Table 4.

Selected evidence of IPF and SSc-ILD treatment with pirfenidone.

STUDY [REF]	STUDY DESIGN	TREATMENT	N	INCLUSION	F/U	EPs	OUTCOME
Azuma et al, 2005 [73]	Prospective, randomized, double-blind, placebo-controlled	Pirfenidone 720 mg bid for 12 months + 5 mg/day PSL	72 and 35	IPF	12 Mo	Lowest SpO2 during the 6MWT Change in VC, episode of exacerbation	– Better in change in VC measurement, episode of acute exacerbation
Taniguchi et al, 2010 [74]	Prospective, randomized, double-blind, placebo-controlled	Pirfenidone 1.8 g/day, 1.2 mg/day, or placebo	108 and 55 and 104	IPF	12 Mo	The change in VC, progression-free survival	– Better in VC decline, progression free survival
Noble et al, 2006 [75]	Prospective, randomized, double-blind, placebo-controlled	Pirfenidone 2403 mg/day, 1197 mg/day, or placebo	174 and 87 and 174	IPF	72 Wk	The change in %FVC	– Better in change in %FVC
Miura et al, 2014 [76]	Case series	Pirfenidone	5	SSc-ILD	N/A	N/A	– Increase in VC
Nagai et al, 2002 [77]	Open-label	Pirfenidone 40 mg/kg/day	10	8 IPF 2 SSc-ILD	12 Mo	Overall survival, chest radiographic score, arterial oxygen pressure	– No deterioration of chest radiographic score, arterial oxygen pressure
Udiwadia ZF et al, 2015 [78]	Case report	Pirfenidone 600 mg/day	1	SSc-ILD	20 Mo	The change of %FVC, %DLCO, 6MWD	– PFT stabilized, 6MWD improved

Abbreviations: EP, endpoint; IPF, idiopathic pulmonary fibrosis; VC, vital capacity of lungs; FVC, forced vital capacity; FEV₁, forced expiratory volume in 1 second; TLC, total lung capacity; DLCO, diffusing capacity of the lung for carbon monoxide; ILD, interstitial lung disease; Mo, months; Wk, weeks; 6MWT, 6-minute walk test; 6MWD, 6-minute walk distance; N/A, not available.