Familial Mediterranean fever (FMF) is the most frequent hereditary inflammatory 
disease characterized by self-limited recurrent attacks of fever and serositis. 
It is transmitted in an autosomal recessive pattern and affects certain ethnic 
groups mainly Jews, Turks, Arabs, and Armenians. FMF is caused by mutations in 
MEFV gene, which encodes pyrin. This protein is expressed mainly in 
myeloid/monocytic cells and modulates IL-1beta processing, NF-kappaB activation, 
and apoptosis. A mutated pyrin probably results in uncontrolled inflammation. 
The most devastating complication of FMF is amyloidosis, leading to chronic 
renal failure. M694V homozygocity, male gender and the alpha/alpha genotype of 
serum amyloid A1 gene are the currently established risk factors for development 
of amyloidosis. Daily colchicine is the mainstay of the therapy for the disease, 
resulting in complete remission or marked reduction in the frequency and 
duration of attacks in most patients. It is also effective in preventing and 
arresting renal amyloidosis.