Familial Mediterranean fever

Curr Opin Rheumatol. 2016 Sep;28(5):523-9. doi: 10.1097/BOR.0000000000000315.


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.

Publication types

  • Review

MeSH terms

  • Antibodies, Monoclonal / therapeutic use
  • Antibodies, Monoclonal, Humanized
  • Antirheumatic Agents / therapeutic use*
  • Colchicine / therapeutic use
  • Familial Mediterranean Fever / complications
  • Familial Mediterranean Fever / drug therapy*
  • Familial Mediterranean Fever / genetics
  • Familial Mediterranean Fever / physiopathology
  • Fever / etiology
  • Fever / physiopathology
  • Hereditary Autoinflammatory Diseases
  • Humans
  • Interleukin 1 Receptor Antagonist Protein / therapeutic use
  • Peritonitis / etiology
  • Peritonitis / physiopathology
  • Pleurisy / etiology
  • Pleurisy / physiopathology
  • Pyrin / genetics
  • Recombinant Fusion Proteins / therapeutic use
  • Synovitis / etiology
  • Synovitis / physiopathology
  • Tubulin Modulators / therapeutic use*


  • Antibodies, Monoclonal
  • Antibodies, Monoclonal, Humanized
  • Antirheumatic Agents
  • Interleukin 1 Receptor Antagonist Protein
  • MEFV protein, human
  • Pyrin
  • Recombinant Fusion Proteins
  • Tubulin Modulators
  • canakinumab
  • rilonacept
  • Colchicine