Table 3 Prognostic significance of histopathology findings in SSc-ILD

Bouros, AJRCCM 2002

SSc cases with LBx

80

62

12¹

6

Treatments: CSSonly/CYC/AZA

13/26/12

-

-

-

Deaths N (%) - FUP 74.5 (16–62)months

22 (30)

16 (26)

6 (50)

-

5 years survival²

-

90%

82%

-

10 years survival²

-

69%

29%

-

median FVC change at 1 and 3 years

-1.7% and − 2.5%

-

-

-

median DLco change at 1 and 3 years

-0.3% and − 1.8%³

-

-

-

Fischer, Chest 2008

SSc cases with LBx

22

14

8

-

Treatments: CSS + CYC or CSS + AZA

unk

-

-

-

Deaths N (%)

11 (50)

6 (43)

5 (63)

-

median survival time in years⁴

-

15.3

3

-

Kim, Sarcoidosis VDLD 2002

SSc cases with LBx

19

13

5

-

Treatments: CSSonly/CSS + CYC

1/12

0/12

1/0

-

> 15% FVC and DLco improvement after treatment, N

-

5

0

-

CRP score: N improved/ N worsened

-

5/0

0/5

-

CRP score after treatment

-

-11.8 (8.9)

1.5 (3.4)

-

De Souza, Respiration 2009

SSc cases with LBx

28

19

1

6 CLF (2 other^^)

Treatment: CYC/antiGERD only

18/0

-

0/6

Δ % FVC decline at 1 year, median (SD)

–2.15 (11.28)

-

–3.87 (6.63)

Δ % FEV1 decline at 1 year, median (SD)

–1.61 (10.54)

-

–9.88 (12.14)

Δ % DLco decline at 1 year, median (SD)

–11.63 (20.98)

-

-17.06 (45.59)

Domiciano, Clir Rheum 2011

SSc cases with LBx

18

18⁵

-

Treatments: CYC/CYC + PRED

9/9

9/9

-

-

Deaths in 2 years

2 (11%)

2 (11%)

-

Yamakawa, PlosOne 2016

SSc cases with LBx

32

20

1

11 unclass

Deaths N (%) - FUP 2.84 (0.15–17.25)years

18 (20)

5 years mortality

24.4%