From: The 4th NextGen therapies of SJIA and MAS, part 4: it is time for IL-18 based trials in systemic juvenile idiopathic arthritis?
Disease
Gene
IL-18 biomarker
Evidence of response to IL-18 blockade
AOSD
n/a
[5, 17]
Phase II [29]
Systemic JIA
[4, 6,7,8,9, 16, 24, 26]
-
SJIA-MAS
“
Refractory SJIA
SJIA-LD
[26, 30,31,32]
Case report [33]
NLRC4-MAS/AIFEC
NLRC4
[18, 19, 34,35,36]
Case report [34]
XLP2
XIAP
[4, 20]
NOCARH
CDC42
[21, 22]
PAPA/PAID
PSTPIP1
[28, 37]