title: The efficacy of anakinra in an adolescent with colchicine-resistant familial Mediterranean fever.
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.
authors.affiliation: Department of Pediatrics, Institute of Child Health, Children's Hospital IRCCS Burlo Garofolo, Via dell'Istria 65/1, 34137 Trieste, Italy.
authors.forename: Lorenzo
authors.initials: L
authors.lastname: Calligaris
authors.forename: Federico
authors.initials: F
authors.lastname: Marchetti
authors.forename: Alberto
authors.initials: A
authors.lastname: Tommasini
authors.forename: Alessandro
authors.initials: A
authors.lastname: Ventura
mesh_terms: D000293:Adolescent; D018501:Antirheumatic Agents / Q000627:therapeutic use*; D003078:Colchicine / Q000494:pharmacology / Q000627:therapeutic use; D004351:Drug Resistance; D010505:Familial Mediterranean Fever / Q000188:drug therapy*; D005260:Female; D006074:Gout Suppressants / Q000494:pharmacology / Q000627:therapeutic use; D006801:Humans; D053590:Interleukin 1 Receptor Antagonist Protein / Q000627:therapeutic use*
chemical_list: D018501:Antirheumatic Agents; D006074:Gout Suppressants; D053590:Interleukin 1 Receptor Antagonist Protein; D003078:Colchicine