Table 3.

Eurofever/PRINTO classification criteria for the main four hereditary periodic fevers, published in 2019 for identifying patients with fevers recurring in a period of at least 6 months (combined with elevation of inflammatory parameters), who can be recruited for experimental studies.

Familial Mediterranean Fever (FMF)	Autosomal Dominant Familial Periodic Fever (Tumor Necrosis Factor Receptor-Associated Periodic Syndrome, TRAPS)	Cryopyrin-Associated Periodic Syndrome (CAPS)	Mevalonate Kinase Deficiency (MKD, Hyper-IgD Syndrome)
Presence of confirmatory MEFV genotype and at least 1 among: - Attacks lasting 1–3 days - Arthritis - Chest pain - Abdominal pain or Presence of a non-confirmatory MEFV genotype and at least 2 among: - Attacks lasting 1–3 days - Arthritis - Chest pain - Abdominal pain	Presence of confirmatory TNFRSF1A genotype and at least 1 among: - Attacks lasting ≥7 days - Myalgia - Migratory skin rash - Positive family history or Presence of a non-confirmatory TNFRSF1A genotype and at least 2 among: - Attacks lasting ≥7 days - Myalgia - Migratory skin rash - Positive family history	Presence of confirmatory NLRP3 genotype and at least 1 among: - Urticaria-like rash - Eye inflammation - Sensorineural hearing loss or Presence of a non-confirmatory NLRP3 genotype and at least 2 among: - Urticaria-like rash - Eye inflammation - Sensorineural hearing loss	Presence of confirmatory MVK genotype and at least 1 among: - Gastrointestinal symptoms - Cervical lymphadenopathy - Aphthous stomatitis