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

• Published in: Seminars in arthritis and rheumatism Vol. 43; no. 3; pp. 387 - 391
• Main Authors: Hentgen, Véronique, Grateau, Gilles, Kone-Paut, Isabelle, Livneh, Avi, Padeh, Shai, Rozenbaum, Michael, Amselem, Serge, Gershoni-Baruch, Ruth, Touitou, Isabelle, Ben-Chetrit, Eldad
• Format: Journal Article
• Language: English
• Published: United States Elsevier Inc 01.12.2013; WB Saunders
• Subjects: Adult; Biological agents; Child; Colchicine; Colchicine - administration & dosage; Colchicine - adverse effects; Colchicine - therapeutic use; Colchicine resistance; Consensus; Consensus development conference; Drug Administration Schedule; Evidence-Based Medicine; Familial Mediterranean fever; Familial Mediterranean Fever - drug therapy; Genetic analysis; Genetics; Guidelines; Humans; Immunology; Life Sciences; Review; Rheumatology; Siblings; Therapy; Colchicine resistance; Therapy; Genetic analysis; Familial Mediterranean fever; Colchicine; Biological agents; Review; Consensus development conference; Siblings; Guidelines
• ISSN: 0049-0172; 1532-866X; 1532-866X
• DOI: 10.1016/j.semarthrit.2013.04.011

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. 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. 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 (2mg in children; 3mg 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. This is a first attempt to resolve practical questions in the daily management of FMF patients.