Research Article

Clinical Features at Onset and Genetic Characterization of Pediatric and Adult Patients with TNF-α Receptor—Associated Periodic Syndrome (TRAPS): A Series of 80 Cases from the AIDA Network

Table 3

Fulfillment of TRAPS Eurofever score according to Federici et al.. 2015 [13] (“TRAPS Eurofever score 2015”) and classification criteria according to Gattorno et al.. 2019 [9] (“genetic + clinical classificative criteria 2019” and “clinical classificative criteria 2019”) in the whole cohort of TRAPS patients and according to age at onset and TNFRSF1A mutations penetrance. List of abbreviations: CAPS: cryopyrin-associated periodic syndrome; FMF: familial Mediterranean fever; MKD: mevalonate kinases deficiency; n.a.: not available; n.s.: not significant; TNFRSF1A: tumor necrosis factor receptor super family member 1 A; TRAPS: tumor necrosis factor receptor-associated periodic syndrome.

Whole TRAPS populationPediatric-onset groupAdult-onset groupHigh-penetrance TNFRSF1A mutationsLow-penetrance TNFRSF1A mutations

TRAPS Eurofever score 2015 (%)71.375.066.7n.s.93.856.8<0.001
TRAPS genetic + clinical classificative criteria 2019 (%)80.084.175.0n.s.93.877.3n.s.
TRAPS clinical classificative criteria 2019 (%)57.561.455.9n.s.71.950.0n.s.
 Fever ≥7days (%)78.884.072.2n.s.87.579.5n.s.
 Fever 5–6 days (%)8.813.62.8n.s.6.311.4n.s.
 Migratory rash (%)6.34.58.3n.s.6.36.8n.s.
 Periorbital oedema (%)17.522.711.1n.s.15.615.9n.s.
 Myalgia (%)66.356.877.7n.s.56.372.7n.s.
 Positive family history (%)38.852.322.20.0156.325.00.01
 Absence of aphthous stomatitis (%)75.072.777.7n.s.90.668.20.02
 Absence of pharyngotonsillitis (%)70.065.975.0n.s.81.368.2n.s.
FMF clinical classificative criteria 2019 (%)12.54.523.50.0112.511.9n.s.
MKD clinical classificative criteria 2019 (%)12.520.52.90.026.314.3n.s.
CAPS clinical classificative criteria 2019 (%)000n.a.00n.a.
No clinical classificative criteria 2019 (%)252526.5n.s.15.633.3n.s.
More than one clinical set of criteria 2019 (%)11.313.68.8n.s.9.49.5n.s.