Clinical characteristics
| Control donor subjects | IPF patients | PH-associated IPF | PH | |
| Subjects n | 18 | 9 | 8 | 4 |
| Age years | 59 (42–71) | 61 (56–75) | 65(52–73) | 45 (42–52) |
| Males/females | 15/3 | 12/5 | 25/11 | 3/1 |
| Smoking | ||||
| Never smoked/smokers | 7/11 | 3/6 | 4/4 | 4/0 |
| Pack-years | 26 (0–32) | 28.3 (6–35) | 31 (9–38) | |
| FEV1 % pred | ND | 72.8 (58–101) | 73 (53–98) | 92 (91–95) |
| FVC % pred | ND | 70.2 (63–79) | 71 (48–76) | ND |
| TLC % pred | ND | 73.5 (45–89) | 66 (43–90) | ND |
| DLCO % pred | ND | 50.1 (34–61) | 40.8 (20–61) | ND |
| Ground glass# % | 0 | 19 (8–39) | 22 (9–35) | 0 |
| Honeycombing¶ % | 0 | 28 (15–40) | 26 (12–39) | 0 |
| PaO2 mmHg | 94 (88–96) | 65 (45–88) | 60 (40–85) | 62 (48–75) |
| mPAP mmHg·L−1·min−1 | ND | 20.5 (16–22) | 43 (36–48) | 69 (42–74) |
| NAC+ (yes/no) | 0/21 | 4/5 | 5/4 | 0/4 |
| Pirfenidone+ (yes/no) | 0/21 | 2/7 | 1/7 | 0/4 |
Data are presented as medians (interquartile range), unless otherwise stated. IPF: idiopathic pulmonary fibrosis; PH: pulmonary hypertension; FEV1: forced expiratory volume in 1 s; FVC: forced vital capacity; TLC: total lung capacity; DLCO: diffusion capacity of the lung for carbon monoxide; PaO2: arterial blood oxygen tension; mPAP: mean pulmonary artery pressure; NAC: N-acetyl-l-cysteine; ND: not determined. #: percentage of pulmonary parenchyma with ground glass on a computed tomography (CT) image; ¶: percentage of pulmonary parenchyma with honeycombing on a CT image; +: patients who received this treatment at the time of pulmonary biopsy.