Familial Mediterranean fever.
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.



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.



New diagnostic criteria, disease severity score, treatment and follow-up guidelines have been proposed, and need validation in the next several years.
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.
Shai
S
Padeh
Yackov
Y
Berkun
D000911:Antibodies, Monoclonal / Q000627:therapeutic use; D061067:Antibodies, Monoclonal, Humanized; D018501:Antirheumatic Agents / Q000627:therapeutic use*; D003078:Colchicine / Q000627:therapeutic use; D010505:Familial Mediterranean Fever / Q000150:complications / Q000188:drug therapy* / Q000235:genetics / Q000503:physiopathology; D005334:Fever / Q000209:etiology / Q000503:physiopathology; D056660:Hereditary Autoinflammatory Diseases; D006801:Humans; D053590:Interleukin 1 Receptor Antagonist Protein / Q000627:therapeutic use; D010538:Peritonitis / Q000209:etiology / Q000503:physiopathology; D010998:Pleurisy / Q000209:etiology / Q000503:physiopathology; D000071198:Pyrin / Q000235:genetics; D011993:Recombinant Fusion Proteins / Q000627:therapeutic use; D013585:Synovitis / Q000209:etiology / Q000503:physiopathology; D050257:Tubulin Modulators / Q000627:therapeutic use*
D000911:Antibodies, Monoclonal; D061067:Antibodies, Monoclonal, Humanized; D018501:Antirheumatic Agents; D053590:Interleukin 1 Receptor Antagonist Protein; C000604192:MEFV protein, human; D000071198:Pyrin; D011993:Recombinant Fusion Proteins; D050257:Tubulin Modulators; C541220:canakinumab; C531377:rilonacept; D003078:Colchicine