Recent advances in the management of children with familial Mediterranean fever

Abstract

Familial Mediterranean fever (FMF) is the most frequent hereditary autoinflammatory disease characterized by self-limited episodes of fever and serositis. FMF is more prevalent among non-Askhenazi Jewish, Turkish, Arabic and Armenian populations. FMF is inherited with an autosomal recessive pattern and is caused by mutations in the MEFV gene located on chromosome 16p13.3, encoding pyrin. AA type amyloidosis and the associated renal impairment are the most severe long-term complications. The diagnosis is established on the grounds of clinical findings. There have been a number of diagnostic criteria in the literature: the Tel Hashomer and Livneh criteria were first developed for the diagnosis of adult FMF patients. A new set of diagnostic criteria was recently proposed by Yalcinkaya et al. Colchicine is the best treatment option for the time being and some new agents have been tried in cases where there is colchicine resistance.