Commentary on Relapsing Polychondritis—The Rheumatologist's Perspective

The case report presented in this issue by Kut et al. serves as an excellent reminder of the challenges of diagnosing pediatric vasculitides. They are rare, mimickers of other diseases, and, in the case of relapsing polychondritis (RP), there are no established pediatric diagnostic criteria. The adult diagnostic criteria have not been validated in the pediatric population.

A recent retrospective study of 10 patients and a literature review of 37 patients found large variability in presentation, severity, and long-term outcome. ¹ Most children present with auricular chondritis, making this case atypical. Dyspnea, stridor, and hoarseness with only antecedent polyarthralgia do not suggest vasculitis. The authors appropriately ruled out malignancy and infection and then pursued less common etiologies. Autoantibodies suggestive of a rheumatic disease were all negative. There have been reports, in the adult literature, of anti-collagen Type II antibodies as markers for RP with elevations correlating with disease activity, but this has not been studied in children. ²

There have never been any randomized clinical trials for RP. Treatment usually consists of corticosteroids, NSAIDs, and a range of immunosuppressive therapies including hydroxychloroquine, methotrexate, azathioprine, dapsone, colchicine, and salazopyrine. Given the relapsing and remitting nature of this disease, unanswered questions include whether there are sufficient data to support continuous therapy versus treatment only during flares, and whether there is a subset of patients destined to have severe cartilage destruction without continuous treatment. Given the rarity of this disease, the development of a clinical registry for long-term follow-up and design of clinical trials would be important.