[Relapsing polychondritis].
Relapsing polychondritis is a rare systemic disease. Clinical manifestations are variable and the condition is frequently associated with other diseases. Chondritis typically involves ears, nose, costal cartilages and upper airways. Other classical features include scleritis and episcleritis, mucocutaneous lesions, arthralgia and constitutional symptoms. Repeated inflammation of cartilaginous structures may lead to deformities of the ears, the nose and the airways. Tracheobronchomalacia and aortitis of the ascending portion resulting in aortic regurgitation or aneurysm are feared complications. Treatment is mainly based on systemic corticosteroids alone or in association with immunosuppressants. Several biological immunosuppressive agents are reported effective in refractory disease.
Arlettaz
Lionel
L
Service d'immunologie et d'allergologie, Département de médecine interne HUG, 1211 Genève 14. lionel.arlettaz@hcuge.ch
Ribi
Camillo
C
D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D000783:Aneurysm / Q000209:etiology; D000894:Anti-Inflammatory Agents, Non-Steroidal / Q000627:therapeutic use; D001022:Aortic Valve Insufficiency / Q000209:etiology; D001025:Aortitis / Q000209:etiology; D003937:Diagnosis, Differential; D004359:Drug Therapy, Combination; D004431:Ear, External / Q000473:pathology; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D007818:Laryngeal Diseases / Q000209:etiology; D009666:Nose / Q000473:pathology; D011081:Polychondritis, Relapsing / Q000150:complications* / Q000188:drug therapy / Q000473:pathology* / Q000517:prevention & control; D015423:Scleritis / Q000209:etiology; D012867:Skin / Q000473:pathology; D014133:Tracheal Diseases / Q000209:etiology; D055089:Tracheobronchomalacia / Q000209:etiology* / Q000473:pathology*; D016896:Treatment Outcome
D000305:Adrenal Cortex Hormones; D000894:Anti-Inflammatory Agents, Non-Steroidal; D007166:Immunosuppressive Agents