Comparison of pulmonary function tests, serum markers and chest computed tomography (CT) findings between patients with idiopathic pleuroparenchymal fibroelastosis (IPPFE) with usual interstitial pneumonia (UIP) and typical idiopathic pulmonary fibrosis (IPF)

VariableIPPFE with UIPTypical IPFp-value
Subjects n64195
FVC % predicted66.7±16.978.5±18.80.0001
FEV1 % predicted86.1±24.997.2±47.30.073
TLC % predicted74.4±15.777.9±16.60.153
RV % predicted90.6±27.382.9±21.90.026
DLCO % predicted66.2±25.260.3±20.40.072
KL-6 U·mL−1823±4851123±7430.003
SP-D ng·mL−1283±162293±2340.742
PaO2 mmHg84.1±13.480.8±13.10.087
PaCO2 mmHg43.6±5.839.4±3.9<0.0001
Fibrosis score1.7±0.71.6±0.60.953
Extent of bronchiectasis1.8±0.81.6±0.70.106
CT pattern: UIP/possible UIP/inconsistent with UIP169/22/4

Data are presented as mean±sd unless otherwise stated. FVC: forced vital capacity; FEV1: forced expiratory volume in 1 s; TLC: total lung capacity; RV: residual volume; DLCO: diffusing capacity of the lung for carbon monoxide; CPI: composite physiological index; KL-6: Krebs von den Lungen-6; SP-D: surfactant protein D; PaO2: arterial oxygen tension; PaCO2: arterial carbon dioxide tension.