title: Pediatric-onset relapsing polychondritis: case series and systematic review.
abstract: OBJECTIVE
  To study the pediatric presentation and evolution of relapsing polychondritis (RP), a rare inflammatory disease characterized by recurrent inflammation of cartilage.
  
  
  METHODS
  We retrospectively collected data from 10 patients observed in 3 French hospitals for relapsing polychondritis, with an age at onset <18 years. We also analyzed 37 cases of pediatric-onset RP from a systematic review.
  
  
  RESULTS
  The mean age at first symptoms was 8.6 years, and the sex ratio was 6 male patients and 4 female patients. Children came to medical attention with joint pain, ocular inflammation, and chondritis. Outcomes included severe visual impairment, chronic destructive chondritis, and 1 death caused by aortic dilatation. Treatment mainly consisted of non-steroidal-anti-inflammatory drugs, corticosteroids, and immunosuppressants. Growth was normal in 7 examined patients. Systematic literature review also suggested a high number of tracheostomy in pediatric cases, but this was not confirmed in our series.
  
  
  CONCLUSIONS
  RP in childhood shares the main clinical features of its adult counterpart, including destructive chondritis and systemic symptoms, but unlike adults, children frequently have a family history of autoimmunity and infrequently have other associated autoimmune diseases. RP can be fatal; close screening for complications is mandatory. Growth does not appear to be impaired by cartilage inflammation.
authors.affiliation: Service de néphrologie et rhumatologie pédiatrique, Hôpital Femme Mère Enfant, Hospices Civils de Lyon et Université de Lyon, Lyon, France. alexbelot@yahoo.fr
authors.forename: Alexandre
authors.initials: A
authors.lastname: Belot
authors.forename: Agnès
authors.initials: A
authors.lastname: Duquesne
authors.forename: Chantal
authors.initials: C
authors.lastname: Job-Deslandre
authors.forename: Nathalie
authors.initials: N
authors.lastname: Costedoat-Chalumeau
authors.forename: Sabaa
authors.initials: S
authors.lastname: Boudjemaa
authors.forename: Bertrand
authors.initials: B
authors.lastname: Wechsler
authors.forename: Pierre
authors.initials: P
authors.lastname: Cochat
authors.forename: Jean-Charles
authors.initials: JC
authors.lastname: Piette
authors.forename: Rolando
authors.initials: R
authors.lastname: Cimaz
mesh_terms: D000293:Adolescent; D002648:Child; D005260:Female; D006801:Humans; D008297:Male; D011081:Polychondritis, Relapsing / Q000175:diagnosis / Q000188:drug therapy