The spectrum of urological disease in familial Mediterranean fever: amyloidosis and beyond

Abstract

Introduction Familial Mediterranean fever (FMF) is the most common inherited monogenic autoinflammatory disease that gained the attention of researchers for decades. Population from East Mediterranean origin are mainly affected. Prevalence of FMF in endemic countries varies from 1:500 to 1:1,000; Turkey has the highest number of patients followed by Israel and Armenia. The most commonly incriminated genotypes are recessive gain-of-function mutations of the Mediterranean fever (MEFV) gene. MEFV gene, M694V, was proved to carry greater risk of developing secondary amyloidosis, a potentially lethal complication. Authors claimed that environmental factors and country of origin might augment the risk of amyloidosis in infants and children with FMF.