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)-1beta. 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.
Titolo: | The efficacy of anakinra in an adolescent with colchicine-resistant familial Mediterranean fever |
Autori: | |
Data di pubblicazione: | 2007 |
Rivista: | |
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)-1beta. 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. |
Handle: | http://hdl.handle.net/11368/1702664 |
Digital Object Identifier (DOI): | http://dx.doi.org/10.1007/s00431-007-0547-3 |
URL: | http://dx.doi.org/10.1007/s00431-007-0547-3 |
Appare nelle tipologie: | 1.1 Articolo in Rivista |