The efficacy of anakinra in an adolescent with colchicine-resistant familial Mediterranean fever
Open Access
- 23 June 2007
- journal article
- case report
- Published by Springer Nature in European Journal of Pediatrics
- Vol. 167 (6), 695-696
- https://doi.org/10.1007/s00431-007-0547-3
Abstract
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)-1β. 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.Keywords
This publication has 6 references indexed in Scilit:
- Diagnostics and Therapeutic Insights in a Severe Case of Mevalonate Kinase DeficiencyPediatrics, 2007
- Colchicine Use in Children and Adolescents With Familial Mediterranean Fever: Literature Review and Consensus StatementPediatrics, 2007
- Treatment options in colchicine resistant familial Mediterranean fever patients: thalidomide and etanercept as adjunctive agents.2006
- Neonatal-Onset Multisystem Inflammatory Disease Responsive to Interleukin-1β InhibitionNew England Journal of Medicine, 2006
- The B30.2 domain of pyrin, the familial Mediterranean fever protein, interacts directly with caspase-1 to modulate IL-1β productionProceedings of the National Academy of Sciences, 2006
- Familial mediterranean fever: revisiting an ancient diseaseEuropean Journal of Pediatrics, 2003