These are classification criteria used in research and not necessarily to make the diagnosis in clinical practice.

Classification criteria Eurofever / PRINO 2019 (Gattorno M, 2019)

Genetic testing shows MEFV genotype and at least one of the following:

• Duration of episode 1-3 days
• Arthritis (joint inflammation)
• Chest pain
• Abdominal pain

OR

Absence of genetic test showing MEFV genotype and at least two of the following:

• Duration of episode 1-3 days
• Arthritis (joint inflammation)
• Chest pain
• Abdominal pain

Suggested criteria (Federci, S, PRINTO / Eurofever 2015)

Occurrence                                                  swath

• Episodes <2 days 9
• Chest pain 13
• Abdoninal pain 9
• Ethnicity * 22
• Absence of Oral stomatitis 9
• Absence of Urticarial eczema 15
• Absence of Enlarged throat lymph nodes 10
• Absence of duration of episodes> 6 days 13

  TOTAL SCORE at least 60

* Turkish, Armenian, Jewish, Arabic, Italian, Spanish, Greek

Sensitivity: 68%. Specificity: 87%

Livneh A (1997): Criteria: ≥1 major gives sensitivity to 57%, specificity on 99%:

• Major: Attaks with
  • Peritonitis
  • Pleurisy
  • Pericarditis
  • MonoArthritis (Hip, knee, ankle)
  • Or high fever alone
• Minor: Uncompleted attaks:
  • The abdomen (abdominal area)
  • Thorax (breast area)
  • Joints (Arthritis)
  • Bone Pain
  • Colchicine-repons
• Supportive: Family incidence, typical ethnicity, <20 years of age first episode, episodes require bed rest, spontaneous remission, asymptomatic intervals. Lab shows inflammation, episodes of ery- / proteinuria, Appendectomy without findings, Parents in the same family

• Familiar Mediterranean Fever (FMF)
• Bindevevssykdommer.no
• Vaskulitt.no