Relapsing polychondritis

Keywords Disease name and synonyms Diagnostic criteria / Definition Differential diagnosis Prevalence Laboratory findings Prognosis Management Etiology Genetic findings Diagnostic methods Genetic counseling Unresolved questions References Abstract Relapsing polychondritis (RP) is a multisystem inflammatory disease of unknown etiology affecting the cartilage. It is characterized by recurrent episodes of inflammation affecting the cartilaginous structures, resulting in tissue damage and tissue destruction. All types of cartilage may be involved. Chondritis of auricular, nasal, tracheal cartilage predominates in this disease, suggesting response to tissue-specific antigens such as collagen II and cartilage matrix protein (matrillin-1). The patients present with a wide spectrum of clinical symptoms and signs that often raise major diagnostic dilemmas. In about one third of patients, RP is associated with vasculitis and autoimmune rheumatic diseases. The most commonly reported types of vasculitis range from isolated cutaneous leucocytoclastic vasculitis to systemic polyangiitis. Vessels of all sizes may be affected and large-vessel vasculitis is a well-recognized and potentially fatal complication. The second most commonly associated disorder is autoimmune rheumatic diseases mainly rheumatoid arthritis and systemic lupus erythematosus. Other disorders associated with RP are hematological malignant diseases, gastrointestinal disorders, endocrine diseases and others. Relapsing polychondritis is generally a progressive disease. The majority of the patients experience intermittent or fluctuant inflammatory manifestations. In Rochester (Minnesota), the estimated annual incidence rate was 3.5/million. The peak age for disease onset is the fifth decade, although cases have been reported at both extremes of life. The treatment of choice is prednisone; however, immunosuppressive drugs seem to have an additional value. Methotrexate was found to be an efficacious and well-tolerated steroid sparing drug.