Relapsing polychondritis is a rare autoimmune disease of the cartilage characterized by recurrent inflammation and destruction of cartilaginous tissue. The common manifestations are chondritis of the ear, nose, trachea, and joints. This inflammatory condition is progressive and deteriorates if left untreated. As these manifestations are not usually present concurrently, the disease is subjected to misdiagnosis or delayed diagnosis. We describe a 25-year-old woman who initially had a fever of unknown origin and refractory nasal obstruction but was eventually diagnosed with relapsing polychondritis owing to bilateral auricular inflammation. Immunotherapy was administered accordingly. The disease continued to progress to manifestations of airway obstruction and tracheal stenosis. Severe narrowing of the trachea and collapse of the left bronchus were noted, and the pulmonary function tests revealed a fixed obstructive pattern. After pulse immunosuppressive therapy, respiratory symptoms were controlled, although respiratory function remained impaired. We believe that early notification of this disease may assist in early diagnosis, and prevention of severe airway complications. (J Taiwan Otolaryngol Head Neck Surg 2022; 57:221-226)
