Table 1 Clinical characteristics.

From: Remodeling of the pulmonary artery in idiopathic pleuroparenchymal fibroelastosis

Factor

IPF (n = 24)

IPPFE (n = 18)

P value†

Age, years

65.2 ± 10.2

59.3 ± 13.2

0.111

Gender, male/female, n

17/7

9/9

0.21

Smoking status, current/former/never, n

4/15/5

1/4/13

0.003

Pack-years smoking

33.5 ± 42.7

7.94 ± 14.9

0.02

Body mass index, kg/m2

22.4 ± 2.98

16.4 ± 3.00

<0.001

Serum KL-6 levels, U/mL*

1127 ± 777

497 ± 214

0.003

Respiratory function parameters*

   FVC, ml

2364 ± 880

1751 ± 810

0.026

   FVC, % predicted

72.6 ± 19.0

54.8 ± 23.4

0.01

   RV, % predicted

56.9 ± 21.4

91.9 ± 37.0

0.001

   RV/TLC, % predicted

86.5 ± 26.2

138 ± 45.6

<0.001

   DLCO, % predicted

61.0 ± 26.1

70.2 ± 25.7

0.31

   DLCO/VA, % predicted

83.9 ± 29.5

87.1 ± 31.9

0.767

  1. *The respiratory functional parameters and serum KL-6 levels were measured less than one year prior to tissue collection.
  2. †p values were calculated for the comparison of IPF and IPPFE patients.
  3. IPF, idiopathic pulmonary fibrosis; IPPFE, idiopathic pleuroparenchymal fibroelastosis; KL-6, Kerbs von Lungren-6 antigen; FVC, forced vital capacity; RV, residual volume; TLC, total lung capacity; DLCO, diffusing capacity of the lung for carbon monoxide; VA, alveolar volume.
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