Familial Mediterranean fever.
Familial Mediterranean fever (FMF) is the most frequent periodic syndrome characterized by recurrent attacks of polyserositis. Fever, abdominal pain, chest pain, and arthritis/arthralgia are the leading symptoms. It is an autosomal recessive disorder, which primarily affects Jewish, Armenian, Turkish, and Arab populations. The FMF gene ( MEFV) has recently been cloned to chromosome 16p, which encodes pyrin. Genotype-phenotype correlation is not well established. Amyloidosis is the most severe complication of FMF. The SAA1-alpha/alpha genotype was associated with an increased risk of amyloidosis. Colchicine treatment not only decreases the frequency and severity of attacks, but also prevents amyloidosis. Certain vasculitides, namely Henoch-Schonlein purpura and polyarteritis nodosa, are more frequent among FMF patients.
Bakkaloglu
Aysin
A
Department of Pediatrics, Pediatric Nephrology and Rheumatology Unit, Faculty of Medicine, Hacettepe University, Ankara 06100, Turkey. aysin@hacettepe.edu.tr
D000686:Amyloidosis / Q000235:genetics / Q000473:pathology; D002648:Child; D010505:Familial Mediterranean Fever / Q000235:genetics / Q000473:pathology*; D006801:Humans; D007674:Kidney Diseases / Q000235:genetics / Q000473:pathology*; D014657:Vasculitis / Q000235:genetics / Q000473:pathology*