title: EULAR recommendations for the management of familial Mediterranean fever.
abstract: Familial Mediterranean fever (FMF) is the most common monogenic autoinflammatory disease, but many rheumatologists are not well acquainted with its management. The objective of this report is to produce evidence-based recommendations to guide rheumatologists and other health professionals in the treatment and follow-up of patients with FMF. A multidisciplinary panel, including rheumatologists, internists, paediatricians, a nurse, a methodologist and a patient representative, was assembled. Panellists came from the Eastern Mediterranean area, Europe and North America. A preliminary systematic literature search on the pharmacological treatment of FMF was performed following which the expert group convened to define aims, scope and users of the guidelines and established the need for additional reviews on controversial topics. In a second meeting, recommendations were discussed and refined in light of available evidence. Finally, agreement with the recommendations was obtained from a larger group of experts through a Delphi survey. The level of evidence (LoE) and grade of recommendation (GR) were then incorporated. The final document comprises 18 recommendations, each presented with its degree of agreement (0-10), LoE, GR and rationale. The degree of agreement was greater than 7/10 in all instances. The more controversial statements were those related to follow-up and dose change, for which supporting evidence is limited. A set of widely accepted recommendations for the treatment and monitoring of FMF is presented, supported by the best available evidence and expert opinion. It is believed that these recommendations will be useful in guiding physicians in the care of patients with FMF.
authors.affiliation: Department of Pediatric Rheumatology, Hacettepe University, Ankara, Turkey.
authors.forename: Seza
authors.initials: S
authors.lastname: Ozen
authors.affiliation: Division of Pediatric Rheumatology, Gülhane Military Medical Academy, FMF Arthritis Vasculitis & Orphan Disease Research Center (FAVOR), Ankara, Turkey.
authors.forename: Erkan
authors.initials: E
authors.lastname: Demirkaya
authors.affiliation: Division of Rheumatology, Department of Internal Medicine, Istanbul University, Istanbul Faculty of Medicine, Istanbul, Turkey.
authors.forename: Burak
authors.initials: B
authors.lastname: Erer
authors.affiliation: Heller Institute of Medical Research and Medicine F, Sheba Medical Center, Tel-Hashomer and Sackler Faculty of Medicine, Tel-Aviv University, Tel Aviv, Israel.
authors.forename: Avi
authors.initials: A
authors.lastname: Livneh
authors.affiliation: Rheumatology Unit, Hadassah-Hebrew University Medical Center, Jerusalem, Israel.
authors.forename: Eldad
authors.initials: E
authors.lastname: Ben-Chetrit
authors.affiliation: Pediatria II-Reumatologia, Istituto G. Gaslini, Genova, Italy.
authors.forename: Gabriella
authors.initials: G
authors.lastname: Giancane
authors.affiliation: Rheumatology Unit, Cerrahpaşa Medical Faculty, İstanbul, Turkey.
authors.forename: Huri
authors.initials: H
authors.lastname: Ozdogan
authors.affiliation: Heller Institute of Medical Research and Medicine F, Sheba Medical Center, Tel Aviv, Israel.
authors.forename: Illana
authors.initials: I
authors.lastname: Abu
authors.affiliation: UO Pediatria 2, Istituto G. Gaslini, Genova, Italy.
authors.forename: Marco
authors.initials: M
authors.lastname: Gattorno
authors.affiliation: National Amyloidosis Centre UCL and Royal Free Hospital, London, UK.
authors.forename: Philip N
authors.initials: PN
authors.lastname: Hawkins
authors.affiliation: Department of Radiation Oncology, Ankara Ataturk Chest Disease and Chest Surgery Training and Research Hospital, Ankara, Turkey.
authors.forename: Sezin
authors.identifier: http://orcid.org/0000-0003-2395-6868
authors.initials: S
authors.lastname: Yuce
authors.affiliation: Department of Pediatric Pneumology and Immunology, Charité University Medicine Berlin, Berlin, Germany.
authors.forename: Tilmann
authors.initials: T
authors.lastname: Kallinich
authors.affiliation: Pediatric Rheumatology Unit, Hacettepe University Faculty of Medicine, Ankara, Turkey.
authors.forename: Yelda
authors.initials: Y
authors.lastname: Bilginer
authors.affiliation: National Human Genome Research Institute, National Institutes of Health, Bethesda, Maryland, USA.
authors.forename: Daniel
authors.initials: D
authors.lastname: Kastner
authors.affiliation: Instituto de Salud Musculoesquelética, Madrid, Spain.
authors.forename: Loreto
authors.identifier: http://orcid.org/0000-0002-4401-2551
authors.initials: L
authors.lastname: Carmona
mesh_terms: D000686:Amyloidosis / Q000150:complications / Q000188:drug therapy; D000894:Anti-Inflammatory Agents, Non-Steroidal / Q000627:therapeutic use*; D018501:Antirheumatic Agents / Q000627:therapeutic use*; D056486:Chemical and Drug Induced Liver Injury / Q000175:diagnosis / Q000209:etiology; D003078:Colchicine / Q000627:therapeutic use*; D003697:Delphi Technique; D005060:Europe; D010505:Familial Mediterranean Fever / Q000175:diagnosis / Q000188:drug therapy*; D005938:Glucocorticoids / Q000627:therapeutic use*; D006801:Humans; D053590:Interleukin 1 Receptor Antagonist Protein / Q000627:therapeutic use; D050257:Tubulin Modulators / Q000627:therapeutic use*
chemical_list: D000894:Anti-Inflammatory Agents, Non-Steroidal; D018501:Antirheumatic Agents; D005938:Glucocorticoids; D053590:Interleukin 1 Receptor Antagonist Protein; D050257:Tubulin Modulators; D003078:Colchicine
keywords: Familial Mediterranean Fever; Multidisciplinary team-care; Treatment