Relapsing polychondritis: a clinical review

Objective: This study comprehensively reviews the literature related to relapsing polychondritis (RP).

Methods: A detailed search via MEDLINE (PubMed) was performed using relapsing polychondritis as the key term. Relevant articles were analyzed with a focus on history, epidemiology, etiology, pathogenesis, clinical manifestations, diagnosis, treatment, and prognosis of RP.

Results: RP is a rare episodic and progressive inflammatory disease of presumed autoimmune etiology first described in 1923. RP affects cartilage in multiple organs, such as the ear, nose, larynx, trachea, bronchi, and joints. In addition, it can affect proteoglycan-rich tissues, such as the eyes, aorta, heart, and skin. The diagnosis of RP is based on the presence of clinical criteria. A standardized therapeutic protocol for RP has not been established. Nonsteroidal anti-inflammatory drugs, dapsone and/or colchicine, may control disease activity in some patients. In other patients, immunosuppressive drugs and prednisone have been effective. RP is a potentially lethal disease; pulmonary infection, systemic vasculitis, airway collapse, and renal failure are the most common causes of death. Earlier studies indicate survival rates between 70% at 4 years and 55% at 10 years. In a recent study, a survival rate of 94% at 8 years may be due to improved medical and surgical management.

Conclusions: RP is a rare, multisystemic, and potentially fatal disease. The pathogenesis and optimal therapeutic approach to patients with RP is poorly understood.