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Familial Mediterranean fever

Last reviewed: 21 Nov 2024
Last updated: 31 Aug 2022

Summary

Definition

History and exam

Key diagnostic factors

  • periodic fever
  • abdominal pain
  • altered bowel habit
  • acute joint pain/swelling
  • response to colchicine
  • erysipelas-like skin lesions
Full details

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
Full details

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
Full details

Diagnostic tests

1st tests to order

  • CBC
  • erythrocyte sedimentation rate
  • CRP
  • serum fibrinogen
  • serum amyloid A
  • LFTs
  • urine analysis
Full details

Tests to consider

  • LDH
  • chest x-ray
  • joint x-ray
  • abdominal CT
  • chest CT
  • echocardiogram
  • joint MRI
  • genetic testing
Full details

Treatment algorithm

ACUTE

acute attack

ONGOING

confirmed FMF

Contributors

Authors

Erkan Demirkaya, MD, MSc, FRCPC

Professor of Paediatrics

Schulich School of Medicine & Dentistry

Department of Paediatrics

Division of Paediatric Rheumatology

University of Western Ontario

London

ON

Canada

Disclosures

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

Acknowledgements

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

Disclosures

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

Chief

Division of Rheumatology, Allergy and Clinical Immunology

Genome and Biomedical Sciences Facility

University of California at Davis

Davis

CA

Disclosures

MEG declares that he has no competing interests.

Isabelle Touitou, MD, PhD

Medical Unit for Autoinflammatory Diseases

Genetic Laboratory

Arnaud de Villeneuve Hospital

Montpellier

France

Disclosures

IT declares that she has no competing interests.

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  • Guidelines

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