Last reviewed:September 2019
Last updated:September  2017



History and exam

Key diagnostic factors

  • presence of risk 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
  • hx 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
  • male gender
  • positive family hx
  • 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

  • FBC
  • ESR
  • CRP
  • serum fibrinogen
  • serum amyloid A
  • LFTs
  • urine analysis
Full details

Investigations to consider

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

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 monograph.

Dr Erkan Demirkaya would like to gratefully acknowledge Dr VĂ©ronique Hentgen and Dr Ghaith Mitri, the previous contributors to this monograph. 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 a number of references cited in this monograph. 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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