Abstract
Relapsing polychondritis (PR) is a multisystem autoimmune disease of unknown origin characterized by the destruction of cartilaginous tissues and proteoglycan. We herein report the case of an 82-year-old male who first presented with left auricular erythema with swelling sparing the lobule. He subsequently developed right sensorineural hearing loss (SNHL), recurrence of left auricular chondritis, and scleritis. Steroid therapy was effective for these symptoms. Although the auricular chondritis was unilateral, we diagnosed him with PR due to the SNHL, scleritis, and efficacy of steroid therapy based on Damiani and Levine's PR criteria. We also analyzed the onset of auricular chondritis in 40 cases who first presented in Japan to determine whether it was unilateral or bilateral. Unilateral onset of auricular chondritis was noted in 32/40(80%) patients, while bilateral onset occurred in 8/40 (20%). Twenty-one of the 32 (65.6%) patients with unilateral chondritis eventually developed bilateral chondritis. Therefore, bilateral auricular chondritis eventually was present in 29/40 (72.5%) patients. Eleven of the 40 (25.5%) patients had left unilateral involvement. The duration from unilateral to bilateral disease was 6.2 months. Many cases of first onset auricular chondritis due to PR were unilateral, and there have been previously reported cases of left unilateral auricular chondritis. A major feature of auricular chondritis due to PR is sparing of the lobule. Therefore, we propose that bilateral chondritis of the auricles as a customary diagnostic criterion should be changed to auricular chondritis (unilateral or bilateral) sparing the lobule.
