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Table 1 Patient characteristics at surgical lung biopsy between the existence and non-existence of pathological emphysema with SSc-associated ILD

From: Emphysematous change with scleroderma-associated interstitial lung disease: the potential contribution of vasculopathy?

Characteristics

All subjects

Existence of pathological pulmonary emphysema

P value

Yes (p-PE with SSc-ILD)

No

No. of patients

21

16

5

 

Female N (%)

18 (85.7)

14 (87.5)

4 (80.0)

> 0.999

Age, mean ± SD

60.3 ± 10.5

60.5 ± 10.5

59.8 ± 11.7

0.679

Never smoker N (%)

13 (61.9)

10 (62.5)

3 (60.0)

> 0.999

Sjögren’s syndrome N (%)

3 (14.3)

2 (12.5)

1 (20.0)

> 0.999

Digital ulcers N (%)

4 (19.0)

3 (18.8)

1 (20.0)

> 0.999

Hypertension N (%)

4 (19.0)

3 (18.8)

1 (20.0)

> 0.999

Chronic kidney disease N(%)

1 (4.8)

1 (6.3)

0 (0.0)

> 0.999

KL-6, U/mL

1350.5 ± 1042.8

1517.1 ± 1102.8

684.0 ± 255.1

0.089

SP-D, ng/mL

223.84 ± 132.42

214.86 ± 139.17

259.75 ± 109.89

0.395

Pulmonary function tests

 FEV1/FVC ratio, %

81.17 ± 5.95

80.67 ± 6.70

82.78 ± 2.02

0.741

 FVC, % predicted

92.79 ± 16.76

90.52 ± 17.00

100.04 ± 15.33

0.215

 DLCO, % predicted

69.85 ± 20.56

65.02 ± 20.11

87.98 ± 9.68

0.0163*

 FVC/DLCO, ratio

1.41 ± 0.37

1.46 ± 0.39

1.22 ± 0.14

0.253

Disease extent on HRCT, %

24.93 ± 10.78

28.13 ± 10.56

15.35 ± 2.94

0.013*

RVSP on echocardiography (available N)

18

15

3

 

 RVSP, mmHg

33.3 ± 11.8

34.8 ± 12.5

26.2 ± 0.7

0.279

 RVSP (< 35/ 35- < 50/ ≥50)

11/ 4/ 3

8/ 4/ 3

3/ 0/ 0

0.730

Staging

 Limited disease N (%)

13 (61.9)

8 (50.0)

5 (100.0)

0.114

 Extensive disease N (%)

7 (33.3)

7 (43.7)

0 (0.0)

 Unknown N (%)

1 (8.3)

1 (6.3)

0 (0.0)

 

Medication (during follow-up)

 PAH-specific drug therapy usea

2 (9.5)

1 (6.3)

1 (20.0)

0.429

 Ca channel blocker or ACE inhibitor

5 (23.8)

4 (25.0)

1 (20.0)

> 0.999

 Steroid use

10 (47.6)

9 (56.3)

1 (20.0)

0.311

 Cyclophosphamide use

4 (19.0)

4 (25.0)

0 (0.0)

0.532

 Cyclosporine, tacrolimus or azathioprine use

5 (23.8)

4 (25.0)

1 (20.0)

> 0.999

 Pirfenidone or nintedanib use

3 (14.3)

3 (18.8)

0 (0.0)

0.549

Deaths N (%) (during follow-up)

2 (9.5)

2 (12.5)

0 (0.0)

 

Median follow-up years (range)

2.44 (0.21–16.06)

2.23 (0.21–16.06)

5.07 (0.81–12.5)

 
  1. Data are presented as mean ± SD, unless otherwise stated. SSc systemic sclerosis, ILD interstitial lung disease, p-PE pathological pulmonary emphysema, KL-6 Krebs von den Lungen-6, SP-D surfactant protein-D, FEV 1 forced expiratory volume in 1 s, FVC forced vital capacity, D LCO diffusing capacity of the lung for carbon monoxide, HRCT high-resolution computed tomography, RVSP right ventricular systolic pressure, PAH pulmonary arterial hypertension. aPAH-specific drugs include only beraprost sodium (N = 2). *P value less than 0.05
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BMC Pulmonary Medicine

ISSN: 1471-2466

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