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Table 4 Patients in the HSCT group were predominantly treated before the age of 50 years (A). Patients with primary rapid progressive SSc were treated with HSCT within the first 3 years of the disease (B). Patients treated after 3 years had comparable survival rates after about 5 to 7 years of follow-up

From: Autologous hematopoietic stem cell transplantation improves long-term survival—data from a national registry

A

Age at HSCT<50y

Age at HSCT≥50y

 

B

Time Dg to HSCT <3y

Time Dg to HSCT ≥3y

 

n

57 (76%)

18 (24%)

p

 

43 (56%)

34 (44%)

p

female

32 (56%)

9 (50%)

0.787

 

18 (42%)

23 (68%)

0.035

male

25 (44%)

9 (50%)

  

24 (56%)

10 (29%)

 

Age at Dg (mean ± SD)

33.9 ± 8.9

52.9 ± 5.7

<0.001

 

41.1 ± 11.6

35.0 ± 10.9

0.018

Age at HSCT

38.5 ± 8.2

57.3 ± 3.7

n.a.

 

43.2 ± 11.6

42.9 ± 10.2

0.949

Time Dg to HSCT

4.2 ± 4.8

3.8 ± 4.6

0.794

 

1.6 ± 0.8

7.4 ± 5.6

n.a.

diffuse cutaneous SSc

52 (91%)

14 (78%)

0.160

 

41 (95%)

27 (79%)

0.042

mRSS at HSCT

17.7 ± 11.7

19.4 ± 12.7

0.579

 

20.8 ± 11.5

14.5 ± 11.4

0.015

DLCO-SB at HSCT

56.0 ± 15.9

46.8 ± 18.4

0.261

 

52.3 ±17.0

55.6 ± 19.4

0.567

fVC at HSCT

73.8 ± 21.4

81.0 ± 18.4

0.261

 

76.1 ± 16.5

75.4 ± 24.8

1.000

ILD with PAH n(%)

5 / 56 (9%)

6 / 18 (33%)

0.024

 

7 / 42 (17%)

4 / 34 (12%)

0.328

Heart involvement

12 / 56 (21%)

7 / 18 (39%)

0.213

 

15 / 42 (36%)

4 / 34 (12%)

0.019

Time HSCT to last f-up

6.9 ± 4.8

4.3 ± 4.2

0.025

 

6.2 ± 4.7

7.0 ± 5.4

0.652

Alive at last f-up

54 / 57 (95%)

15 / 18 (83%)

0.145

 

38 / 43 (88%)

33 / 34 (97%)

0.220

Dead at last f-up

3 / 57 (5%)

3 / 18 (17%)

  

5 / 43 (12%)

1 / 34 (3%)