Relapsing polychondritis

Relapsing polychondritis is a recurring inflammatory disorder of unknown etiology causing inflammatory reactions in the cartilaginous structures of the nose, ears, trachea, and the joints. It is considered an auto-immune disorder. It was first described by Jaksch-Wartenhorst (1), in a patient with systemic illness characterized by external ear swelling, collapse of the nasal bridge, fever, and arthritis. Pearson and colleagues (2) first coined the term relapsing polychondritis (RP), and described, in detail, the clinical features of several of their own patients along with those in the literature. Since then, this disorder has been described worldwide and occurs in all age groups, although it peaks in the fifth decade. Over 30% of patients have an associated disorder, usually autoimmune or hematologic. These include systemic vasculitis syndromes, systemic lupus erythematosus (SLE), Sjögren's syndrome, overlap connective tissue disorders, rheumatoid arthritis (RA), spondyloarthropathies, dysmyelopoietic syndromes, Hodgkin's disease, diabetes mellitis, and psoriasis vulgaris (3). It is a rare disease occuring in 3.5 person's per million (Michet, personal communication). The 5-year survival is decreased to approximately 74%. The male:female ratio is equal (4).