Canakinumab treatment in four children with colchicine resistant familial mediterranean fever.
نویسندگان
چکیده
Familial Mediterranean Fever (FMF) is an autosomal recessive and autoinflammatory disease, characterized with inflammation of serous membranes such as peritoneum, pleura, synovium with fever and pain. Colchicine is the main treatment of FMF, but 5-10 % of patients are unresponsive to colchicine. We report using anti-interleukin-1 agents anakinra and canakinumab in four colchicine-resistant patients who were successfully treated. Three of the patients were siblings.
منابع مشابه
Canakinumab investigated for treating familial Mediterranean fever.
INTRODUCTION Familial Mediterranean fever (FMF) is the most common hereditary autoinflammatory syndrome. The treatment of choice is colchicine. However, ~40% of patients are only partial responders and 5-10% are non-responders. Advances in the understanding of the role of pyrin in the regulation of interleukin (IL)-1β activation has led to use of anti-IL-1 agents for colchicine-resistant FMF. ...
متن کاملPW01-015 – Canakinumab in adults with colchicin resistant FMF
Introduction Familial Mediterranean fever (FMF) is associated with variations in the MEFV gene resulting in proteolytic activation of IL-1b through the inflammasome complex. There is no established treatment available for those resistant or intolerant to standard of care colchicine treatment. Canakinumab, a fully human selective anti-IL-1b monoclonal antibody with a half-life of ~4-weeks binds ...
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Familial Mediterranean fever is an autosomal recessive autoinflammatory disorder mainly affecting Mediterranean populations, which is associated with mutations of the MEFV gene that encodes pyrin. Functional studies suggest that pyrin is implicated in the maturation and secretion of interleukin-1 (IL-1). The IL-1 receptor antagonist or anti-IL-1 monoclonal antibody may therefore represent a rat...
متن کاملThe experience of canakinumab in renal amyloidosis secondary to Familial Mediterranean fever
INTRODUCTION Familial Mediterranean fever (FMF) is an autosomal recessive disease characterized by self-limited recurrent attacks of fever and serositis. Patients may develop renal amyloidosis. Colchicine prevents attacks and renal amyloidosis. Five to 10 % of the patients with FMF are resistant or intolerant to colchicine. CASE DESCRIPTION Herein, we reported our experience with clinical-lab...
متن کاملBone Mineral Density in Egyptian Children with Familial Mediterranean Fever
Background: Familial Mediterranean fever (FMF) has episodic or subclinical inflammation that may lead to a decrease in bone mineral density (BMD). The objective of this study was to assess BMD in Egyptian children with FMF on genetic basis.Methods: A cross sectional study included 45 FMF patients and 25 control children of both sexes in the age range between 3-16 years old. The patients were re...
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عنوان ژورنال:
- JPMA. The Journal of the Pakistan Medical Association
دوره 67 6 شماره
صفحات -
تاریخ انتشار 2017