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Semin Arthritis Rheum. 2013 Dec;43(3):387-91. doi: 10.1016/j.semarthrit.2013.04.011. Epub 2013 Jun 3.

Evidence-based recommendations for the practical management of Familial Mediterranean Fever.

Author information

1
Centre national de Référence des maladies Auto-Inflammatoires, CeRéMAI, Centre Hospitalier de Versailles, 177, rue de Versailles, 78150 Le Chesnay Cedex, France. Electronic address: Vhentgen@ch-versailles.fr.

Abstract

AIM:

Familial Mediterranean Fever (FMF) is the most common recurrent autoinflammatory fever syndrome. Still, many issues-e.g.: colchicine dosage adjustment, maximum dosage of colchicine in children and adults, definition of colchicine resistance, alternative treatment solutions in colchicine-resistant patients, and genetic screening for asymptomatic siblings-have not yet been standardized. The current paper aims at summarizing consensus recommendations to approach these issues.

METHODS:

A literature review concerning these practical management questions was performed through PubMed. On the basis of this analysis, expert recommendations were developed during a consensus meeting of caregivers from France and Israel.

RESULTS:

A patient experiencing more than four FMF attacks a year needs colchicine dose adjustment. In case of persistent attacks (≥6 per year) in patients with maximum doses of colchicine (2 mg in children; 3 mg in adults), alternative treatment to colchicine with IL1 inhibitors should be considered. Routine genetic testing for MEFV mutations in asymptomatic siblings of an index case is not recommended.

CONCLUSION:

This is a first attempt to resolve practical questions in the daily management of FMF patients.

KEYWORDS:

Biological agents; Colchicine; Colchicine resistance; Consensus development conference; Familial Mediterranean fever; Genetic analysis; Guidelines; Review; Siblings; Therapy

[Indexed for MEDLINE]

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