Churg–Strauss Syndrome in Childhood: A Systematic Literature Review and Clinical Comparison with Adult Patients.

Objective: To describe the clinical characteristics of children with Churg–Strauss syndrome (CSS) in comparison with adult patients. Materials and methods: A systematic literature analysis was performed in the Medline database up to November 2007 and in rheumatology and pulmonology meeting scientific abstracts 2003-2007. Articles with reported childhood CSS cases were retrieved; clinical data were recorded. Descriptive statistical analyses and a comparison with 2 published adult CSS cohorts were performed. Results: Thirty-three cases of childhood CSS were identified. The mean age was 12 years and the male-to-female ratio was 0.74. All patients had significant eosinophilia and asthma. Histological evidence of eosinophilia and/or vasculitis was present in virtually all patients. Antineutrophil cytoplasmic antibodies were found in 25% of children with CSS. Initial treatment was corticosteroid monotherapy in 76% of childhood CSS patients, while 24% received additional immunosuppressive therapy. Another 18% required further immunosuppression at follow-up due to frequent relapses. Six deaths (18%), all related to the underlying disease, occurred after a mean disease duration of 14 months. As compared with adult CSS patients, children had a predominance of cardiopulmonary disease manifestations, a lower rate of peripheral nerve involvement, and higher mortality. Conclusions: Many aspects of CSS are similar in childhood and adult patients. However, pulmonary and cardiac involvement is predominant in pediatric CSS and mortality is substantial. [Copyright &y& Elsevier]