TABLE 1

Real-life experience with pirfenidone in observational studies

First author [ref.]	Country	Patients n	Patient characteristics	Efficacy outcome	Adverse events		Treatment discontinuation due to adverse events %
First author [ref.]	Country	Patients n	Patient characteristics	Efficacy outcome	GI	Skin	Treatment discontinuation due to adverse events %
Bonella [18]	Germany	45	Age: 69±7 years Baseline FVC (% pred): 61±15 Baseline D_LCO (% pred): 48±14	Stable lung function in 28 (70%) out of 40 patients; subjective improvement in cough in 12 (33%) out of 36 patients	17 (38)	10 (22)	13
Okuda [19]	Japan	76	Age: 70.5±8.3 years Baseline FVC (% pred): 65.3±16.1 Baseline D_LCO (% pred): 55.9±17.8	Reduction in FVC and D_LCO decline	18 (24)^#	19 (25)^¶	18
Arai [20]	Japan	41	Age: 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]	Germany	63	Age: 68±7 years Baseline FVC (% pred): 70±19 Baseline D_LCO (% pred): 40±14	Stable lung function in 62% of patients	N/A	N/A	13
Chaudhuri [22]	UK	40	Age: 65.8 (48–80) years Baseline FVC (% pred): 77.3 (range 46–146) Baseline D_LCO (% pred): 42.4 (14–81)	Reduction in FVC and D_LCO decline at 9 months	87	10	15
Loeh [23]	Germany and Italy	197	Age: 69.8±0.6 years Baseline FVC (% pred): 65±18.8 Baseline D_LCO (% pred): 39.8±17	Decrease in annual decline in FVC; greater effect was observed in progressive IPF	N/A	N/A	N/A
Harari [24]	Italy	128	Age: 69±7 years Baseline FVC (% pred): 75±18 Baseline D_LCO (% pred): 47±15	Decrease in annual decline in FVC^ƒ; greater effect in patients with more severe disease (GAP II–III and FVC at baseline ≤75% pred)	N/A	N/A	N/A

Data are presented as mean±sd, mean (range) or n (%), unless otherwise stated. GI: gastrointestinal; FVC: forced vital capacity; D_LCO: 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 D_LCO). ^#: 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.