title: Evidence-based recommendations for the practical management of Familial Mediterranean Fever.
abstract: OBJECTIVE
  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.
  
  
  CONCLUSIONS
  This is a first attempt to resolve practical questions in the daily management of FMF patients.
authors.affiliation: 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.
authors.forename: Véronique
authors.initials: V
authors.lastname: Hentgen
authors.forename: Gilles
authors.initials: G
authors.lastname: Grateau
authors.forename: Isabelle
authors.initials: I
authors.lastname: Kone-Paut
authors.forename: Avi
authors.initials: A
authors.lastname: Livneh
authors.forename: Shai
authors.initials: S
authors.lastname: Padeh
authors.forename: Michael
authors.initials: M
authors.lastname: Rozenbaum
authors.forename: Serge
authors.initials: S
authors.lastname: Amselem
authors.forename: Ruth
authors.initials: R
authors.lastname: Gershoni-Baruch
authors.forename: Isabelle
authors.initials: I
authors.lastname: Touitou
authors.forename: Eldad
authors.initials: E
authors.lastname: Ben-Chetrit
mesh_terms: D000328:Adult; D002648:Child; D003078:Colchicine / Q000008:administration & dosage / Q000009:adverse effects / Q000627:therapeutic use*; D032921:Consensus; D004334:Drug Administration Schedule; D019317:Evidence-Based Medicine; D010505:Familial Mediterranean Fever / Q000188:drug therapy*; D006801:Humans
chemical_list: D003078:Colchicine
keywords: Biological agents; Colchicine; Colchicine resistance; Consensus development conference; Familial Mediterranean fever; Genetic analysis; Guidelines; Review; Siblings; Therapy