A LONGITUDINAL STUDY OF PATIENTS WITH SECONDARY ANTIPHOSPHOLIPID SYNDROME IN VASCULITIS

Background: Antiphospholipid antibodies (aPL) are a heterogeneous family of antibodies found in autoimmune disorders. It has been observed that the presence of different aPLs has been associated with various clinical manifestations.Objectives: The objective of this study is to perform a longitudinal follow-up of the patients with primary vasculitis and antiphospholipid antibodies (aPLs) marking out the multiple organ lesions.Methods: 18 patients with primary vasculitis (microscopic polyangiitis-3, classical polyarteritis- 2, Wegener's granulomatosis –4, Schonlein-Henoch purpura- 2, Takayasu's arteritis-2, giant cell arteritis- 2, Churg-Strauss syndrome-1, undifferentiated small vessel vasculitis-2) were taken into study. Secondary antiphospolipid syndrome (APS) was found in half of them. The serum levels of Ig G anticardiolipin antibodies (aCL) and the presence of lupus anticoagulant (LA) were assessed. Physical examinations, laboratory and imagistic investigations were performed.Results: Out of the 18 patients, 5 were aCL positive (aCL+), 2 patients were LA positive (LA+) and 2 patients were aCL, LA positive (aCL+, LA+). Livedo reticularis was observed in a large majority of patients (not necessarily associated with the presence of APS). Four patients presented splinter hemorrhages; extensive skin ulcers were more frequent in LA+ patients, while multiple small cutaneous ulcerations were rather associated with aCL. Thrombocytopenia appeared in half of the cases and thrombosis in 1/3 of them. Other disorders are less frequent: cerebrovascular accidents, migraine, osteonecrosis (but we found an aCL+ patient who developed osteonecrosis in two sites). Catastrophic antiphospholipid syndrome was not seen in this series.Conclusion: Organic disorder in the antiphospolipid syndrome is polymorphic. Correlations can be done between different aPLs and clinical manifestations, but results are not clear yet. There is still need for more research in this field as it is very important for an accurate diagnosis of autoimmune disorders.Citation: , volume , supplement , year 2003, page Session: Vasculitis