Do Biological Agents be Useful in the Treatment of Amyloidosis Related FMF?

Abstract

Familial Mediterranean Fever (FMF), is an autosomal recessive disease associated with mutations in the MEFV gene. A 13-year-old female patient was admitted to hospital with vomiting and increasing abdominal pain. Her physical examination showed generalized edema. Laboratory examination revealed proteinuria, hypoalbuminemia, hypercholesterolemia. Renal biopsy showed AA-type amyloidosis and homozygous M694V mutation was detected. Colchicine was started. However, heavy proteinuria persisted despite colchicine treatment and infliximab treatment was administered. Since patient developed edema, proteinuria, hypoalbuminemia, after tenth dose of infliximab therapy, resistance was considered and treatment was discontinued. Anakinra therapy was initiated. Anakinra treatment was stopped after six months due to the patient was rejected any more using that treatment and end stage renal failure was developed. Although biological agents may be useful in the treatment of amyloidosis caused by FMF, the progression of illness despite treatment with these agents has been reported in many patients.