Churg-Strauss syndrome in childhood: a systematic literature review and clinical comparison with adult patients

Semin Arthritis Rheum. 2009 Oct;39(2):108-15. doi: 10.1016/j.semarthrit.2008.05.004. Epub 2008 Jul 17.


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.

Publication types

  • Comparative Study
  • Research Support, Non-U.S. Gov't
  • Review
  • Systematic Review

MeSH terms

  • Adolescent
  • Adult
  • Age Factors
  • Asthma / complications
  • Asthma / pathology
  • Asthma / physiopathology
  • Child
  • Child, Preschool
  • Churg-Strauss Syndrome / complications*
  • Churg-Strauss Syndrome / physiopathology
  • Churg-Strauss Syndrome / therapy
  • Eosinophilia / complications
  • Eosinophilia / pathology
  • Eosinophilia / physiopathology
  • Female
  • Humans
  • Male