Isr Med Assoc J
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Case Reports
"Trap" the diagnosis: a man with recurrent episodes of febrile peritonitis, not just familial Mediterranean fever.
Monogenic periodic fever syndromes are characterized by recurrent episodes of fever, accompanied by localized inflammatory manifestations. Among them, familial Mediterranean fever (FMF) is the most studied and is by far the most prevalent periodic fever syndrome in Israel. ⋯ Genetic tests confirmed the diagnosis. This case should alert us that even in Israel, a patient with periodic fever not fully consistent with the typical features of FMF, should be evaluated for other periodic fever syndromes.
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Amyloidosis of familial Mediterranean fever (FMF) may lead to end-stage renal failure, culminating in kidney transplantation in some patients. ⋯ Compliance with treatment and genetic makeup but not severity of FMF constitutes major risk factors for the development of amyloidosis in FMF. Transplantation seems to prevent FMF attacks. The protective role of immunosuppressive therapy cannot be excluded.