title: Churg-Strauss syndrome in childhood: a systematic literature review and clinical comparison with adult patients.
abstract: OBJECTIVE
  To describe the clinical characteristics of children with Churg-Strauss syndrome (CSS) in comparison with adult patients.
  
  
  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.
authors.affiliation: Department of Internal Medicine 3 and Institute for Clinical Immunology, University of Erlangen-Nuremberg, Erlangen, Germany. jochen.zwerina@uk-erlangen.de
authors.forename: Jochen
authors.initials: J
authors.lastname: Zwerina
authors.forename: Gerhard
authors.initials: G
authors.lastname: Eger
authors.forename: Matthias
authors.initials: M
authors.lastname: Englbrecht
authors.forename: Bernhard
authors.initials: B
authors.lastname: Manger
authors.forename: Georg
authors.initials: G
authors.lastname: Schett
mesh_terms: D000293:Adolescent; D000328:Adult; D000367:Age Factors; D001249:Asthma / Q000150:complications / Q000473:pathology / Q000503:physiopathology; D002648:Child; D002675:Child, Preschool; D015267:Churg-Strauss Syndrome / Q000150:complications* / Q000503:physiopathology / Q000628:therapy; D004802:Eosinophilia / Q000150:complications / Q000473:pathology / Q000503:physiopathology; D005260:Female; D006801:Humans; D008297:Male