Relapsing polychondritis is a rare, episodic autoimmune disorder characterized by widespread, potentially destructive inflammation of cartilage throughout the body, including ears (most commonly involved), nose, cardiac and respiratory structures, joints, and eye. Males and females of all ages and races can be affected, although white individuals seem more susceptible. The disease is variable in duration and severity. Nasal bridge involvement can progress to cause saddle nose deformity from cartilage collapse. Laryngotracheobronchial disease may be insidious but can lead to the fatal complication of laryngeal collapse. Involvement of the inner ear, cardiovascular system, and skin is less common. Cardiovascular problems include aortic insufficiency (due to progressive dilation of the aortic root) and vasculitis. Skin lesions are most often caused by cutaneous vasculitis.
In up to one-third of patients, relapsing polychondritis can be associated with other connective tissue diseases (eg, SLE or RA), systemic vasculitis, or malignancy. Ocular manifestations, which occur in up to 60% of patients, include episcleritis, scleritis, uveitis, and, rarely, retinal vasculitis.
Treatment focuses on reducing symptoms and preserving the integrity of cartilaginous structures. Pharmacotherapy includes systemic corticosteroids, dapsone, methotrexate, and cyclophosphamide. Patients may require surgical interventions such as tracheostomy, aortic aneurysm repair, and cardiac valve replacement.
Excerpted from BCSC 2020-2021 series: Section 1 - Update on General Medicine. For more information and to purchase the entire series, please visit https://www.aao.org/bcsc.