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Familial Mediterranean fever

Last reviewed: 5 Sep 2023
Last updated: 31 Aug 2022



History and exam

Key diagnostic factors

  • periodic fever
  • abdominal pain
  • altered bowel habit
  • acute joint pain/swelling
  • response to colchicine
  • erysipelas-like skin lesions
More key diagnostic factors

Other diagnostic factors

  • pleuritic chest pain
  • acute myalgia
  • hepatosplenomegaly
  • history of appendectomy
  • abdominal scars
  • chronic joint pain
  • pleural rub
  • chronic myalgia/fibromyalgia
  • headache
  • neck stiffness
  • photophobia
  • acute scrotal pain + swelling
  • visual disturbances
Other diagnostic factors

Risk factors

  • Mediterranean ancestry
  • age <20 years old
  • male sex
  • positive family history
  • genetic mutation in MEFV gene
  • country of origin: Armenia, Turkey, or the Mediterranean basin
  • psychological stress
  • viral illness
  • physical exertion
  • extreme exhaustion
  • use of vasoconstrictor sympathomimetics
More risk factors

Diagnostic investigations

1st investigations to order

  • CBC
  • erythrocyte sedimentation rate
  • CRP
  • serum fibrinogen
  • serum amyloid A
  • LFTs
  • urine analysis
More 1st investigations to order

Investigations to consider

  • LDH
  • chest x-ray
  • joint x-ray
  • abdominal CT
  • chest CT
  • echocardiogram
  • joint MRI
  • genetic testing
More investigations to consider

Treatment algorithm


acute attack


confirmed FMF



Erkan Demirkaya, MD, MSc, FRCPC

Professor of Paediatrics

Schulich School of Medicine & Dentistry

Department of Paediatrics

Division of Paediatric Rheumatology

University of Western Ontario





ED is an author of a number of references cited in this topic.


Dr Erkan Demirkaya would like to gratefully acknowledge Dr Véronique Hentgen and Dr Ghaith Mitri, the previous contributors to this topic.


VH has been reimbursed by Novartis Pharmaceuticals (the manufacturer of canakinumab) for attending several conferences, has received consulting fees from Novartis Pharmaceuticals, and is an author of references cited in this topic. GM declares that he has no competing interests.

Peer reviewers

M. Eric Gershwin, MD

Distinguished Professor of Medicine

The Jack and Donald Chia Professor of Medicine


Division of Rheumatology, Allergy and Clinical Immunology

Genome and Biomedical Sciences Facility

University of California at Davis




MEG declares that he has no competing interests.

Isabelle Touitou, MD, PhD

Medical Unit for Autoinflammatory Diseases

Genetic Laboratory

Arnaud de Villeneuve Hospital




IT declares that she has no competing interests.

  • Differentials

    • Acute sarcoidosis
    • Rheumatoid arthritis
    • Vasculitis
    More Differentials
  • Guidelines

    • EULAR recommendations for the management of familial Mediterranean fever
    • Evidence-based recommendations for genetic diagnosis of familial Mediterranean fever
    More Guidelines
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