Diagnosis and classification of relapsing polychondritis

•RP is a systemic disease involving cartilaginous structures throughout the body.•It often starts with cartilage involvement, but a multi-organ damage is possible.•It can occur as an overlap syndrome in patients with many other disorders.•The prognosis is poor for patients displaying respiratory tract involvement.•Diagnosis depends on the recognition of RP features and symptom-driven testing.

Relapsing polychondritis is a rare and potentially fatal autoimmune disease of unknown etiology, characterized by inflammation and destruction of different cartilaginous structures, including the ear, nose, larynx, trachea, bronchi, peripheral joints, eye, heart and skin, with high risk of misdiagnosis. The spectrum of clinical presentations is protean and may vary from intermittent episodes of painful and disfiguring auricular and nasal chondritis or polyarthritis to severe progressive multi-organ damage. A laryngotracheobronchial involvement appears in nearly half of patients and is complicated by local obstructions, which may be life-threatening. A highly medical specialized approach is required for diagnosis of relapsing polychondritis. This review comprehensively examines the literature related to the clinical sceneries of the disease and focuses on both diagnostic tools used in clinical studies and recent findings related to its etiopathogenesis.