Treatment of relapsing polychondritis

Abstract

Relapsing polychondritis (RP) manifests as an inflammation of cartilaginous structures throughout the body. Since RP affects cartilage tissues and proteoglycan-rich structures, symptoms appear in the ear, nose, eye, joints, and respiratory system. Otolaryngologists are frequently involved in RP's initial diagnosis, since it is likely to affect the head and neck regions. The etiology of RP remains unknown and it is relatively uncommon, so appropriate treatment remains to be determined. We studied the clinical course and treatment response. We studied clinical courses in 12 cases of RP treated at our hospital in the 11 years from 1991 to 2001. In 11 of the 12 cases, conventional steroid therapy was implemented. Five required steroid pulse therapy, and 7 immunosuppressive drugs to control the disease. Strong initial therapy such as steroid pulse therapy plus immunosuppressive drugs suppressed inflammation and decreased its relapse in severe RP patients. One patient could be controlled only with nonsteroidal antiinflammatory drugs. Side effects such as cataracts and diabetes from steroid therapy or pancytopenia from Dapson were found in some patients. All 12 are alive at present. Six were subsequently released from steroid therapy. Anti-type II collagen antibody was not useful as an indicator for treatment but helpful as one of a complementary factors for diagnosis. Manifestations of RP are sometimes self-limited, but in most cases, inflammation is recurrent, gradually worsening and finally becoming life-threatening. It is difficult to determine the severity of RP and choose appropriate treatment in its initial stage. Because severe RP cannot be controlled with conventional steroid therapy, strong therapy is indicated in initial treatment if patients have serious symptoms.