|
Number of cases
|
2/6
|
4/6
|
5/5
|
6/6
|
40/55
|
|
Age at sJIA onset (years)
|
0.6, 2.5
|
0.5, 1.6, 3.2, 9.9
|
0.5, 0.6, 2.5, 3.2, 9.9
|
0.5 to 9.9
|
Median 2.3 (IQR:1.2-5.1)
|
|
Prior to lung disease
|
| MAS≧1 episode |
0/2
|
0/4
|
0/5
|
0/6
|
29/40 (73%)
|
|
Period of disease quiescence on treatment2
|
0/2
|
1/4
|
0/5
|
1/6
|
18/40 (45%)
|
|
Atypical clinical findings occurring between 6 and 1 month prior to lung disease
|
|
Atypical rash
|
2/2
|
2/4
|
3/5
|
4/6
|
23/40 (58%)
|
|
Acute clubbing
|
1/2
|
3/4
|
3/5
|
4/6
|
31/40(78%)
|
|
Lymphopenia <60% lower limit of normal3
|
1/1
|
3/4
|
3/4
|
4/5
|
17/36(47%)
|
|
Tocilizumab reaction
|
1/2
|
0/4
|
1/4
|
1/5
|
13/28(46%)
|
|
Peripheral eosinophilia w/in prior year4
|
1/2
|
2/4
|
2/5
|
3/6
|
17/39(44%)
|
|
Clinical features at lung disease
|
|
Unexpected CT pattern(s) of diffuse lung disease5
|
0/1
|
4/4
|
4/5
|
4/5
|
35/39 (90%)
|
|
Hyper-enhancing lymph nodes on CT6
|
0/1
|
1/2
|
1/3
|
1/3
|
11/24 (46%)
|
|
Ferritin >1000ng/ml
|
1/1
|
2/4
|
2/4
|
3/5
|
14/27 (52%)
|
|
Overt MAS within prior 6 months
|
0/1
|
0/4
|
0/4
|
0/5
|
11/40 (28%)
|
|
Hypoxia
|
2/2
|
3/4
|
4/5
|
5/67
|
14/40 (35%)7
|
|
Abnormal chest auscultation
|
2/2
|
3/4
|
4/5
|
5/67
|
5/40 (13%)7
|
|
Viral infection
|
1/2
|
4/4
|
4/5
|
5/67
|
11/40 (28%)7
|
|
Pulmonary hypertension or vascular disease8
|
1/2
|
4/4
|
4/5
|
5/6
|
17/40(43%)
|
|
Features during lung disease
|
|
MAS
|
1/2
|
2/4
|
2/5
|
3/6
|
16/40(40%)
|
|
Alive at data close
|
1/2
|
2/4
|
2/5
|
3/6
|
28/40(70%)
|
