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Supplemental material
Ann Rheum Dis
DISCOVERY COHORT
VALIDATION COHORT
SSc-PAH
SSc-no PAH
SSc-PAH
SSc-no PAH
N
N
N
N
Sex (female), n (%) Age (yo), mean ( SD)
15
14 (93%)
16
14 (88%) 50 ( 14)
24
17 (71%)
17
15 (88%)
65 ( 7)
68 ( 9)
67 ( 7)
15
16
24
17
ANA , n (%)
13
13 (100%)
14
14 (100%)
17
17 (100%)
14
14 (100%)
- ACA, n (%)
12
9 (75%)
9
4 (44%)
8
7 (88%)
14
10 (71%)
- ATA, n (%)
12
0 (0%)
9
1 (11%)
8
0 (0%)
14
4 (29%)
- Anti-U1RNP, n (%)
12
1 (8%)
9
2 (22%)
8
0 (0%)
14
0 (0%)
Disease duration - since RP onset (y), mean ( SD)
19 ( 13)
13 ( 13)
18 ( 12)
24 ( 17)
10
11
8
15
- since first non-RP symptom onset (y), median [Q1;Q3] - since diagnosis (y), median [Q1;Q3]
8
8 [2;13]
10
10 [3;12]
6
7 [0;17]
14
7 [4;10]
15 1.25 [0.05;6]
16
7 [1;11]
9
0.5 [0;9] 28 ( 6)
6
7 [3;13]
BMI (kg/m 2 ), mean ( SD)
28 ( 4)
24 ( 5)
25 ( 4)
15
15
16
14
mRSS , median [Q1;Q3]
5
3 [2;5]
9
3 [2;5]
7
3 [3;5]
14
6 [3;8]
NYHA functional class
15
8
18
4
- class I, n (%)
0 (0%)
8 (100%)
0 (0%)
2 (50%)
- class II, n (%)
7 (47%)
0 (0%)
6 (33%)
2 (50%)
- class III, n (%)
8 (53%)
0 (0%)
10 (56%)
0 (0%)
- class IV, n (%)
0 (0%)
0 (0%)
2 (11%)
0 (0%)
BNP (pg/mL), median [Q1;Q3]
12
147 [69;505]
2
25 [24;27]
14
84 [39;264]
Nt-pro-BNP (pg/mL), median [Q1;Q3] eGFR (mL/min/1.73m 2 ), mean ( SD)
14 727 [319;1098]
5 126 [88;134]
68 ( 15) 60 ( 9) 72 ( 19) 85 ( 10)
78 ( 21) 64 ( 6) 26 ( 5)
82 ( 20) 65 ( 9) 59 ( 16) 88 ( 18)
74 ( 16) 63 ( 6) 28 ( 4)
12
7
16
15
LVEF (%), mean ( SD)
12
11
14
15
Estimated sPAP (mmHg), mean ( SD)
12
7
15
10
FVC (% predicted), mean ( SD)
16 110 ( 15)
13 111 ( 14)
13
20
DLCO (% predicted), median [Q1;Q3]
15
35 [8;52]
16
92 [87;109]
10
41 [33;65]
12
65 [59;82]
2022;0:1–9. doi: 10.1136/ard-2022-223237 Ann Rheum Dis , et al. Sanges S
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