Clinical characteristics of antiphospholipid antibody syndrome in children

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