PEDIATRIC AND HERITABLE DISORDERS: Edited by Polly J. FergusonFamilial Mediterranean feverPadeh, Shaia; Berkun, YackovbAuthor Information aDepartment of Pediatrics A, Edmond and Lily Safra Children's Hospital, Sheba Medical Center, Tel Hashomer bDepartment of Pediatrics, Hadassah Hebrew University Medical Center, Mount Scopus, Jerusalem, Israel Correspondence to Shai Padeh, MD, Department of Pediatrics A, Edmond and Lily Safra Children's Hospital, Sheba Medical Center, Tel Hashomer 52621, Israel. Tel: +972 3 530 4430; fax: +972 3 547 4429; e-mail: [email protected] Current Opinion in Rheumatology: September 2016 - Volume 28 - Issue 5 - p 523-529 doi: 10.1097/BOR.0000000000000315 Buy Metrics Abstract Purpose of review Familial Mediterranean fever (FMF) is the oldest and most common of the hereditary autoinflammatory diseases (AIDs). A large body of information has been accumulated over recent years on the pathophysiology, diagnosis and treatment of these diseases. The purpose of this review is to bring an up-to-date summary of the clinic manifestations, diagnostic criteria and treatment of FMF. Recent findings An overview of the pathophysiologic basis of FMF as part of the AID is discussed. Over the last year, attempts to establish new criteria for childhood FMF, new guidelines for treatment and follow-up of disease and novel treatment for FMF were made. A comparison of the different disease severity scores for research purposes suggests that a new score is needed. New evidence for antiinterleukin-1 blockade as a new treatment modality is described. Summary New diagnostic criteria, disease severity score, treatment and follow-up guidelines have been proposed, and need validation in the next several years. Copyright © 2016 Wolters Kluwer Health, Inc. All rights reserved.