TABLE 1.

Demographic and therapeutic data of the cohort of patients with PFAPA syndrome recruited from the AIDA International Registry and treated with SSK12.

Patients, n°	85
Males/females	49/36
Median (IQR) age at last follow-up visit (years)	4.58 (3.42)
Median (IQR) age at onset ± (months)	24.00 (22.50)
Median (IQR) age at SSK12 start (months)	55.00 (43.00)
Median follow-up ± IQR (months)	6.00 (7.00)
Median (IQR) disease duration before SSK12 treatment (months)	19.00 (28.00)
Previous treatments	GCs (n = 75) NSAIDs and/or acetaminophen (n = 51) Colchicine (n = 1)
Concomitant treatments	GCs (n = 71) NSAIDs and/or acetaminophen (n = 32)
Genetic data	Patients undergoing genetic testing (n = 22) Mutations encountered (n = 6): [MEFV (E148Q); MEFV (E230K); MEFV (V726A); NLRP3 (c.2113C > A); TLR9; ALPK1]

ALPK1, Alpha-protein kinase 1; GCs, glucocorticoids; IQR, interquartile range; MEFV, Familial Mediterranean fever gene; NSAIDs, non-steroidal anti-inflammatory drugs; NLRP3, nucleotide-binding domain, Leucine-rich–containing family, Pyrin domain–containing-3; TLR9, toll-like receptor 9.