[Primary antiphospholipid syndrome: clinical and biological study of 36 cases].

BACKGROUND: The aim of this study was to analyze the clinical and biological characteristics and follow up in a series of patients with the primary antiphospholipid syndrome.

METHODS: Thirty-six patients were prospectively studied. The antiphospholipid antibodies were determined with the following techniques: IgG and IgM anticardiolipin antibodies by ELISA, lupic anticoagulant by coagulometric tests and serology of syphilis by a reaginic test.

RESULTS: The mean age of the patients was of 29.9 years with a female/male relation of 4.1 to 1. The most frequently observed clinical manifestations were miscarriage and fetal death in a total of 22 patients (61%). Thrombotic phenomena were observed in 15 patients (42%) with predominance of venous thrombosis of the deep venous system of the limbs and pulmonary embolism. Thrombocytopenia was detected in 9 cases (25%), autoimmune hemolytic anemia in 3 (8%) and low or moderate titres of antinuclear antibodies in 12 (33%). Other infrequent clinical manifestations were cardiac valve lesions, livedo reticularis and epilepsy. Three patients (8%) had relatives with systemic lupus erythematosus. The antiphospholipid antibody study demonstrated the presence of anticardiolipin antibodies in 28 patients (78%), lupic anticoagulant in 22 (67%) (not determined in 3 patients) and falsely positive test for syphilis in 5 (14%). Patients with thrombosis received anticoagulant therapy, with all evolving favorably except two who died (multiple pulmonary thromboembolism in one case and cerebral hemorrhage in the other) mortality of the series thus being 5.6%. Fourteen women wished to become pregnant following fetal loss and were treated with acetylsalicylic acid in 3 cases together with prednisone (15 to 30 mg/day) with successful pregnancies being achieved in 12 cases (86%).

CONCLUSIONS: The primary antiphospholipid syndrome is a well differentiated clinical entity and the presence of antiphospholipid antibodies should be investigated in young people presenting thrombosis or fetal loss for no apparent reason.