PRIMARY ANTIPHOSPHOLIPID SYNDROME - CLINI CAL AND BIOLOGICAL STUDY OF36 CASES

BACKGROUND: The aim of this study was to analyze the clinical and biol ogical characteristics and follow up in a series of patients with the primary antiphospholipid syndrome. METHODS: Thirty-six patients were p rospectively 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 s yphilis 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 freque ntly observed clinical manifestations were miscarriage and fetal death in a total of 22 patients (61%). Thrombotic phenomenae were observed in 15 patients (42%) with predominance of venous thrombosis of the dee p venous system of the limbs and pulmonary embolism. Thrombocytopenia was detected in 9 cases (25%), autoimmune hemolytic anemia in 3 (8%) a nd 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 syst emic lupus erythematosus. The antiphospholipid antibody study demonstr ated the presence of anticardiolipin antibodies in 28 patients (78%), lupic anticoagulant in 22 (67%) (not determined in 3 patients) and fal sely positive test for syphilis in 5 (14%). Patients with thrombosis r eceived 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%. Four teen women wished to become pregnant following fetal loss and were tre ated 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 di fferentiated clinical entity and the presence of antiphospholipid anti bodies should be investagated in young people presenting thrombosis or fetal loss for no apparent reason.