Relapsing Polychondritis Treatment & Management

Updated: Sep 25, 2017
  • Author: Nicholas Compton, MD; Chief Editor: Herbert S Diamond, MD  more...
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Treatment

Medical Care

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-alpha inhibitors [46] : infliximab, [47, 48] etanercept, [47] adalimumab [49]
  • Anakinra, an interleukin 1 receptor antagonist [50, 51]
  • Leflunomide, which inhibits pyrimidine synthesis [52]
  • Rituximab, an anti-CD20 chimeric antibody [53]
  • Tocilizumab, a humanized monoclonal antibody against the interleukin-6 receptor (IL-6R) [54]

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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Surgical Care

Surgeries encountered in the care of patients with relapsing polychondritis may include the following:

  • Tracheostomy
  • Permanent tracheotomy placement
  • Tracheal stent placement
  • Aortic aneurysm repair
  • Cardiac valve replacement
  • Saddle-nose deformity repair

Subglottic stenosis can be treated with submucosal corticosteroid injection followed by serial dilation. Wierzbicka et al reported good airway patency for more than 24 months in eight of 12 patients with relapsing polychondritis or other autoimmune disorders treated with this approach. [55]

The benefits of any proposed surgery must be weighed adequately against the patient's risk for infection, especially in the event of acute relapse, since patients are at an increased risk of infection whether or not they are using corticosteroids.

Additionally, patients with relapsing polychondritis and tracheal disease may be at particular risk regarding complications resulting from tracheal intubation and extubation.

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Consultations

Relapsing polychondritis is a complex condition that requires a team approach for patient care, as follows:

  • Dermatologists or specialists in infectious diseases are often involved early in the course of the disease to evaluate the patient for infectious causes of cellulitis or perichondritis.
  • Rheumatologists usually become the primary care provider and should be involved early in patient care.
  • Ophthalmologists should also be involved early to diagnose, monitor, and treat the potentially devastating ocular complications.
  • Cardiologists, neurologists, nephrologists, and otolaryngologists may be asked to manage other aspects of relapsing polychondritis.
  • Plastic surgeons can aid in nasal reconstruction if saddle-nose deformity is present.
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