Journal
EUROPEAN JOURNAL OF PEDIATRICS
Volume 167, Issue 6, Pages 695-696Publisher
SPRINGER
DOI: 10.1007/s00431-007-0547-3
Keywords
familial Mediterranean fever; colchicine resistance; anakinra
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Colchicine is the treatment of choice in familial Mediterranean fever (FMF) for the prevention of both attacks and secondary amyloidosis. The overall nonresponder rate is about 5-10%. Anakinra is known to have good effectiveness in a severe autoinflammatory syndrome [chronic infantile neurological cutaneous and articular (CINCA) syndrome] and other recurrent hereditary periodic fevers. Pyrin-the protein involved in FMF-has a role in activating the proinflammatory cytokine interleukin (IL)-1 beta. We report the effectiveness of the addition of an IL-1-receptor inhibitor (anakinra) to colchicine in controlling the febrile attacks and acute phase response in an adolescent with FMF resistant to colchicine.
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