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Familial Mediterranean fever

Last reviewed: 20 Aug 2025
Last updated: 01 Apr 2025

Summary

Definition

病史和体格检查

关键诊断因素

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

其他诊断因素

  • 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
完整详情

危险因素

  • 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
完整详情

诊断性检查

首要检查

  • CBC
  • erythrocyte sedimentation rate
  • CRP
  • serum fibrinogen
  • serum amyloid A
  • LFTs
  • urine analysis
完整详情

需考虑的检查

  • LDH
  • chest x-ray
  • joint x-ray
  • abdominal CT
  • chest CT
  • echocardiogram
  • joint MRI
  • genetic testing
完整详情

治疗流程

急症处理

acute attack

持续性治疗

confirmed FMF

撰稿人

作者

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.

Peer reviewer acknowledgements

BMJ Best Practice topics are updated on a rolling basis in line with developments in evidence and guidance. The peer reviewers listed here have reviewed the content at least once during the history of the topic.

Disclosures

Peer reviewer affiliations and disclosures pertain to the time of the review.

References

Our in-house evidence and editorial teams collaborate with international expert contributors and peer reviewers to ensure that we provide access to the most clinically relevant information possible.

Key articles

Gattorno M, Hofer M, Federici S, et al. Classification criteria for autoinflammatory recurrent fevers. Ann Rheum Dis. 2019 Aug;78(8):1025-32.Full text  Abstract

Giancane G, Ter Haar NM, Wulffraat N, et al. Evidence-based recommendations for genetic diagnosis of familial Mediterranean fever. Ann Rheum Dis. 2015 Apr;74(4):635-41. Abstract

Ozen S, Demirkaya E, Erer B, et al. EULAR recommendations for the management of familial Mediterranean fever. Ann Rheum Dis. 2016 Apr;75(4):644-51. Abstract

Reference articles

A full list of sources referenced in this topic is available to users with access to all of BMJ Best Practice.
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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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