ANTIPHOSPHOLIPID ANTIBODIES IN PEDIATRIC SYSTEMIC LUPUS-ERYTHEMATOSUS, JUVENILE CHRONIC ARTHRITIS AND OVERLAP SYNDROMES - SLE PATIENTS WITHBOTH LUPUS ANTICOAGULANT AND HIGH-TITER ANTICARDIOLIPIN ANTIBODIES ARE AT RISK FOR CLINICAL MANIFESTATIONS RELATED TO THE ANTIPHOSPHOLIPID SYNDROME

Antiphospholipid antibodies (APA) are often associated with severe cli nical manifestations, especially in the setting of systemic lupus eryt hematosus (SLE). Here we have investigated the prevalence of anticardi olipin antibodies (aCL) and lupus anticoagulant (LA) in paediatric pat ients affected with SLE, JCA and overlap syndromes (OS) and correlated the presence of aCL and LA with clinical features. aCL were assayed b y enzyme-limited immunoassay; LA was determined by activated partial t hromboplastin time and the kaolin clotting time test. aCL and LA assay s were performed in parallel on at least two occasions over a 7-30-mon th follow-up. Fifteen out of nineteen (79%) SLE patients had aCL and 8 /19 (42%) had LA. Six SLE patients displayed manifestations that were APA-related: deep venous thromboses, autoimmune haemolytic anaemia, pu lmonary hypertension, neurological alterations. Five out of six sympto matic patients had both LA and high-titre aCL. In contrast, JCA and OS patients had usually low-titre aCL, no detectable LA and no APA-relat ed manifestations. aCL persisted at high titre over time in SLE patien ts, but was only transiently detected in JCA and OS patients. This stu dy shows that the simultaneous positivity for LA and high-titre aCL al lows the identification of paediatric SLE patients who are at risk not only for thrombosis, but also for other APA-related clinical features .