Ultrasonographic assessment reveals detailed distribution of synovial inflammation in Blau syndrome

Table 2 Conventional activity measures for joint inflammation, physical function, and the presence of camptodactyly

Patient number	Tender joint count		Swollen joint count		Global assessment VAS (/100 mm)		Acute inflammatory response (normal range^§)		DAS28 -ESR	DAS28 -CRP	MMP-3^§§ (ng/mL)	CHAQ	Campto- dactyly
	/28	/40	/28	/40	Patient/parent	Physician	ESR, mm/h	CRP, mg/L
1	NR^†	NR^†	0	0	0	2	3 (2 to 10)	3.0 (0 to 3)	NA	NA	27.2	0	No
2	0	0	22	24	10	5	6 (2 to 10)	1.0 (0 to 3)	2.7	2.7	85.3	0	No
3	0	0	0	0	0	3	29 (2 to 10)	32.0 (0 to 3)	2.4	2.2	24.9	0	No
4	0	0	1	1	8	6	19 (3 to 15)	0.7 (0 to 2.6)	2.5	1.5	64.0	0.13	Yes
5*	0	0	23	25	10	50	22 (1 to 10)	6.0 (0 to 3)	3.7	3.1	800.0	0	Yes
5**	0	0	7	9	0	23	3 (1 to 10)	0.0 (0 to 3)	1.5	1.7	21.8	0	Yes
6*	0	0	2	3	1	5	3 (5 to 13)	3.0 (0 to 2)	1.3	1.9	18.7	0	Yes
6**	0	0	2	2	2	1	2 (5 to 13)	0.4 (0 to 2)	0.9	1.5	18.8	0	Yes
7	0	0	1	1	0	0	27 (3 to 15)	1.4 (0 to 2.6)	2.6	1.6	426.4	0	Yes
8	4	4	13	13	65	45	8 (2 to 10)	1.0 (0 to 2.6)	4.5	4.3	36.9	0.63	Yes
9	0	0	0	0	15	8	4 (2 to 10)	0.0 (0 to 2.6)	1.2	1.2	216.8	1.63^‡	Yes
10	0	0	3	5	4	8	12 (2 to 10)	1.0 (0 to 3)	2.3	1.8	129.0	0.13^‡	Yes

*1^st ultrasound examination ; **2^nd ultrasound examination; ^†not reported because the patient was not able to understand and answer the question; ^‡health assessment questionnaire-damage index (HAQ-DI) for adult was obtained; ^§normal range in the institute for patient’s sex is provided; ^§§normal ranges are 36.9 to 121.0 ng/mL for males and 17.3 to 59.7 ng/mL for females. VAS, visual analogue scale; ESR, erythrocyte sedimentation rate; CRP, C-reactive protein; DAS28, disease activity score 28; MMP-3, matrix metalloproteinase-3; CHAQ, childhood-health assessment questionnaire; NR, not reported; NA, not applicable.

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