title: [Familial Mediterranean fever – an important disease in a globalised world].
abstract: Familial Mediterranean fever - an important disease in a globalised world Familial Mediterranean fever (FMF) is characterized by recurrent febrile attacks during 1/2-3 days associated with peritonitis, pleuritis and arthritis. FMF is the most common monogenic autoinflammatory disease in the world, with over 100 000 affected individuals. It is particularly common in individuals with an origin in the eastern Mediterranean Basin, where the disease has a prevalence of 100-200 per 100 000. The gene for FMF (MEFV) was identified in 1997 with an autosomal recessive inheritance; however, a significant proportion (≈25%) of clinical patients lack two mutations. MEFV codes for the protein pyrin, whose exact function still needs to be defined. The most serious complication of FMF is amyloid A amyloidosis, in particular renal amyloidosis. FMF is efficiently treated with daily doses of colchicine resulting in an almost normal life expectancy and amyloidosis confined to non-compliant patients. In today's globalized world we need to adapt to a new context that includes inherited conditions, which have historically been uncommon in our part of the world. One of these conditions is FMF, that should primarily be suspected in individuals with an origin in the eastern Mediterranean Basin and recurrent attacks of fever.
authors.affiliation: NU-sjukvården - Barn- och ungdomskliniken Uddevalla, Sweden NU-sjukvården - Barn- och ungdomskliniken Uddevalla, Sweden.
authors.forename: Per
authors.initials: P
authors.lastname: Wekell
authors.affiliation: Institutionen för medicin, Sahlgrenska Akademin, Göteborgs Universitet - Avdelningen för reumatologi och inflammationsforskning Göteborg, Sweden Institutionen för medicin, Sahlgrenska Akademin, Göteborgs Universitet - Avdelningen för reumatologi och inflammationsforskning Göteborg, Sweden.
authors.forename: Anna
authors.initials: A
authors.lastname: Karlsson
authors.affiliation: Institutionen för kliniska vetenskaper, Sahlgrenska Akademin, Göteborgs Universitet - 2. Avdelningen för pediatrik Göteborg, - .
authors.forename: Anders
authors.initials: A
authors.lastname: Fasth
authors.affiliation: Institutionen för kliniska vetenskaper, Sahlgrenska Akademin, Göteborgs Universitet - Avdelningen för pediatrik Göteborg, Sweden Institutionen för kliniska vetenskaper, Sahlgrenska Akademin, Göteborgs Universitet - Avdelningen för pediatrik Göteborg, Sweden.
authors.forename: Stefan
authors.initials: S
authors.lastname: Berg
mesh_terms: D000328:Adult; D000686:Amyloidosis / Q000209:etiology; D002648:Child; D003078:Colchicine / Q000627:therapeutic use; D010505:Familial Mediterranean Fever / Q000175:diagnosis / Q000188:drug therapy / Q000235:genetics / Q000503:physiopathology; D014943:Global Health; D006801:Humans; D019083:Mediterranean Region; D050257:Tubulin Modulators / Q000627:therapeutic use
chemical_list: D050257:Tubulin Modulators; D003078:Colchicine