Real-life experience with pirfenidone in observational studies

First author [ref.]CountryPatients nPatient characteristicsEfficacy outcomeAdverse eventsTreatment discontinuation due to adverse events %
Bonella [18]Germany45Age: 69±7 years Baseline FVC (% pred): 61±15 Baseline DLCO (% pred): 48±14Stable lung function in 28 (70%) out of 40 patients; subjective improvement in cough in 12 (33%) out of 36 patients17 (38)10 (22)13
Okuda [19]Japan76Age: 70.5±8.3 years Baseline FVC (% pred): 65.3±16.1 Baseline DLCO (% pred): 55.9±17.8Reduction in FVC and DLCO decline18 (24)#19 (25)18
Arai [20]Japan41Age: 70 (65.5–75.5) years Baseline VC (% pred): 66.7 (54.8–77.8)Significant reduction in VC decline in patients with severity grades I–II (Japanese Respiratory Society criteria)24 (59)+5 (12)§15
Oltmanns [21]Germany63Age: 68±7 years Baseline FVC (% pred): 70±19 Baseline DLCO (% pred): 40±14Stable lung function in 62% of patientsN/AN/A13
Chaudhuri [22]UK40Age: 65.8 (48–80) years Baseline FVC (% pred): 77.3 (range 46–146) Baseline DLCO (% pred): 42.4 (14–81)Reduction in FVC and DLCO decline at 9 months871015
Loeh [23]Germany and Italy197Age: 69.8±0.6 years Baseline FVC (% pred): 65±18.8 Baseline DLCO (% pred): 39.8±17Decrease in annual decline in FVC; greater effect was observed in progressive IPFN/AN/AN/A
Harari [24]Italy128Age: 69±7 years Baseline FVC (% pred): 75±18 Baseline DLCO (% pred): 47±15Decrease in annual decline in FVCƒ; greater effect in patients with more severe disease (GAP II–III and FVC at baseline ≤75% pred)N/AN/AN/A
  • Data are presented as mean±sd, mean (range) or n (%), unless otherwise stated. GI: gastrointestinal; FVC: forced vital capacity; DLCO: diffusing capacity of the lung for carbon monoxide; VC: vital capacity; N/A: not applicable; GAP: GAP severity index (a simple point-scoring system based on gender (G), age (A) and two lung physiology (P) variables (FVC and DLCO). #: gastric distress (n=9) and nausea (n=9); : photosensitivity (n=14) and rash (n=5); +: anorexia and/or nausea; §: photosensitivity; ƒ: relative change in FVC. Reproduced and modified from [25] with permission from the publisher.