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Pediatric antiphospholipid syndrome : clinical and immunologic features of 121 patients in an international registry
Avčin, Tadej ...
Objectives: The purpose of this study was to obtain data on the association ofantiphospholipid antibodies with clinical manifestations in childhood and toenable future studies to determine the impact ...of treatment and long-term outcome of pediatric antiphospholipid syndrome. Patients and methods: A European registry extended internationally of pediatric patients with antiphospholipid syndrome was established as a collaborative project of the European Antiphospholipid Antibodies Forum and Lupus Working Group of the Pediatric Rheumatology European Society. To be eligible for enrollment the patient must meet the preliminary criteria for the classification of pediatricantiphospholipid syndrome and the onset of antiphospholipid syndrome must have occurred before the patient's 18th birthday. Results: As of December1, 2007, there were 121 confirmed antiphospholipid syndrome cases registered from 14 countries. Fifty-six patients were male, and 65 were female, with a mean age at the onset of antiphospholipid syndrome of 10.7 years. Sixty (49.5%) patients had underlying autoimmune disease. Venous thrombosis occurred in 72 (60%), arterial thrombosis in 39 (32%), small-vesselthrombosis in 7 (6%), and mixed arterial and venous thrombosis in 3 (2%). Associated nonthrombotic clinical manifestations included hematologicmanifestations (38%), skin disorders (18%), and nonthrombotic neurologic manifestations (16%). Laboratory investigations revealed positive anticardiolipin antibodies in 81% of the patients, anti-beta(2)-glycoprotein Iantibodies in 67%, and lupus anticoagulant in 72%. (Abstract truncated at 2000 characters)
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