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.

Keywords: Churg-Strauss syndrome, vasculitis, childhood, ANCA