OR7-005 – Canakinumab in childhood colchicine resistant FMF
نویسندگان
چکیده
Introduction Familial Mediterranean Fever (FMF) is the most common hereditary autoinflammatory syndrome affecting >10,000 people in Israel. FMF is caused by mutations in the MEFV gene, which encodes for the pyrin protein that is part of the inflammation complex that activates IL-1b. Evidence from case reports/series and one controlled study supports IL-1 blockage as a potential treatment for FMF. Canakinumab (CAN) is a selective fully human monoclonal anti-IL-1b antibody.
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 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 کاملQuality of life changes with canakinumab therapy in adults with colchicine resistant FMF
Introduction Familial Mediterranean Fever (FMF), the most common form of the hereditary autoinflammatory disorders, is characterized by recurrent attacks of fever along with serosal or synovial inflammation lasting usually 12 to 72 hours. FMF is associated with impaired functional ability, and the persistent disabling features and chronic pain, emotional and physical limitations can have a nega...
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 ...
متن کاملCanakinumab treatment in four children with colchicine resistant familial mediterranean fever.
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 pati...
متن کاملذخیره در منابع من
با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید
عنوان ژورنال:
دوره 11 شماره
صفحات -
تاریخ انتشار 2013