Demographic and baseline patient characteristics
| Variable | IPPFE with UIP | Typical IPF | p-value |
| Subjects | 64 | 195 | |
| Males/females | 43/21 | 162/33 | 0.012 |
| Age years | 72.7±7.1 | 72.7±7.1 | 0.972 |
| BMI kg·m−2 | 17.6±2.9 | 22.7±3.5 | <0.0001 |
| Current/former/never smokers | 6/34/24 | 26/136/33 | 0.003 |
| mMRC score 0/I/II/III/IV | 5/25/23/6/5 | 22/61/60/40/12 | 0.248 |
| Severity of IPF GAP stage I/II/III | 28/24/12 | 99/68/28 | 0.555 |
| Primary lung cancer | 0 (0%) | 12 (6.2%) | 0.042 |
| Pneumothorax | 20 (31.3%) | 3 (1.5%) | <0.0001 |
| Acute exacerbation | 19 (29.7%) | 64 (32.8%) | 0.757 |
| Nintedanib | 8 (12.5%) | 38 (19.4%) | 0.06 |
| Pirfenidone | 26 (40.6%) | 53 (27.1%) | 0.06 |
| Histological UIP diagnosis | 10 (15.6%) | 42 (21.5%) | 0.370 |
| Histological PPFE diagnosis | 10 (15.6%) | 0 (0%) | <0.0001 |
| Observation period months | 30.7±19.7 | 38.9±34.2 | 0.07 |
Data are presented as n, mean±sd or n (%), unless otherwise stated. IPPFE: idiopathic pleuroparenchymal fibroelastosis; UIP: usual interstitial pneumonia; IPF: idiopathic pulmonary fibrosis; BMI: body mass index; mMRC: modified Medical Research Council; GAP: gender, age and lung physiology; PPFE: pleuroparenchymal fibroelastosis.