Anakinra for secondary amyloidosis in an adolescent with FMF and Behcet Disease
نویسندگان
چکیده
Familial Mediterranean Fever (FMF) is associated with mutation in the gene coding for pyrin which lead to accentuated innate immune responses involving the IL1 and probably Th1 pathways. We present a teenager who had FMF and Behçet disease and developed secondary amyloidosis. We hypothesized that anti-IL1 treatment would be beneficial for both controlling the disease activity and maintaining renal function.
منابع مشابه
Colchicine-resistant familial Mediterranean fever in a renal transplantation patient: successful treatment with anakinra
Familial Mediterranean fever (FMF) is an autosomal recessively inherited autoinflammatory disease characterized by recurrent fever, abdominal pain and arthritis. It is common in Turks, Arabs and non-Ashkenazi Jews, and uncontrolled disease can result in AA amyloidosis [1]. Colchicine is a microtubule -depolymerizing drug widely used in the treatment of gout arthritis and in the prevention of FM...
متن کاملDramatic beneficial effect of interleukin-1 inhibitor treatment in patients with familial Mediterranean fever complicated with amyloidosis and renal failure.
BACKGROUND Familial Mediterranean fever (FMF) is an autosomal recessive autoinflammatory disorder, for which systemic AA amyloidosis is the major complication revealed most of the time by renal abnormalities. Current treatment is daily colchicine that prevents both recurrent inflammatory attacks and amyloidosis deposition in most patients. However, some patients still develop amyloidosis and re...
متن کاملP01-031 – Anakinra for colchicine resistant FMF
Methods We plan to include patients, agreeing with clinical and genetic diagnosis of FMF, who suffer from FMF attacks, at least once per month, in one of the sites commonly involved by FMF (Chest, abdomen, lower extremity large joints, and skin), despite treatment with colchicine 2 mg/ day or less (in case of colchicine intolerance). Involvement with other diseases relevant (vasculitis, spondyl...
متن کاملFMF Genotype-phenotype correlation in Iranian Azeri Turks: Association between M694V/R761H mutation and amyloidosis
Objective(s):Familial Mediterranean fever (FMF), an inherited autosomal recessive disorder, is frequently present among individuals of Mediterranean origin. Differences in the clinical manifestations of FMF between different ethnic groups have been documented. The aim of the present study was to determine the most common characteristics of FMF and the relationship between clinical findings and ...
متن کاملAnti-IL1 therapy in patients with refractory FMF living inGermany
Results Within our cohort of 160 adult patients with FMF, we identified 15 patients (4 female and 11 male) who were treated with anakinra (n=13) or canakinumab (n=2). Twelve of 15 patients (80%) were of turkish-armenian ancestry. The median FMF severity score was 8 (range 5-14). Patients carrying two high-penetrance MEFV mutations (M694V or M680I) had a severity score of 9 (8/15=53%). Patients ...
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