The Eurofever/PRINTO clinical classification for PFAPA and hereditary recurrent fevers

Periodic fever with aphthous stomatitis, pharyngitis, and adenitis	Cryopyrin-associated periodic syndromes	Familial Mediterranean fever	Tumor necrosis factor receptor-associated periodic fever syndrome	Mevalonate kinase deficiency
At least 7 out of 8: Presence: Pharyngotonsillitis Duration of episodes, 3 to 6 days Cervical lymphadenitis Periodicity Absence: Diarrhea Chest pain Skin rash Arthritis	Presence of at least 2 of 5: Urticarial rash Cold/stress-triggered episodes Sensorineural hearing loss Chronic aseptic meningitis Skeletal abnormalities (epiphysial overgrowth/frontal bossing)	At least 6 out of 9: Presence: Eastern Mediterranean ethnicity Duration of episodes, 1 to 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 to 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 3 of 6: Age at onset <1 year Gastrointestinal symptoms Painful lymph nodes Aphthous stomatitis Triggers Maculopapular rash
Sensitivity: 0.97	Sensitivity: 0.80	Sensitivity: 0.91	Sensitivity: 0.87	Sensitivity: 0.91
Specificity: 0.93	Specificity: 0.91	Specificity: 0.92	Specificity: 0.92	Specificity: 0.82
Accuracy: 0.99	Accuracy: 0.85	Accuracy: 0.97	Accuracy: 0.96	Accuracy: 0.92

PRINTO: Pediatric Rheumatology International Trial Organization.

Reproduced with permission from: Gattorno M, Hofer M, Federici S, et al. Classification criteria for autoinflammatory recurrent fevers. Ann Rheum Dis 2019; 78(8):1025-1032. Copyright © 2019 BMJ Publishing Group Ltd.

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The Eurofever/PRINTO clinical classification for PFAPA and hereditary recurrent fevers