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Table 2 Pulmonary function tests and mRSS at baseline and 6 months, and HRCT outcome in 26 systemic sclerosis patients treated with imatinib

From: Low-dose oral imatinib in the treatment of systemic sclerosis interstitial lung disease unresponsive to cyclophosphamide: a phase II pilot study

Patient (PIN)

FVC (l) T0

FVC (l) T6

ΔFVC (T0 – T6) (%)

DLco (ml/mmHg/minute) T0

DLco (ml/mmHg/minute) T6

ΔDLco (T0 – T6) (%)

HRCTaT0

HRCTaT6

HRCT (evaluation at T6)

mRSS T0

mRSS T6

ΔmRSS (T0 – T6) (%)

Months from the end of CYC/cumulative dose of CYC/other therapies

86

3.23

3.84

−5.88

8.45

10.78

27.57

19/18

4/18

Improved

4

3

−25

21/10 g/Aza

509

3.36

2.71

–19.3

10.64

14.05

32.05

6/2

4/2

Improved

24

31

29.16

48/7.5 g/Mtx

642

1.53

1.57

2.61

8

10

25

9/9

6/9

Improved

5

6

20

11/70 g

649

1.79

1.83

2.23

8.44

9.54

19.62

6/8

7/9

Unchanged

3

3

0

3/10 g/CyA

18

2.35

2,31

–1.7

18.99

19.92

4.9

6/8

3/8

Improved

0

0

0

42/13.5 g/Aza

115

2.69

2.45

–8.92

16.1

17

6.83

4/2

4/2

Unchanged

20

20

0

12/18 g/Aza, Rtx

205

1.52

1.43

–5.92

4.9

5

2.04

6/20

0/20

Improved

5

6

29.16

22/21 g

214

3.16

3,16

0

12.9

14

8.53

10/8

1/9

Improved

29

25

−13.79

9/6 g

219

1.97

1.83

−7.1

8.5

8.1

−4,7

20/20

20/20

Unchanged

10

16

60

36/6 g/Aza

297

2,19

2.24

2.28

7.2

7.49

4.02

13/7

12/7

Unchanged

22

17

−22.72

20/6 g

304

2.41

2.56

6.22

15.1

14.4

−4.63

12/3

11/3

Unchanged

11

9

−18.18

3/6 g

371

3.14

3.56

13.37

17.8

19.7

10.67

6/8

0/8

Improved

6

6

0

4/8.2 g

432

1.77

1.82

2.82

5.31

5.77

8.66

14/17

13/17

Unchanged

9

9

0

8/9 g

482

1.55

1.43

−7.74

7.68

7.39

−3.78

11/14

1/14

Improved

5

4

−20

7/9 g

522

3.20

3.08

−3.75

13.18

13.2

0.15

12/6

4/5

Improved

28

31

10.71

10/9 g

586

2.21

2.42

9.5

8.6

9.1

5.81

6/12

5/12

Unchanged

28

30

7.14

3/6 g

696

3.29

3.27

−0.6

14.5

14.19

2.76

11/6

7/7

Improved

8

10

25

15/7,2 g

763

3.81

3.75

−1.5

16.4

16.9

3.05

14/13

8/13

Improved

17

20

17.64

15/6 g/Rtx

801

2.09

2.13

1.9

10.5

11.5

9.52

16/12

8/13

Improved

15

11

26.66

3/7,2 g

144

3.56

3.57

0.28

9.65

6.16

–36.16

9/14

4/16

Worsened

15

15

0

6/10 g

358

2.24

1.81

–19.2

8.30

7.6

–8.63

8/14

4/14

Improved

14

24

71.42

12/38 g/Aza

459

2.44

1.97

–19.26

10.4

9.9

–4.81

14/20

14/20

Unchanged

25

27

8

15/9 g

534

3.26

3.01

−7.6

15.12

14.93

−1.26

5/4

10/12

Worsened

0

0

0

28/10 g/Aza

552

1.95

1.95

0

8.98

7.86

−11.36

18/18

20/20

Worsened

28

31

10.71

8/20 g/Aza, MMF

706

1.86

1.7

−8.6

6.21

3.55

−42.83

6/20

6/20

Unchanged

10

10

0

7/6 g/Aza

789

2.04

2.12

3.9

10.7

10.4

−2.8

10/4

10/10

Worsened

7

10

42.85

21/7.2 g

  1. Bold data indicate that patients responders: Roman data indicate that patients stabilized; italic data indicate that patients worsened. Aza, azathioprine; CyA, cyclosporine A; CYC, cyclophosphamide; DLco, diffusion lung capacity of carbon monoxide; FVC, forced vital capacity; HRCT, high-resolution computed tomography; Mtx, methotrexate; MMF, mycofenomycophenolate mophetil; mRSS, modified Rodnan skin score; Rtx, rituximab; T0, baseline; T6, 6-month visit; Δ = (T6/T0 × 100) – 100. aNumber of segments with ground-glass/honeycombing.