The 4th NextGen therapies of SJIA and MAS, part 4: it is time for IL-18 based trials in systemic juvenile idiopathic arthritis?

Table 2 The proposed “IL-18opathies”

Disease	Gene	IL-18 biomarker	Evidence of response to IL-18 blockade
AOSD	n/a	[5, 17]	Phase II [29]
Systemic JIA	n/a	[4, 6,7,8,9, 16, 24, 26]	-
SJIA-MAS	n/a	“	-
Refractory SJIA	n/a	“	-
SJIA-LD	n/a	[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]	-

AOSD Adult-Onset Still Disease, SJIA-LD SJIA associated lung disease, AIFEC Autoinflammation with infantile enterocolitis, XLP X-linked lymphoproliferative disease, NOCARH Neonatal-onset cytopenia, autoinflammation, rash, hemophagocytosis, PAPA Pyogenic arthritis, pyoderma gangrenosum, acne syndrome, PAID PSTPIP1-associated inflammatory disease, CR Case report

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