Familial Mediterranean fever (FMF) is
characterized by recurrent episode of fever, serositis, oligoarticular
arthritis, and rash, beginning between the ages of 5 and 15 years and
tending to occur every 2 to 4 weeks. Abdominal pain of short duration is
present in 90% of patients and represents acute peritonitis. Peritoneal
adhesions may form and cause small-bowel obstruction. Acute scrotal pain may
be a manifestation of FMF and should be distinguished from testicular
torsion. Pleuritis occurs in approximately 30% of cases and can lead to
recurrent atelectasis. Monoarticular arthritis involving large joints is
present in up to 70% of patients. The most serious complication of FMF is
amyloidosis of the AA type, which can lead to renal failure and death.
Splenomegaly is a common complication of amyloidosis; other organs are
rarely involved. Amyloidosis is mostly prevalent among Sephardic Jews.
Symptoms of an acute attack appear suddenly and last from a few hours up to
96 hours. The disease has a variable and unpredictable course in each
patient. Prophylactic therapy with colchicine prevents inflammatory attacks
and the development of amyloidosis. The therapy should be continued
throughout pregnancy and during lactation as long-term follow-up does not
reveal any adverse effect on the children. The association of FMF with
seronegative spondyloarthropathies remains controversial.