Table 7.

Selected evidence of IPF or SSc-ILD treatment with tyrosine kinase inhibitors.

STUDY [REF]	STUDY DESIGN	TREATMENT	N	INCLUSION	F/U	EPs	OUTCOME
Daniels et al, 2010 [121]	Randomized, double-blinded, placebo-controlled	Imatinib 600 mg/day vs placebo	119	IPF	96 w	TTDP Change in %FVC, %DLCO	– N o change of TTDP, change in %FVC, %DLCO
Spiera et al, 2011 [122]	Open-label	Imatinib 400 mg/day	30	dcSSc (16 with ILD)	12 Mo	Change in %FVC, %DLCO	– Increase in %FVC and–Trend toward increase in %DLCO
Khanna et al, 2011 [123]	Open-label	Imatinib 600 mg/day	20	SSc-ILD FVC <85%, DOE, GGO on HRCT	12 Mo	%FVC, %DLCO, %TLC	– 7 patients dropped – T rends toward improvement of %FVC, %DLCO, %TLC
Pope et al, 2011 [124]	Randomized, double-blinded, placebo-controlled	Imatinib 400 mg/day vs placebo	9 and 1	dcSSc	6 Mo	FVC, TLC, DLCO	– 5 of 9 patients dropped
Fraticelli et al, 2014 [125]	Open-label	Imatinib 200 mg/day	30	SSc-ILD patients with grade 2 by MBDI plus HRCT findings or BAL findings	6 Mo	FVC, DLCO, PaO2, FVC, HRCT	– 4 patients dropped – 4 good response, 15 stabilized, 7 worsened
Richeldi et al, 2014 [126]	Randomized, double-blinded, placebo-controlled	Nintedanib(300 mg/day) vs placebo	7	IPF	52 wk	FVC	– Reduced the decline in FVC

Abbreviations: EP, endpoint; HRCT, high-resolution computed tomography; TTDP, time to disease progression (10% decline in%FVC from baseline); PFT, pulmonary function test; FVC, forced vital capacity; TLC, total lung capacity; DLCO, diffusing capacity of the lung for carbon monoxide; ILD, interstitial lung disease; MBDI, Mahler Baseline Dyspnea Index; Mo, months; wk, weeks; DOE, dyspnea on exertion.