Top

Published in:

Open Access 01-06-2008 | Short Report

The efficacy of anakinra in an adolescent with colchicine-resistant familial Mediterranean fever

Authors: Lorenzo Calligaris, Federico Marchetti, Alberto Tommasini, Alessandro Ventura

Published in: European Journal of Pediatrics | Issue 6/2008

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.

Chae JJ, Wood G, Masters SL, Richard K, Park G, Smith JB, Kastner DL (2006) The B30.2 domain of pyrin, the familial Mediterranean fever protein, interacts directly with caspase-1 to modulate IL-1β production. Proc Natl Acad Sci USA 103:9982–9987PubMedCrossRef

Goldbach-Mansky R, Dailey NJ, Canna SW, Gelabert A, Jones J, Rubin BI, Kim HJ, Brewer C, Zalewski C, Wiggs E, Hill S, Turner ML, Karp BI, Aksentijevich I, Pucino F, Penzak SR, Haverkamp MH, Stein L, Adams BS, Moore TL, Fuhlbrigge RC, Shaharm B, Jarvis JN, O’Neil K, Vehe RK, Beitz LO, Gardner G, Hannan WP, Warren RW, Horn W, Cole JL, Paul SM, Hawkins PN, Pham TH, Snyder C, Wesley RA, Hoffmann SC, Holland SM, Butman J, Kastner DL (2006) Neonatal-onset multisystem inflammatory disease responsive to interleukin-1β inhibition. N Engl J Med 355:581–592PubMedCrossRef

Kallinich T, Haffner D, Niehues T, Huss K, Lainka E, Neudorf U, Schafer C, Stojanov C, Timmann C, Keitzer R, Ozdogan H, Ozen S (2007) Colchicine use in children and adolescents with familial Mediterranean fever: literature review and consensus statement. Pediatrics 119:e474–e483PubMedCrossRef

Nevyjel M, Pontillo A, Calligaris L, Tommasini A, D’Osualdo A, Watheram HR, Granzotto M, Crovella S, Barbi E, Ventura A (2007) Diagnostic and therapeutic insights in a severe case of mevalonate kinase deficiency. Pediatrics 119:e523–e527PubMedCrossRef

Ozen S (2003) Familial mediterranean fever: revisiting an ancient disease. Eur J Pediatr 162(7–8):449–454PubMedCrossRef

Seyahi E, Ozdogan H, Celik S, Ugurlu S, Yazici H (2006) Treatment options in colchicine resistant familial Mediterranean fever patients: thalidomide and etanercept as adjunctive agents. Clin Exp Rheumatol 24(5 Suppl 42):S099–S103

Title: The efficacy of anakinra in an adolescent with colchicine-resistant familial Mediterranean fever
Authors: Lorenzo Calligaris
Federico Marchetti
Alberto Tommasini
Alessandro Ventura
Publication date: 01-06-2008
Publisher: Springer-Verlag
Published in: European Journal of Pediatrics / Issue 6/2008
Print ISSN: 0340-6199
Electronic ISSN: 1432-1076
DOI: https://doi.org/10.1007/s00431-007-0547-3

Keynote webinar | Spotlight on medication adherence

Springer Medicine

The efficacy of anakinra in an adolescent with colchicine-resistant familial Mediterranean fever

Abstract

Keynote webinar | Spotlight on medication adherence

Springer Medicine

Abstract

Please log in to get access to this content

Other articles of this Issue 6/2008

Uncomplicated outcome after an accidental overdose of nevirapine in a newborn

Acute myeloid leukaemia presenting as recurrent generalized urticaria in infancy

Adult height in children with short stature and idiopathic delayed puberty after different management

Heterozygous ABCA3 mutation associated with non-fatal evolution of respiratory distress

Imerslund-Gräsbeck syndrome in a 15-year-old German girl caused by compound heterozygous mutations in CUBN

Haemophilus paraphrophilus, a rare cause of intracerebral abscess in children