Last reviewed: 6 Apr 2021
Last updated: 21 Nov 2019



History and exam

Key diagnostic factors

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

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

Risk factors

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

Diagnostic investigations

1st investigations to order

  • CBC
  • ¬†erythrocyte sedimentation rate
  • CRP
  • serum fibrinogen
  • serum amyloid A
  • LFTs
  • urine analysis

Investigations to consider

  • LDH
  • chest x-ray
  • joint x-ray
  • abdominal CT
  • chest CT
  • echo
  • joint MRI
  • genetic testing

Treatment algorithm


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 reviewersVIEW ALL

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.

Medical Unit for Autoinflammatory Diseases

Genetic Laboratory

Arnaud de Villeneuve Hospital




IT declares that she has no competing interests.

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