERIES OF 23 PATIENTS

Twenty-three patients with the 'primary' antiphospholipid syndrome were studied over 2-6 years. Twenty-two (96% had antiphospholipid antibodies detected by ELISA (87% had antibodies to thromboplastin and 70% to cardiolipin). and 18 out of the 21 tested patients (86% had lupus anticoagulant activity by coagulative assays. Mean age of the cohort was 29.9 years and the sex ratio (female: male) 4.75:1. Eleven patients presented 18 venous and/or arterial thrombosis and 13 had 25 foetal losses (84% occurred during the second and third trimester). Other clinical features were migraine, livedo reticularis, and epilepsy. Three patients had relatives with systemic lupus erythematosus. Thrombocytopaenia was seen in 33% anti-nuclear antibodies in low or moderate titre in 30% and haemolytic anaemia in 13% During the follow-up, two patients presented recurrent thrombosis despit anticoagulant therapy, one of them dying because of recurrent pulmonary thromboembolism. Four patients achieved successful term pregnancies after treatment with aspirin and a further patient after treatment with aspirin and low dose prednisolone. No patient developed systemic lupus erythematosus or any other definable connective tissue disease. The 'primary' antiphospholipid syndrome may exist as a distinct clinical entity and all younger patients presenting with thrombotic events, foetal losses and/or thrombocytopaenia, without any evidence of a well defined disease, should be tested for antiphospholipid antibodies in order to rule out this syndrome. © 1991 Informa UK Ltd All rights reserved: reproduction in whole or part not permitted.