Cogan's syndrome in a patient with familial Mediterranean fever.

摘要

Familial Mediterranean fever (FMF) is an autoinflammatory disease caused by mutations in the Mediterranean fever {MEFV) gene. Flares of inflammation occur, with fever and abdominal or chest pain indicating serositis. Symptoms indicating muscle and joint inflammatory are common during or between flares (1).A 54-year-old man was admitted in 2009 for polyarthitis of the wrists, knees, and meta-carpo-phalangeal joints. One year prior to this presentation, the patient had a history of progressive bilateral sensorineural hearing loss. Cerebral and auditory conducts MRI was normal. The patient was of arme-nian origin. A diagnosis of FMF was made in 1982 (recurrent fever and pleurisy); and was confirmed in 2002: identification of mutations in the MEFV gene (M680II V726A). Colchicine was effective in prevention of flare of inflammation and amyloidosis.