Relapsing polychondritis in childood: rare but important

Article Outline

• Copyright

French investigators report a case series with detailed clinical information and longterm follow-up of 10 children with relapsing polychondritis (RP). They also report a systematic review of other pediatric cases and compare the clinical characteristics of RP in children with those of adults (in whom disease is more common). Together these data provide a snapshot of childhood presentation and complications of RP, which in most ways looks like adult-onset disease. But the authors point out a few important clinical points. Chondritis is the heralding event in most patients. Children frequently have a family history of autoimmune disorders. There is a long delay in diagnosis in children, likely related to lack of familiarity of pediatricians with the entity. Cardiac complications may be under-recognized in children, and were fatal in one French adolescent. The authors also report that despite multiple episodes of inflammatory chondritis, linear growth appears to be unimpaired in children with RP – a finding that provides food for thought about cartilage versus bone health, local versus systemic inflammation, and their impact on growth.