Table 4.

Application of preliminary clinical score to patients and controls

Clinical preliminary score	Probable Autoinflammatory interferonopathies, n = 12	JIA, n = 6	JSLE, n = 4	DADA2, n = 5	JDM, n = 4
Skin manifestations	12/12	0/6	0/4	1/5	1/4
Vasculopathy	6/12	0/6	0/4	1/5	0/4
Lipodystrophy	2/12	0/6	0/4	0/5	0/4
Joint manifestations	5/12	0/6	0/4	0/5	2/4
Myositis	3/12	0/6	0/4	0/5	0/4
CNS manifestations	8/12	0/6	0/4	0/5	0/4
Pulmonary involvement	2/12	0/6	0/4	0/5	1/4
Leukopenia/lymphopenia with flares	4/12	0/6	4/4	1/5	0/4
Clinical score, median (min–max)	3 (3–5)	0 (0)	1 (0–1)	0 (0–1)	1 (0–2)

All items were scored as no (0) or yes (1)

CNS central nervous system, min–max minimum–maximum