Demographic and baseline patient characteristics

VariableIPPFE with UIPTypical IPFp-value
Age years72.7±7.172.7±7.10.972
BMI kg·m−217.6±2.922.7±3.5<0.0001
Current/former/never smokers6/34/2426/136/330.003
mMRC score 0/I/II/III/IV5/25/23/6/522/61/60/40/120.248
Severity of IPF GAP stage I/II/III28/24/1299/68/280.555
Primary lung cancer0 (0%)12 (6.2%)0.042
Pneumothorax20 (31.3%)3 (1.5%)<0.0001
Acute exacerbation19 (29.7%)64 (32.8%)0.757
Nintedanib8 (12.5%)38 (19.4%)0.06
Pirfenidone26 (40.6%)53 (27.1%)0.06
Histological UIP diagnosis10 (15.6%)42 (21.5%)0.370
Histological PPFE diagnosis10 (15.6%)0 (0%)<0.0001
Observation period months30.7±19.738.9±34.20.07

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.