| |
N (N for dcSSc)
|
Total
|
dcSSc
|
lcSSc
|
p
|
|---|
|
Demographics
|
|
Female sex
|
625/179
|
493 (79)
|
124 (69)
|
369 (83)
|
< 0.001
|
|
Age at first RP (years)
|
554/155
|
45.4 ± 15.7
|
45.8 ± 15.7
|
45.3 ± 15.8
|
0.736
|
|
Age at first non-RP symptom (years)
|
502/160
|
50.6 ± 14.5
|
48.5 ± 14.4
|
51.5 ± 14.4
|
0.031
|
|
Age at diagnosis (years)
|
625/179
|
52.7 ± 14.9
|
49.5 ± 14.5
|
53.9 ± 14.9
|
< 0.001
|
|
Disease duration from first non-RP symptom to diagnosis (years)
|
499/160
|
0.8 [2.2]
|
0.7 [1.4]
|
0.9 [2.7]
|
0.042
|
|
Follow-up time from inclusion to death or last visit (years)
|
625/179
|
4.4 [5.3]
|
4.0 [5.2]
|
4.8 [5.3]
|
0.023
|
|
Genetic background
|
|
European
|
503/147
|
453 (90)
|
118 (80)
|
335 (94)
|
< 0.001
|
|
African
|
503/147
|
50 (10)
|
29 (20)
|
21 (6)
|
< 0.001
|
|
Skin involvement
|
|
lcSSc
|
625/179
|
446 (71)
|
–
|
–
| |
|
mRSS
|
342/123
|
9.2 ± 10.2
|
19.6 ± 10.1
|
3.5 ± 3.6
|
< 0.001
|
|
Telangiectasia
|
572/160
|
264 (46)
|
65 (41)
|
199 (48)
|
0.112
|
|
Calcinosis
|
549/152
|
64 (12)
|
7 (5)
|
57 (14)
|
< 0.001
|
|
Digital ulcers (past or active)
|
538/145
|
161 (30)
|
66 (46)
|
95 (24)
|
< 0.001
|
|
Pulmonary involvement
|
|
NYHA
| | | | |
0.702
|
|
Classes I–II
|
515/150
|
425 (83)
|
122 (81)
|
303 (83)
| |
|
Classes III–IV
|
515/150
|
90 (17)
|
28 (19)
|
62 (17)
| |
|
6MWD (meters)
|
274/61
|
427 ± 127
|
432 ± 135
|
425 ± 125
|
0.705
|
|
TLC < 70% predicted
|
472/145
|
64 (14)
|
33 (23)
|
31 (9)
|
< 0.001
|
|
FVC < 70% predicted
|
475/148
|
82 (17)
|
44 (30)
|
38 (12)
|
< 0.001
|
|
DLCO < 70% predicted
|
471/141
|
249 (53)
|
102 (72)
|
147 (45)
|
< 0.001
|
|
Interstitial lung disease
|
582/166
|
262 (45)
|
115 (69)
|
147 (35)
|
< 0.001
|
|
PH (echo. and/or RHC)
|
547/157
|
67 (12)
|
18 (11)
|
49 (13)
|
0.775
|
|
sPAP (echo.)
| | | | |
0.004
|
|
< 35 mmHg
|
397/118
|
307 (77)
|
89 (75)
|
218 (78)
| |
|
35–46 mmHg
|
397/118
|
43 (11)
|
21 (18)
|
22 (8)
| |
|
> 46 mmHg
|
397/118
|
47 (12)
|
8 (7)
|
39 (14)
| |
|
PAH (RHC)
|
490/116
|
40 (8)
|
4 (3)
|
36 (10)
|
0.033
|
|
Heart involvement
|
|
Arrhythmia
|
519/150
|
17 (3)
|
5 (3)
|
12 (3)
|
1.000
|
|
AV block
|
512/146
|
7 (1)
|
4 (3)
|
3 (1)
|
0.106
|
|
BB block
|
479/128
|
16 (3)
|
6 (5)
|
10 (3)
|
0.388
|
|
LVEF (%)
|
402/102
|
64.9 ± 7.1
|
65.5 ± 8.6
|
64.7 ± 6.6
|
0.251
|
|
Diastolic dysfunction
|
423/110
|
20 (5)
|
6 (5)
|
14 (4)
|
0.613
|
|
Pericarditis
|
478/136
|
32 (7)
|
14 (10)
|
18 (5)
|
0.066
|
|
Valvular disease
|
430/111
|
29 (7)
|
5 (5)
|
24 (8)
|
0.380
|
|
Renal involvement
|
|
GFR < 80 ml/min
|
459/136
|
179 (39)
|
38 (28)
|
141 (44)
|
0.002
|
|
Scleroderma renal crisis
|
428/139
|
44 (10)
|
31 (22)
|
13 (5)
|
< 0.001
|
|
Gastrointestinal involvement
|
611/172
|
429 (70)
|
135 (78)
|
294 (67)
|
0.006
|
|
BMI (kg/m2)
|
514/159
|
24.4 ± 5.0
|
23.6 ± 4.0
|
24.7 ± 5.3
|
0.016
|
|
Albuminemia < 35 g/l
|
331/108
|
52 (16)
|
28 (26)
|
24 (11)
|
< 0.001
|
|
Muscular involvement
|
604/172
|
137 (23)
|
71 (41)
|
66 (15)
|
< 0.001
|
|
CPK > 200 IU/l
|
250/82
|
66 (26)
|
33 (40)
|
33 (20)
|
< 0.001
|
|
Joint involvement
|
598/172
|
291 (49)
|
127 (74)
|
164 (39)
|
< 0.001
|
|
Cancer
|
625/179
|
49 (8)
|
17 (10)
|
32 (7)
|
0.327
|
|
Hemoglobin
|
559/163
|
13.0 ± 1.6
|
12.5 ± 1.6
|
13.1 ± 1.5
|
< 0.001
|
|
Anemia
|
559/163
|
127 (23)
|
52 (32)
|
75 (19)
|
0.001
|
|
CRP > 8 mg/l
|
470/136
|
118 (25)
|
57 (42)
|
61 (18)
|
< 0.001
|
|
Serologic features
|
|
ACA
|
557/151
|
221 (40)
|
6 (4)
|
215 (53)
|
< 0.001
|
|
Anti-Scl70 antibodies
|
504/149
|
177 (35)
|
90 (60)
|
87 (25)
|
< 0.001
|
|
Anti-U1RNP antibodies
|
342/63
|
15 (4)
|
4 (6)
|
11 (4)
|
0.492
|
|
Anti-RNAP3 antibodies
|
345/72
|
18 (5)
|
13 (18)
|
5 (2)
|
< 0.001
|
|
Anti-PMScl antibodies
|
343/62
|
16 (5)
|
3 (5)
|
13 (5)
|
1.000
|
|
Anti-SSa antibodies
|
387/79
|
60 (16)
|
20 (25)
|
40 (13)
|
0.014
|
|
Anti-SSb antibodies
|
338/62
|
11 (3)
|
3 (5)
|
8 (3)
|
0.431
|
|
APL antibodies
|
441/129
|
31 (7)
|
16 (12)
|
15 (5)
|
0.007
|
|
Low complement
|
482/130
|
18 (4)
|
6 (5)
|
12 (3)
|
0.589
|
|
Smoking
|
572/158
|
215 (38)
|
69 (44)
|
146 (35)
|
0.067
|
- Results are expressed as n (%) for qualitative variables and mean ± SD or median [IQR] for quantitative variables
- N number of patients with available data, lcSSc limited cutaneous systemic sclerosis, mRSS modified Rodnan score, GFR glomerular filtration rate, AV block atrioventricular block, BB block bundle branch block, LVEF left ventricular ejection fraction, PH pulmonary hypertension, PAH pulmonary arterial hypertension, echo echocardiography, RHC right heart catheterization, 6MWD 6-min walking distance, sPAP systolic pulmonary arterial pressure, TLC total lung capacity, FVC forced vital capacity, DLCO diffusing capacity of the lungs for carbon monoxide, CRP C reactive protein, BMI body mass index, ACA anti-centromere antibodies, APL antiphospholipid antibodies
