title: [Clinical aspects of Familial Mediterranean fever].
abstract: Familial Mediterranean fever (FMF) is a hereditary autoinflammatory disease characterized by recurrent and short duration (1-3 days) of fever, and serositis. Based on the nationwide survey of FMF in Japan, the estimated number of Japanese FMF patients is about three hundred. High grade fever was observed in 95.5%, chest pain in 35.8% abdominal pain in 62.7% and arthritis in 31.3% among Japanese FMF patients. AA amyloidosis was confirmed in 5 patients (3.7%). Colchicine was effective in 91.8% of Japanese FMF patients. A significant number of FMF patients exist in Japan, and early diagnosis and treatments should be required to prevent AA amyloidosis.
authors: Clinical Research Center, NHO Nagasaki Medical Center.
authors: Kiyoshi
authors: K
authors: Migita
authors: Kazunaga
authors: K
authors: Agematsu
mesh_terms: D000293:Adolescent; D000328:Adult; D000686:Amyloidosis / Q000209:etiology / Q000517:prevention & control; D002648:Child; D002675:Child, Preschool; D003078:Colchicine / Q000627:therapeutic use; D003598:Cytoskeletal Proteins / Q000235:genetics; D042241:Early Diagnosis; D010505:Familial Mediterranean Fever / Q000175:diagnosis / Q000188:drug therapy / Q000235:genetics / Q000503:physiopathology; D005260:Female; D006801:Humans; D007564:Japan / Q000453:epidemiology; D008297:Male; D008875:Middle Aged; D009154:Mutation; D000071198:Pyrin; D016896:Treatment Outcome; D055815:Young Adult
chemical_list: D003598:Cytoskeletal Proteins; C000604192:MEFV protein, human; D000071198:Pyrin; D003078:Colchicine