Male sex coupled with articular manifestations cause a 4-fold increase in susceptibility to amyloidosis in patients with familial Mediterranean fever homozygous for the M694V-MEFV mutation.

Abstract excerpt

OBJECTIVE: Familial Mediterranean fever (FMF) is an autosomal recessive disorder characterized by attacks of fever, serositis, and a predisposition to the development of amyloidosis. The wide clinical variability of the disease has been partly attributed to MEFV allelic heterogeneity and partly to the influence of additional genetic and/or environmental modifiers. Of these, male sex was found to influence disease...

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