How is relapsing polychondritis (RP) treated?

Updated: Dec 24, 2020
  • Author: Nicholas Compton, MD; Chief Editor: Herbert S Diamond, MD  more...
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No controlled trials of therapy for relapsing polychondritis (RP) have been published. The goal of treatment is to abate current symptoms and to preserve the integrity of cartilaginous structures.

The mainstay of treatment is systemic corticosteroid therapy. Prednisone (20-60 mg/d) is administered in the acute phase and is tapered to 5-25 mg/d for maintenance. Severe flares may require 80-100 mg/d. Most patients require a low daily dose of prednisone for maintenance; however, intermittent administration of high doses during only flares of the condition is successful in rare cases. McAdam et al found that continuous prednisone decreased the severity, frequency, and duration of relapses. [16] See the images below.

Same patient as in Image 5 after 4-6 weeks of ster Same patient as in Image 5 after 4-6 weeks of steroid treatment. Note resolution of auricular inflammation with nodularity and forward listing of the ears. Courtesy of the University of Washington, Division of Dermatology.
Close-up view of same patient as in Image 6. Forwa Close-up view of same patient as in Image 6. Forward flopping of ear with nodularity after steroid treatment. Courtesy of the University of Washington, Division of Dermatology.

Other medications reported to control symptoms and, perhaps, progression of the disease, include dapsone (25-200 mg/d), azathioprine, methotrexate (MTX; 7.5-22.5 mg/wk), cyclophosphamide, and cyclosporine. MTX has been dosed beginning at 7.5 mg/wk, increasing up to 22.5 mg/wk in conjunction with steroid administration and has been found to significantly decrease corticosteroid requirements while controlling symptoms.

Case reports have described successful treatment with the following:

  • Anti–tumor necrosis factor (TNF)–alpha inhibitors [50] : infliximab, [51, 52] etanercept, [51] adalimumab [53]
  • Anakinra, an interleukin 1 receptor antagonist [54, 55]
  • Leflunomide, which inhibits pyrimidine synthesis [56]
  • Rituximab, an anti-CD20 chimeric antibody [57]
  • Tocilizumab, a humanized monoclonal antibody against the interleukin-6 receptor (IL-6R) [58]

In a French multicenter retrospective cohort study that included 41 patients with relapsing polychondritis treated with biologics (105 instances; TNF inhibitors, n=60; tocilizumab, n=17; anakinra, n=15; rituximab, n=7; abatacept, n=6), the overall response rate during the first 6 months of treatment was 62.9%; however the complete response rate was 19.0%. Reduction in corticosteroid doses was highly variable. [59]

Differences in clinical response rates varied with organ involvement. There were trends toward a lower response rate in patients with associated myelodysplastic syndrome and a higher response rate for nasal/auricular chondritis, sternal chondritis, and concomitant exposure to non-biologic disease-modifying antirheumatic drugs. [59]

Orally administered nonsteroidal anti-inflammatory drugs (NSAIDs) have not been effective.

Medical care must include assessment for and treatment of other confounding or concurrent autoimmune disorders.

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