Table 3

The Eurofever clinical diagnostic/classification criteria*

FMFMKDCAPSTRAPS
PresenceScorePresenceScorePresenceScorePresenceScore
Duration of episodes < 2 days9Age at onset <2 years10Urticarial rash25 Periorbital oedema21
Chest pain13Aphthous stomatitis11Neurosensorial hearing loss25Duration of episodes >6 days19
Abdominal pain9Generalised enlargement of lymph nodes or splenomegaly8Conjunctivitis10Migratory rash†18
Eastern Mediterranean‡ ethnicity22Painful lymph nodes13Myalgia6
North Mediterranean‡ ethnicity7Diarrhoea (sometimes/often)20Relatives affected7
Diarrhoea (always)37
AbsenceAbsenceAbsenceAbsence
Aphthous stomatitis9Chest pain11Exudative pharyngitis25Vomiting14
Urticarial rash15Abdominal pain15Aphthous stomatitis15
Enlarged cervical lymph nodes10
Duration of episodes >6 days13
Cut-off≥60Cut-off≥42Cut-off≥52Cut-off≥43
  • *The clinical features should be related to the typical fever episodes (ie, exclusion of intercurrent infection or other comorbidities).†Centrifugal migratory, erythematous patches most typically overlying a local area of myalgia, usually on the limbs or trunk.

  • ‡Eastern Mediterranean: Turkish, Armenian, non-Ashkenazi Jewish, Arab. North Mediterranean: Italian, Spanish, Greek.

  • CAPS, cryopyrin-associated periodic syndromes; FMF, familial Mediterranean fever; MKD, mevalonate kinase deficiency; TRAPS, receptor-associated periodic fever syndrome.