The 4th NextGen Therapies for SJIA and MAS: part 1 the elephant in the room: diagnostic/classification criteria for systemic juvenile idiopathic arthritis and adult-onset still’s disease

Table 2 Comparison of clinical features in pediatric and adult Still’s disease from representative series

Series	Schneider	Mozziconacci and Prieur	Russo	Pouchot	Gerfaud-Valentin	Ichida	Ruscitti	Ruscitti
Date	1992	2003/2004	2013	1991	2014	2014	2016	2022
Terminology	soJRA	soJRA	sJIA	ASD	AOSD	AOSD	AOSD	sJIA	AOSD
n	38	100	132	62	57	71	100	166	194
Age of onset (y)	6.3	4.5	5	24	36	32	45	5	41
female	55	51	59	45	53	65	66	52	47
Fever	100	100	100	100	95	99	100	100	98
Rash	90	90	71	87	77	79	78	73	73
Arthritis	90	80	100	94	95^a	93	86^a	100	60
Sore throat	NR	NR	NR	92	53	57	64	10	59
Persistent course	53	50	58	36	26	NR	33	22	14

Abbreviations: AOSD Adult-onset Still’s disease, ASD Adult Still’s disease, soJRA Systemic-onset juvenile rheumatoid arthritis, sJIA Systemic juvenile idiopathic arthritis. Age of onset is mean or median, depending on series
^aArthralgia or arthritis in 95%. References: Schneider [15], Mozziconacci and Prieur [16, 17], Russo [18], Pouchot [19], Gerfaud-Valentin [20], Ichida [21], Ruscitti 1996 [22], and Ruscitti 2022 [14]

ISSN: 1546-0096