Relapsing polychondritis--report of ten cases

Abstract

Relapsing polychondritis is a rare disease of unknown etiology. There are approximately 211 reported cases in the world literature. This is a report of ten cases from the Cleveland Clinic Foundation. McAdam's diagnostic criteria for R.P. were reviewed and modified. For diagnosis, all patients had to have 1. at least three or more diagnostic criteria, histologic confirmation not necessary; 2. one or more of McAdam's signs with positive histologic confirmation; or 3. chondritis in two or more separate anatomic locations with response to steroids and/or Dapsone. Chondritis of the auricles (9/10 patients) and arthropathy (8/10 patients) are the most common presenting signs. Chondritis was also seen in the nose (6/10) and the upper respiratory tract involving the larynx and trachea (4/10). Cochlear and vestibular damage and ocular inflammation were each seen in 5/10 patients. Patients were treated with steroids and/or Dapsone. Both drugs were reliable in abating episodes of activity and in decreasing recurrences. These results further support Dapsone as an alternate form of treatment for RP.