title: [Childhood vasculitis].
abstract: BACKGROUND
  The primary vasculitides are rare conditions in childhood. The most common disease subtypes are Schönlein-Henoch purpura and Kawasaki's syndrome, which frequently have a self-limiting course. In the majority of vasculitides, the etiology remains unknown. Environmental exposure, including infections, is suspected to trigger an autoinflammatory response in predisposed individuals.
  
  
  OBJECTIVE
  The aim of this review is to present the various aspects of childhood vasculitis.
  
  
  METHODS
  Reviews and special original papers on childhood vasculitis, published classification criteria and current therapy guidelines were reviewed and summarized.
  
  
  RESULTS
  The classification of vasculitides in childhood has been modified from the previous adult Chapel Hill classification for vasculitides in 2008. Most therapy recommendations for children are adapted from results of studies in adults. This review covers the current classifications, pathogenesis, clinical manifestations and therapy recommendations for children.
  
  
  CONCLUSIONS
  Although etiology and pathogenesis of many vasculitides in childhood are still unknown, clarifying diagnostic methods and effective therapeutic options are available. The knowledge about various forms of disease manifestation may contribute to an early diagnosis and timely initiation of treatment, which may prevent devastating irreversible impairment.
authors.affiliation: Klinik für Kinder- und Jugendmedizin, Autoinflammation Reference Center Tübingen, Universitätsklinikum Tübingen, Hoppe-Seyler-Str. 1, 72076, Tübingen, Deutschland. kuemmerle.deschner@uni-tuebingen.de.
authors.forename: J B
authors.initials: JB
authors.lastname: Kümmerle-Deschner
authors.affiliation: Klinik für Kinder- und Jugendmedizin, Autoinflammation Reference Center Tübingen, Universitätsklinikum Tübingen, Hoppe-Seyler-Str. 1, 72076, Tübingen, Deutschland.
authors.forename: J
authors.initials: J
authors.lastname: Thomas
authors.affiliation: Rheumatology, Department of Pediatrics, Alberta Children's Hospital, University of Calgary, 2888 Shaganappi Trail NW, T3B 6A8, Calgary, Alberta, Canada.
authors.forename: S M
authors.initials: SM
authors.lastname: Benseler
mesh_terms: D002648:Child; D002675:Child, Preschool; D003937:Diagnosis, Differential; D005858:Germany; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D038622:Internationality; D008297:Male; D010372:Pediatrics / Q000592:standards*; D017410:Practice Guidelines as Topic; D012219:Rheumatology / Q000592:standards*; D063189:Symptom Assessment / Q000592:standards*; D014657:Vasculitis / Q000175:diagnosis* / Q000628:therapy*
keywords: Granulomatosis with Polyangiitis; Kawasaki syndrome; Polyarteritis nodosa; Purpura Schoenlein-Henoch; Takayasu Arteritis