Table 1 Open in new tab Demographic and...

Characteristics

Patients with data available, n

Total lcSSc (N = 827)

ACA-lcSSc (n = 537)

P-value

ATA-lcSSc (n = 194)

P-value

ANA-lcSSc (n = 96)

Female, n (%)

827

740 (89.5)

498 (92.7)

<0.0001

161 (83)

0.766

81 (84.4)

Age at RP onset, years

820

45.6 (15.2)

46.9 (15.3)

0.284

44.2 (15.2)

0.423

46.3 (14.5)

Time from RP to SSc, years, median (IQR)

820

1.9 (0.1–7.9)

2.5 (0.3–9.9)

<0.0001

0.7 (0–3)

0.978

0.8 (0–4)

Age at SSc onset, years

827

51.4 (13.7)

53 (13.3)

<0.0001

46.9 (14.2)

0.043

51.1 (13.3)

Age, years

827

52.8 (13.7)

54.4 (13.3)

<0.0001

48.4 (14.3)

0.05

52.4 (13.3)

Disease duration, years

827

1.4 (0.9)

1.000

1.4 (0.9)

1.000

1.4 (0.8)

BMI

455

25.4 (4.9)

25.6 (5.1)

1.000

25.5 (4.7)

1.000

25.1 (4.4)

ESR >20 mm/h^a, n (%)

396

113 (28.5)

65 (26)

0.08

36 (35.3)

0.344

12 (27.3)

CRP elevation, n (%)

381

58 (13.2)

25 (8.8)

<0.0001

25 (23.6)

0.665

8 (16.7)

Hb^a, g/dl

13.2 (1.3)

1.000

13.3 (1.3)

0.928

13.7 (1.0)

mRSS

752

4.8 (4.2)

4.3 (4)

0.001

5.8 (4.8)

1.000

5.1 (3.9)

Puffy fingers (ever), n (%)

451

207 (45.9)

123 (42.4)

0.025

62 (56.4)

0.236

22 (43.1)

DUs ever, n (%)

820

219 (26.7)

141 (26.5)

0.893

59 (30.7)

1.000

19 (20.0)

DUs current, n (%)

820

200 (24.4)

130 (24.4)

0.759

53 (27.6)

0.315

17 (17.9)

Telangiectasia^a, n (%)

138

67 (48.6)

49 (55.1)

0.030

10 (30.3)

0.360

8 (50)

Joint synovitis, n (%)

821

97 (11.8)

46 (8.6)

0.002

33 (17.2)

1.000

18 (18.8)

TFRs, n (%)

819

25 (3.1)

9 (1.7)

0.008

11 (5.7)

1.000

6 (6.3)

Muscle weakness, n (%)

821

110 (13.4)

52 (9.8)

<0.0001

41 (21.1)

0.985

17 (17.7)

CK elevation, n (%)

783

55 (7.0)

18 (3.6)

<0.0001

25 (13.3)

1.000

12 (13.3)

GI involvement, n (%)

826

492 (59.6)

338 (63.1)

0.015

101 (52.1)

1.000

53 (55.2)

GERD, n (%)

824

463 (56.2)

323 (60.5)

0.005

93 (47.9)

1.000

47 (49.0)

Stomach symptoms, n (%)

822

123 (15)

74 (13.9)

0.922

31 (16.1)

1.000

18 (18.8)

Intestinal symptoms, n (%)

824

145 (17.6)

94 (17.6)

1.000

33 (17)

1.000

18 (18.8)

SRC, n (%)

823

7 (0.9)

2 (0.4)

0.581

2 (1)

0.395

3 (3.1)

Serum creatinine^a, mg/dl, median (IQR)

0.7 (0.7–0.9)

0.8 (0.7–0.9)

1.000

0.7 (0.7–0.8)

0.054

0.65 (0.55–0.7)

Proteinuria, n (%)

781

22 (2.8)

8 (1.6)

0.011

10 (5.4)

1.000

4 (4.4)

ILD, n (%)

784

163 (20.8)

52 (10.3)

<0.0001

87 (46.3)

0.004

24 (27.0)

Restrictive LD, n (%)

783

97 (12.4)

43 (8.4)

<0.0001

39 (21.4)

0.628

15 (16.3)

FVC, % predicted, median (IQR)

417

103 (91–115)

106 (95–118)

<0.0001

95 (84.5–106)

0.357

101 (85–114)

TLC^a, % predicted, median (IQR)

287

99 (88–110)

100 (90–111)

0.012

93 (81–107)

0.799

97 (82–110)

DLCO, % predicted, median (IQR)

656

77 (64–89)

78 (66–90)

0.006

72 (59–86)

1.000

71 (58–90)

FEV1^a, % predicted, median (IQR)

289

99 (86–109)

100 (90–112)

0.006

94 (83–103)

0.565

98.5 (87–105)

PH^a, n (%)

321

14 (4.4)

11 (5.1)

0.350

1 (1.4)

0.439

2 (5.6)

sPAP^a, mmHg

311

28.4 (10.7)

28.5 (11.2)

1.000

27.7 (6.7)

1.000

29.3 (13.2)

PMI, n (%)

827

154 (18.1)

100 (18.6)

1.000

36 (18.6)

1.000

18 (18.8)

PMI (<45%), n (%)

812

147 (17.9)

95(18.1)

1.000

34(17.8)

1.000

18 (18.8)

Ejection fraction^a, %

391

62.7 (5.8)

62.7 (5.7)

1.000

62.5 (6.7)

1.000

63.5 (4.5)

Cardiac blocks, n (%)

769

52 (6.8)

26 (5.2)

0.104

17 (9.3)

1.000

9 (10.3)

Diastolic dysfunction, n (%)

773

109 (14.1)

77 (15.4)

0.632

22 (12.3)

1.000

10 (10.8)

Pericardial effusion^a, n (%)

398

15 (3.8)

9 (3.5)

1.000

4 (4.1)

0.483

4 (8.7)

Any severe, n (%)

827

293 (35.4)

149 (27.7)

<0.0001

104 (53.6)

0.111

40 (41.7)

EScSG activity index 2001, median (IQR)

827

0.5 (0–1)

0.5 (0–0.5)

<0.0001

0.5 (0–1.5)

0.126

0.5 (0–1)

EScSG activity index 2016^a, median (IQR)

2.7 (1.3–3.7)

2.4 (0.7–3.4)

0.263

3.5 (1.5–5)

0.260

2.4 (1.8–2.6)

EScSG 2001 active, n (%)

796

31 (3.7)

13 (2.4)

0.002

16 (8.2)

0.214

2 (2.1)

EScSG 2016 active^a, n (%)

35 (54.7)

18 (48.6)

0.180

14 (73.7)

0.189

3 (37.5)

Corticosteroids, n (%)

827

174 (21)

73 (13.6)

<0.0001

63 (32.5)

0.464

38 (39.6)

Prednisone equivalent, mg/day, median (IQR)

827

5 (3.5–5)

5 (2.5–5)

0.133

5 (4–6)

0.567

5 (3.4–5)

Immunosuppressants, n (%)

827

267 (32.3)

124 (23.1)

<0.0001

90 (46.4)

0.315

53 (55.2)

Characteristics

Patients with data available, n

Total lcSSc (N = 827)

ACA-lcSSc (n = 537)

P-value

ATA-lcSSc (n = 194)

P-value

ANA-lcSSc (n = 96)

Female, n (%)

827

740 (89.5)

498 (92.7)

<0.0001

161 (83)

0.766

81 (84.4)

Age at RP onset, years

820

45.6 (15.2)

46.9 (15.3)

0.284

44.2 (15.2)

0.423

46.3 (14.5)

Time from RP to SSc, years, median (IQR)

820

1.9 (0.1–7.9)

2.5 (0.3–9.9)

<0.0001

0.7 (0–3)

0.978

0.8 (0–4)

Age at SSc onset, years

827

51.4 (13.7)

53 (13.3)

<0.0001

46.9 (14.2)

0.043

51.1 (13.3)

Age, years

827

52.8 (13.7)

54.4 (13.3)

<0.0001

48.4 (14.3)

0.05

52.4 (13.3)

Disease duration, years

827

1.4 (0.9)

1.000

1.4 (0.9)

1.000

1.4 (0.8)

BMI

455

25.4 (4.9)

25.6 (5.1)

1.000

25.5 (4.7)

1.000

25.1 (4.4)

ESR >20 mm/h^a, n (%)

396

113 (28.5)

65 (26)

0.08

36 (35.3)

0.344

12 (27.3)

CRP elevation, n (%)

381

58 (13.2)

25 (8.8)

<0.0001

25 (23.6)

0.665

8 (16.7)

Hb^a, g/dl

13.2 (1.3)

1.000

13.3 (1.3)

0.928

13.7 (1.0)

mRSS

752

4.8 (4.2)

4.3 (4)

0.001

5.8 (4.8)

1.000

5.1 (3.9)

Puffy fingers (ever), n (%)

451

207 (45.9)

123 (42.4)

0.025

62 (56.4)

0.236

22 (43.1)

DUs ever, n (%)

820

219 (26.7)

141 (26.5)

0.893

59 (30.7)

1.000

19 (20.0)

DUs current, n (%)

820

200 (24.4)

130 (24.4)

0.759

53 (27.6)

0.315

17 (17.9)

Telangiectasia^a, n (%)

138

67 (48.6)

49 (55.1)

0.030

10 (30.3)

0.360

8 (50)

Joint synovitis, n (%)

821

97 (11.8)

46 (8.6)

0.002

33 (17.2)

1.000

18 (18.8)

TFRs, n (%)

819

25 (3.1)

9 (1.7)

0.008

11 (5.7)

1.000

6 (6.3)

Muscle weakness, n (%)

821

110 (13.4)

52 (9.8)

<0.0001

41 (21.1)

0.985

17 (17.7)

CK elevation, n (%)

783

55 (7.0)

18 (3.6)

<0.0001

25 (13.3)

1.000

12 (13.3)

GI involvement, n (%)

826

492 (59.6)

338 (63.1)

0.015

101 (52.1)

1.000

53 (55.2)

GERD, n (%)

824

463 (56.2)

323 (60.5)

0.005

93 (47.9)

1.000

47 (49.0)

Stomach symptoms, n (%)

822

123 (15)

74 (13.9)

0.922

31 (16.1)

1.000

18 (18.8)

Intestinal symptoms, n (%)

824

145 (17.6)

94 (17.6)

1.000

33 (17)

1.000

18 (18.8)

SRC, n (%)

823

7 (0.9)

2 (0.4)

0.581

2 (1)

0.395

3 (3.1)

Serum creatinine^a, mg/dl, median (IQR)

0.7 (0.7–0.9)

0.8 (0.7–0.9)

1.000

0.7 (0.7–0.8)

0.054

0.65 (0.55–0.7)

Proteinuria, n (%)

781

22 (2.8)

8 (1.6)

0.011

10 (5.4)

1.000

4 (4.4)

ILD, n (%)

784

163 (20.8)

52 (10.3)

<0.0001

87 (46.3)

0.004

24 (27.0)

Restrictive LD, n (%)

783

97 (12.4)

43 (8.4)

<0.0001

39 (21.4)

0.628

15 (16.3)

FVC, % predicted, median (IQR)

417

103 (91–115)

106 (95–118)

<0.0001

95 (84.5–106)

0.357

101 (85–114)

TLC^a, % predicted, median (IQR)

287

99 (88–110)

100 (90–111)

0.012

93 (81–107)

0.799

97 (82–110)

DLCO, % predicted, median (IQR)

656

77 (64–89)

78 (66–90)

0.006

72 (59–86)

1.000

71 (58–90)

FEV1^a, % predicted, median (IQR)

289

99 (86–109)

100 (90–112)

0.006

94 (83–103)

0.565

98.5 (87–105)

PH^a, n (%)

321

14 (4.4)

11 (5.1)

0.350

1 (1.4)

0.439

2 (5.6)

sPAP^a, mmHg

311

28.4 (10.7)

28.5 (11.2)

1.000

27.7 (6.7)

1.000

29.3 (13.2)

PMI, n (%)

827

154 (18.1)

100 (18.6)

1.000

36 (18.6)

1.000

18 (18.8)

PMI (<45%), n (%)

812

147 (17.9)

95(18.1)

1.000

34(17.8)

1.000

18 (18.8)

Ejection fraction^a, %

391

62.7 (5.8)

62.7 (5.7)

1.000

62.5 (6.7)

1.000

63.5 (4.5)

Cardiac blocks, n (%)

769

52 (6.8)

26 (5.2)

0.104

17 (9.3)

1.000

9 (10.3)

Diastolic dysfunction, n (%)

773

109 (14.1)

77 (15.4)

0.632

22 (12.3)

1.000

10 (10.8)

Pericardial effusion^a, n (%)

398

15 (3.8)

9 (3.5)

1.000

4 (4.1)

0.483

4 (8.7)

Any severe, n (%)

827

293 (35.4)

149 (27.7)

<0.0001

104 (53.6)

0.111

40 (41.7)

EScSG activity index 2001, median (IQR)

827

0.5 (0–1)

0.5 (0–0.5)

<0.0001

0.5 (0–1.5)

0.126

0.5 (0–1)

EScSG activity index 2016^a, median (IQR)

2.7 (1.3–3.7)

2.4 (0.7–3.4)

0.263

3.5 (1.5–5)

0.260

2.4 (1.8–2.6)

EScSG 2001 active, n (%)

796

31 (3.7)

13 (2.4)

0.002

16 (8.2)

0.214

2 (2.1)

EScSG 2016 active^a, n (%)

35 (54.7)

18 (48.6)

0.180

14 (73.7)

0.189

3 (37.5)

Corticosteroids, n (%)

827

174 (21)

73 (13.6)

<0.0001

63 (32.5)

0.464

38 (39.6)

Prednisone equivalent, mg/day, median (IQR)

827

5 (3.5–5)

5 (2.5–5)

0.133

5 (4–6)

0.567

5 (3.4–5)

Immunosuppressants, n (%)

827

267 (32.3)

124 (23.1)

<0.0001

90 (46.4)

0.315

53 (55.2)

Table 1

Open in new tab

Demographic and clinical characteristics of patients with the limited cutaneous form at baseline

Characteristics	Patients with data available, n	Total lcSSc (N = 827)	ACA-lcSSc (n = 537)	P-value	ATA-lcSSc (n = 194)	P-value	ANA-lcSSc (n = 96)
Female, n (%)	827	740 (89.5)	498 (92.7)	<0.0001	161 (83)	0.766	81 (84.4)
Age at RP onset, years	820	45.6 (15.2)	46.9 (15.3)	0.284	44.2 (15.2)	0.423	46.3 (14.5)
Time from RP to SSc, years, median (IQR)	820	1.9 (0.1–7.9)	2.5 (0.3–9.9)	<0.0001	0.7 (0–3)	0.978	0.8 (0–4)
Age at SSc onset, years	827	51.4 (13.7)	53 (13.3)	<0.0001	46.9 (14.2)	0.043	51.1 (13.3)
Age, years	827	52.8 (13.7)	54.4 (13.3)	<0.0001	48.4 (14.3)	0.05	52.4 (13.3)
Disease duration, years	827	1.4 (0.9)	1.4 (0.9)	1.000	1.4 (0.9)	1.000	1.4 (0.8)
BMI	455	25.4 (4.9)	25.6 (5.1)	1.000	25.5 (4.7)	1.000	25.1 (4.4)
ESR >20 mm/h^a, n (%)	396	113 (28.5)	65 (26)	0.08	36 (35.3)	0.344	12 (27.3)
CRP elevation, n (%)	381	58 (13.2)	25 (8.8)	<0.0001	25 (23.6)	0.665	8 (16.7)
Hb^a, g/dl	64	13.2 (1.3)	13.2 (1.3)	1.000	13.3 (1.3)	0.928	13.7 (1.0)
mRSS	752	4.8 (4.2)	4.3 (4)	0.001	5.8 (4.8)	1.000	5.1 (3.9)
Puffy fingers (ever), n (%)	451	207 (45.9)	123 (42.4)	0.025	62 (56.4)	0.236	22 (43.1)
DUs ever, n (%)	820	219 (26.7)	141 (26.5)	0.893	59 (30.7)	1.000	19 (20.0)
DUs current, n (%)	820	200 (24.4)	130 (24.4)	0.759	53 (27.6)	0.315	17 (17.9)
Telangiectasia^a, n (%)	138	67 (48.6)	49 (55.1)	0.030	10 (30.3)	0.360	8 (50)
Joint synovitis, n (%)	821	97 (11.8)	46 (8.6)	0.002	33 (17.2)	1.000	18 (18.8)
TFRs, n (%)	819	25 (3.1)	9 (1.7)	0.008	11 (5.7)	1.000	6 (6.3)
Muscle weakness, n (%)	821	110 (13.4)	52 (9.8)	<0.0001	41 (21.1)	0.985	17 (17.7)
CK elevation, n (%)	783	55 (7.0)	18 (3.6)	<0.0001	25 (13.3)	1.000	12 (13.3)
GI involvement, n (%)	826	492 (59.6)	338 (63.1)	0.015	101 (52.1)	1.000	53 (55.2)
GERD, n (%)	824	463 (56.2)	323 (60.5)	0.005	93 (47.9)	1.000	47 (49.0)
Stomach symptoms, n (%)	822	123 (15)	74 (13.9)	0.922	31 (16.1)	1.000	18 (18.8)
Intestinal symptoms, n (%)	824	145 (17.6)	94 (17.6)	1.000	33 (17)	1.000	18 (18.8)
SRC, n (%)	823	7 (0.9)	2 (0.4)	0.581	2 (1)	0.395	3 (3.1)
Serum creatinine^a, mg/dl, median (IQR)	65	0.7 (0.7–0.9)	0.8 (0.7–0.9)	1.000	0.7 (0.7–0.8)	0.054	0.65 (0.55–0.7)
Proteinuria, n (%)	781	22 (2.8)	8 (1.6)	0.011	10 (5.4)	1.000	4 (4.4)
ILD, n (%)	784	163 (20.8)	52 (10.3)	<0.0001	87 (46.3)	0.004	24 (27.0)
Restrictive LD, n (%)	783	97 (12.4)	43 (8.4)	<0.0001	39 (21.4)	0.628	15 (16.3)
FVC, % predicted, median (IQR)	417	103 (91–115)	106 (95–118)	<0.0001	95 (84.5–106)	0.357	101 (85–114)
TLC^a, % predicted, median (IQR)	287	99 (88–110)	100 (90–111)	0.012	93 (81–107)	0.799	97 (82–110)
DLCO, % predicted, median (IQR)	656	77 (64–89)	78 (66–90)	0.006	72 (59–86)	1.000	71 (58–90)
FEV1^a, % predicted, median (IQR)	289	99 (86–109)	100 (90–112)	0.006	94 (83–103)	0.565	98.5 (87–105)
PH^a, n (%)	321	14 (4.4)	11 (5.1)	0.350	1 (1.4)	0.439	2 (5.6)
sPAP^a, mmHg	311	28.4 (10.7)	28.5 (11.2)	1.000	27.7 (6.7)	1.000	29.3 (13.2)
PMI, n (%)	827	154 (18.1)	100 (18.6)	1.000	36 (18.6)	1.000	18 (18.8)
PMI (<45%), n (%)	812	147 (17.9)	95(18.1)	1.000	34(17.8)	1.000	18 (18.8)
Ejection fraction^a, %	391	62.7 (5.8)	62.7 (5.7)	1.000	62.5 (6.7)	1.000	63.5 (4.5)
Cardiac blocks, n (%)	769	52 (6.8)	26 (5.2)	0.104	17 (9.3)	1.000	9 (10.3)
Diastolic dysfunction, n (%)	773	109 (14.1)	77 (15.4)	0.632	22 (12.3)	1.000	10 (10.8)
Pericardial effusion^a, n (%)	398	15 (3.8)	9 (3.5)	1.000	4 (4.1)	0.483	4 (8.7)
Any severe, n (%)	827	293 (35.4)	149 (27.7)	<0.0001	104 (53.6)	0.111	40 (41.7)
EScSG activity index 2001, median (IQR)	827	0.5 (0–1)	0.5 (0–0.5)	<0.0001	0.5 (0–1.5)	0.126	0.5 (0–1)
EScSG activity index 2016^a, median (IQR)	64	2.7 (1.3–3.7)	2.4 (0.7–3.4)	0.263	3.5 (1.5–5)	0.260	2.4 (1.8–2.6)
EScSG 2001 active, n (%)	796	31 (3.7)	13 (2.4)	0.002	16 (8.2)	0.214	2 (2.1)
EScSG 2016 active^a, n (%)	64	35 (54.7)	18 (48.6)	0.180	14 (73.7)	0.189	3 (37.5)
Corticosteroids, n (%)	827	174 (21)	73 (13.6)	<0.0001	63 (32.5)	0.464	38 (39.6)
Prednisone equivalent, mg/day, median (IQR)	827	5 (3.5–5)	5 (2.5–5)	0.133	5 (4–6)	0.567	5 (3.4–5)
Immunosuppressants, n (%)	827	267 (32.3)	124 (23.1)	<0.0001	90 (46.4)	0.315	53 (55.2)

Characteristics	Patients with data available, n	Total lcSSc (N = 827)	ACA-lcSSc (n = 537)	P-value	ATA-lcSSc (n = 194)	P-value	ANA-lcSSc (n = 96)
Female, n (%)	827	740 (89.5)	498 (92.7)	<0.0001	161 (83)	0.766	81 (84.4)
Age at RP onset, years	820	45.6 (15.2)	46.9 (15.3)	0.284	44.2 (15.2)	0.423	46.3 (14.5)
Time from RP to SSc, years, median (IQR)	820	1.9 (0.1–7.9)	2.5 (0.3–9.9)	<0.0001	0.7 (0–3)	0.978	0.8 (0–4)
Age at SSc onset, years	827	51.4 (13.7)	53 (13.3)	<0.0001	46.9 (14.2)	0.043	51.1 (13.3)
Age, years	827	52.8 (13.7)	54.4 (13.3)	<0.0001	48.4 (14.3)	0.05	52.4 (13.3)
Disease duration, years	827	1.4 (0.9)	1.4 (0.9)	1.000	1.4 (0.9)	1.000	1.4 (0.8)
BMI	455	25.4 (4.9)	25.6 (5.1)	1.000	25.5 (4.7)	1.000	25.1 (4.4)
ESR >20 mm/h^a, n (%)	396	113 (28.5)	65 (26)	0.08	36 (35.3)	0.344	12 (27.3)
CRP elevation, n (%)	381	58 (13.2)	25 (8.8)	<0.0001	25 (23.6)	0.665	8 (16.7)
Hb^a, g/dl	64	13.2 (1.3)	13.2 (1.3)	1.000	13.3 (1.3)	0.928	13.7 (1.0)
mRSS	752	4.8 (4.2)	4.3 (4)	0.001	5.8 (4.8)	1.000	5.1 (3.9)
Puffy fingers (ever), n (%)	451	207 (45.9)	123 (42.4)	0.025	62 (56.4)	0.236	22 (43.1)
DUs ever, n (%)	820	219 (26.7)	141 (26.5)	0.893	59 (30.7)	1.000	19 (20.0)
DUs current, n (%)	820	200 (24.4)	130 (24.4)	0.759	53 (27.6)	0.315	17 (17.9)
Telangiectasia^a, n (%)	138	67 (48.6)	49 (55.1)	0.030	10 (30.3)	0.360	8 (50)
Joint synovitis, n (%)	821	97 (11.8)	46 (8.6)	0.002	33 (17.2)	1.000	18 (18.8)
TFRs, n (%)	819	25 (3.1)	9 (1.7)	0.008	11 (5.7)	1.000	6 (6.3)
Muscle weakness, n (%)	821	110 (13.4)	52 (9.8)	<0.0001	41 (21.1)	0.985	17 (17.7)
CK elevation, n (%)	783	55 (7.0)	18 (3.6)	<0.0001	25 (13.3)	1.000	12 (13.3)
GI involvement, n (%)	826	492 (59.6)	338 (63.1)	0.015	101 (52.1)	1.000	53 (55.2)
GERD, n (%)	824	463 (56.2)	323 (60.5)	0.005	93 (47.9)	1.000	47 (49.0)
Stomach symptoms, n (%)	822	123 (15)	74 (13.9)	0.922	31 (16.1)	1.000	18 (18.8)
Intestinal symptoms, n (%)	824	145 (17.6)	94 (17.6)	1.000	33 (17)	1.000	18 (18.8)
SRC, n (%)	823	7 (0.9)	2 (0.4)	0.581	2 (1)	0.395	3 (3.1)
Serum creatinine^a, mg/dl, median (IQR)	65	0.7 (0.7–0.9)	0.8 (0.7–0.9)	1.000	0.7 (0.7–0.8)	0.054	0.65 (0.55–0.7)
Proteinuria, n (%)	781	22 (2.8)	8 (1.6)	0.011	10 (5.4)	1.000	4 (4.4)
ILD, n (%)	784	163 (20.8)	52 (10.3)	<0.0001	87 (46.3)	0.004	24 (27.0)
Restrictive LD, n (%)	783	97 (12.4)	43 (8.4)	<0.0001	39 (21.4)	0.628	15 (16.3)
FVC, % predicted, median (IQR)	417	103 (91–115)	106 (95–118)	<0.0001	95 (84.5–106)	0.357	101 (85–114)
TLC^a, % predicted, median (IQR)	287	99 (88–110)	100 (90–111)	0.012	93 (81–107)	0.799	97 (82–110)
DLCO, % predicted, median (IQR)	656	77 (64–89)	78 (66–90)	0.006	72 (59–86)	1.000	71 (58–90)
FEV1^a, % predicted, median (IQR)	289	99 (86–109)	100 (90–112)	0.006	94 (83–103)	0.565	98.5 (87–105)
PH^a, n (%)	321	14 (4.4)	11 (5.1)	0.350	1 (1.4)	0.439	2 (5.6)
sPAP^a, mmHg	311	28.4 (10.7)	28.5 (11.2)	1.000	27.7 (6.7)	1.000	29.3 (13.2)
PMI, n (%)	827	154 (18.1)	100 (18.6)	1.000	36 (18.6)	1.000	18 (18.8)
PMI (<45%), n (%)	812	147 (17.9)	95(18.1)	1.000	34(17.8)	1.000	18 (18.8)
Ejection fraction^a, %	391	62.7 (5.8)	62.7 (5.7)	1.000	62.5 (6.7)	1.000	63.5 (4.5)
Cardiac blocks, n (%)	769	52 (6.8)	26 (5.2)	0.104	17 (9.3)	1.000	9 (10.3)
Diastolic dysfunction, n (%)	773	109 (14.1)	77 (15.4)	0.632	22 (12.3)	1.000	10 (10.8)
Pericardial effusion^a, n (%)	398	15 (3.8)	9 (3.5)	1.000	4 (4.1)	0.483	4 (8.7)
Any severe, n (%)	827	293 (35.4)	149 (27.7)	<0.0001	104 (53.6)	0.111	40 (41.7)
EScSG activity index 2001, median (IQR)	827	0.5 (0–1)	0.5 (0–0.5)	<0.0001	0.5 (0–1.5)	0.126	0.5 (0–1)
EScSG activity index 2016^a, median (IQR)	64	2.7 (1.3–3.7)	2.4 (0.7–3.4)	0.263	3.5 (1.5–5)	0.260	2.4 (1.8–2.6)
EScSG 2001 active, n (%)	796	31 (3.7)	13 (2.4)	0.002	16 (8.2)	0.214	2 (2.1)
EScSG 2016 active^a, n (%)	64	35 (54.7)	18 (48.6)	0.180	14 (73.7)	0.189	3 (37.5)
Corticosteroids, n (%)	827	174 (21)	73 (13.6)	<0.0001	63 (32.5)	0.464	38 (39.6)
Prednisone equivalent, mg/day, median (IQR)	827	5 (3.5–5)	5 (2.5–5)	0.133	5 (4–6)	0.567	5 (3.4–5)
Immunosuppressants, n (%)	827	267 (32.3)	124 (23.1)	<0.0001	90 (46.4)	0.315	53 (55.2)

Values are presented as mean (s.d.) unless stated otherwise.

Data available in less than half of patients.

Significant P-values (<0.05) are in bold.

CK: creatine kinase; DLCO: diffusing capacity for carbon monoxide; DUs: digital ulcers; EScSG: European Scleroderma Study Group; FEV1: forced expiratory volume in the first second; FVC: forced vital capacity; GI: gastrointestinal; GERD: gastroesophageal reflux disease; Hb: haemoglobin; ILD: interstitial lung disease; LD: lung disease; sPAP: systolic pulmonary artery pressure; immunosuppressants: CYC, SSZ, HCQ, MTX, LEF, AZA, MMF, ciclosporin A, d-penicillamine, rituximab, imatinib, anti-TNF inhibitors, tocilizumab or abatacept; mRSS: modified Rodnan Skin Score; PH: pulmonary hypertension; PMI: primary myocardial involvement; SRC: scleroderma renal crisis; TFRs: tendon friction rubs; TLC: total lung capacity.

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