Pediatric-onset relapsing polychondritis: case series and systematic review

J Pediatr. 2010 Mar;156(3):484-9. doi: 10.1016/j.jpeds.2009.09.045. Epub 2009 Nov 1.


Objectives: To study the pediatric presentation and evolution of relapsing polychondritis (RP), a rare inflammatory disease characterized by recurrent inflammation of cartilage.

Study design: 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.

Conclusion: 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.

Publication types

  • Review
  • Systematic Review

MeSH terms

  • Adolescent
  • Child
  • Female
  • Humans
  • Male
  • Polychondritis, Relapsing* / diagnosis
  • Polychondritis, Relapsing* / drug therapy