Table 3

Eurofever/PRINTO clinical classification criteria for PFAPA and hereditary recurrent fevers and their performance in the 281 for whom consensus was achieved

PFAPACAPSFMFTRAPSMKD
At least seven out of eight:
Presence
  • Pharyngotonsillitis.

  • Duration of episodes, 3–6 days.

  • Cervical lymphadenitis.

  • Periodicity.


Absence
  • Diarrhoea.

  • Chest pain.

  • Skin rash.

  • Arthritis.

Presence of at least two of five *:
  • Urticarial rash.

  • Cold/Stress-triggered episodes.

  • Sensorineural hearing loss.

  • Chronic aseptic meningitis.

  • Skeletal abnormalities (epiphysial overgrowth/frontal bossing).

At least six out of nine:
Presence
  • Eastern Mediterranean ethnicity.

  • Duration of episodes, 1–3 days.

  • Chest pain.

  • Abdominal pain.

  • Arthritis.


Absence
  • Aphthous stomatitis.

  • Urticarial rash.

  • Maculopapular rash.

  • Painful lymph nodes.

Score ≥5 points:
Presence
  • Fever ≥7 days (2 points).

  • Fever 5–6 days (1 point).

  • Migratory rash (1 point).

  • Periorbital oedema (1 point).

  • Myalgia (1 point).

  • Positive family history (1 point).


Absence
  • Aphthous stomatitis (1 point).

  • Pharyngotonsillitis (1 point).

Presence of at least three of six:
  • Age at onset <1 years.

  • Gastrointestinal symptoms.

  • Painful lymph nodes.

  • Aphthous stomatitis.

  • Triggers.

  • Maculopapular rash.

Sensitivity: 0.97Sensitivity: 0.80Sensitivity: 0.91Sensitivity: 0.87Sensitivity: 0.91
Specificity: 0.93Specificity: 0.91Specificity: 0.92Specificity: 0.92Specificity: 0.82
Accuracy: 0.99Accuracy: 0.85Accuracy: 0.97Accuracy: 0.96Accuracy: 0.92
  • *Modified by Kuemmerle-Deschner et al. 14 See online supplementary table 6 for glossary. See table 2 for prerequisite criteria.

  • CAPS, cryopyrin-associated periodic syndromes; FMF, familial Mediterranean fever; MKD, mevalonate kinase deficiency; PFAPA, periodic fever, aphthosis, pharyngitis and adenitis; PRINTO, pediatric rheumatology international trial organization; TRAPS, tumour necrosis factor receptor-associated periodic fever syndrome.