CLINICAL CHARACTERISTICS OF ANTIPHOSPHOLIPID ANTIBODY SYNDROME IN CHILDREN

Objective: To evaluate the clinical features and outcome of antiphosph olipid syndrome (APS) in children. Study design: Retrospective chart r eview of patients seen at the Children's Hospital of Philadelphia and Children's Seashore House Pediatric Rheumatology Center between 1988 a nd 1993. Results: Nine patients with ages ranging from 8 months to 17 years are presented. Clinical features of five patients with primary A PS, described in detail, were digital ischemia, stroke, chorea, Addiso n disease, and pulmonary vaso-occlusive disease. The four children wit h secondary APS had systemic lupus erythematosus. Clinical features of these patients include livedo reticularis, deep venous thrombosis, an d pulmonary hypertension. Antiphospholipid titers, results of coagulat ion studies, and serologic findings did not predict outcome. Conclusio n: APS in children has diverse clinical features similar to those in a dults and should be considered in cases of unexplained vaso-occlusive disease.