Table 1 Studies included in this review
Study | Study type | Experimental treatment | Country | Sample size1 | Disease duration (mo) mean (SD) | Baseline SGRQ score2 | Baseline spirometric values2 | ||
|---|---|---|---|---|---|---|---|---|---|
All | IPF | FVC% predicted | DLCO% predicted | ||||||
Antoniou et al., 2006 [13] | RCT | Interferon gamma b | Greece | 50 | 50 | T = 49.4 (24.3) C = 42.7 (16.8) | -- | T = 71.8 (15.0) C = 70.7 (17.7) | -- |
Berry et al., 2012 [14] | Secondary validation study | n/a | US | 405 | 239 | -- | -- | 66.0 (52–78) | -- |
Chang et al., 1999 [15] | Standalone validation study | n/a | US | 50 | 33 | -- | Total = 38.9 [28.7–55.2] Symptoms domain = 50.5 [31.5–69.8], Activity domain = 54.4 [39.9–72.9], Impact domain = 28.4 [18.8–45.1] | 65.0 (49.0–81.0) | 49.0 (36.5–59.8) |
du Bois et al., 2011 [16] | RCT | Interferon gamma b | Multi-national | 822 | 822 | -- | Total = 41.8 (18) | 72.5 (12.7) | 47.4 (9.2) |
Han et al., 2013 [17] | RCT | Sildenafil | US | 119 | 119 | 20.4 | -- | 56.9 | 26.0 |
Horton et al., 2012 [18] | RCT | Thalidomide | US | 23 | 23 | 20.5 (3–59) | Total = 57.4 (18.8) Symptoms domain = 67.7 (19.7), Activity domain = 64.3 (22.7), Impact domain = 48.1 (20.7) | 70.4 (13.7) | 57.4 (14.4) |
King, Jr. et al., 2008 [19] | RCT | Bosentan | Multi-national | 158 | 158 | T = 12.2 (12.2) C = 12.1 (12.0) | T-Total = 45.7 (18.1), C-Total = 45.2 (19) | T = 65.9 (10.5), C = 69.5 (12.6) | T = 42.3 (9.5), C = 41.4 (9.5) |
King, Jr. et al., 2009 [20] | RCT | Interferon gamma b | Multi-national | 826 | 826 | – | T-Total = 41.6 (17.9), C-Total = 42.4 (18.2) | T = 72.2 (12.3), C = 73.1 (13.4) | T = 47.4 (9.2), C = 47.3 (9.3) |
Lechtzin et al., 2013 [21] | RCT | Thalidomide | US | 24 | 24 | -- | Total = 57.4 (18.8), Symptoms domain = 67.7 (19.7), Activity domain = 64.3 (22.7), Impact domain = 48.1 (20.7) | 70.4 (13.7) | 57.4 (14.4) |
Mishra et al., 2011 [22] | Within-subject trial | Oral doxycycline | India | 6 | 6 | -- | Total = 50.90 (8.38) | n/a | n/a |
Naji et al., 2006 [23] | Within-subject trial | Pulmonary rehabilitation | Ireland | 26 | 19 | -- | Total = 48 (27.6, 67.9) | 66.7 (20.7) | 42.5 (14) |
Nishiyama et al., 2005 [24] | Standalone validation study | n/a | Japan | 41 | 41 | -- | Total = 35.7 (20.6) [range 1.6–77.6], Symptoms domain = 40.1 (24.6) [range 4.4–85.6], Activity domain = 44.5 (26.7) [range 0–93.9], Impact domain = 28.9 (19.8) [range 0–77.0] | n/a | n/a |
Nishiyama et al., 2008 [25] | RCT | Pulmonary rehabilitation | Japan | 28 | 28 | -- | T-Total = 50.2 (16.3), T-Symptoms domain = 56.4 (22.3), T-Activity domain = 64.7 (17.1), T-Impact domain = 39.7 (17.6), C-Total = 37.8 (22.7), C-Symptoms domain = 38.0 (25.8), C-Activity domain = 50.4 (26.2), C-Impact domain = 29.9 (23.7) | T = 66.1 (13.2), C = 68.7 (19.5) | T = 59.4 (16.7), C = 48.6 (16.7) |
Noth et al., 2012 [26] | RCT | Warfarin | US | 145 | 145 | T = 21.6, C = 25.2 | T-Total = 46.2 (18.0), C-Total = 50.1 (17.2) | T = 58.9 (16.2), C = 58.7 (16.1) | T = 33.8 (12.4), C = 34.6 (13.4) |
Patel et al., 2012 [27] | Standalone validation study | n/a | UK | 173 | 49 | 48.0 | -- | 82 (34–143) | -- |
Peng et al., 2008 [28] | Standalone validation study | n/a | China | 68 | 68 | 14.0 (14.0) | Total = 54 (15), Symptoms domain = 65 (16), Activity domain = 56 (15), Impact domain = 49 (19) | 66 (18) | 54 (16) |
Raghu et al., 2004 [29] | RCT | Interferon gamma b | Multi-national | 330 | 330 | -- | -- | T = 63.9 (10.7), C = 64.1 (11.3) | -- |
Raghu et al., 2008 [30] | RCT | Etanercept | Multi-national | 88 | 88 | T = 14.7 (19.8), C = 12.3 (13.6) | T-Total = 40.8 (18.1), C-Total = 42.9 (19.4) | T = 64.7 (14.1), C = 63.0 (12.7) | T = 36.3 (12.6), C = 36.9 (10.8) |
Raghu et al., 2013 [31] | RCT | Ambrisentan | Multi-national | 492 | 492 | T = 13.2, C = 10.8 | T-Total = 44.5 (21.6), C-Total = 40.5 (21.1) | T = 68.7 (13.1), C = 69.9 (13.8) | T = 42.0 (13.8), C = 45.6 (13.3) |
Rammaert et al., 2009 [32] | Within-subject trial | Pulmonary rehabilitation | France | 13 | 13 | -- | -- | 67 (14) | 32 (13) |
Richeldi et al., 2011 [33] | RCT | Nintedanib (BIBF 1120) | Multi-national | 428 | 428 | T, 50 mg qd = 16.8 (15.6), T, 50 mg bid = 13.2 (14.4), T, 100 mg bid = 14.4 (14.4), T, 150 mg bid = 12 (14.4), C = 16.8 (18) | T, 50 mg qd-Total = 43.7 (17.5), T, 50 mg bid-Total = 42.5 (17.0), T, 100 mg bid-Total = 43.7 (16.6), T, 150 mg bid-Total = 40.1 (18.3), C-Total = 41.2 (17.9) | T, 50 mg qd = 80.4 (17.8), T, 50 mg bid = 79.8 (15.8), T, 100 mg bid = 85.5 (19.2), T, 150 mg bid = 79.1 (18.5), C = 81.7 (17.6) | – |
Swigris et al., 2010 [34] | Secondary validation study | Bosentan | Multi-national | 158 | 158 | -- | Total = 44.8 (19.5), Symptoms domain = 50.1 (21.9), Activity domain = 60.6 (22.8), Impact domain = 33.7 (20.6) | 67.0 (12.8) | 40.98 (10.1) |
Swigris et al., 2012 [35] | Secondary validation study | Sildenafil | US | 180 | 180 | 24.0 | Activity domain = 69.6 (17.6) | 56.8 (14.2) | 26.3 (6.1) |
Tzanakis et al., 2005 [36] | Standalone validation study | n/a | Greece | 25 | 25 | 31.2 | Total = 37.7 (18.9), Symptoms domain = 55.9 (25.3), Activity domain = 36.2 (21.4), Impact domain = 29.6 (21) | 68.8 (16) | -- |
Tzouvelekis et al., 2013 [37] | Within-subject trial | Adipose-derived stromal cells | Greece | 14 | 14 | -- | -- | -- | -- |
Verma et al., 2011 [38] | Standalone validation study | n/a | Canada | 137 | 137 | – | Total = 63.4 (3.7–96.3), Symptoms domain = 59.8 (0–97.2), Activity domain = 81.6 (6.0–99.5), Impact domain = 54.1 (0–96.4) | 61.7 (19.8) | 49.5 (17.9) |
Yorke et al., 20103[10] | Secondary validation study | Bosentan | Multi-national | 158 | 158 | -- | -- | 61.0 (12.2) | -- |
Yorke et al., 2011 [39] | Standalone validation study | n/a | Multi-national | 101 | 67 | -- | Total = 53 (24), Symptoms domain = 61 (23), Activity domain = 65 (30), Impact domain = 41 (24) | 77 (19.5) | 51.6 (21) |
Zimmermann et al., 2007 [40] | Standalone validation study | n/a | Brazil | 22 | 22 | -- | Total = 48.4 (17.9), Symptoms domain = 46.4 (20.3), Activity domain = 62.4 (19), Impact domain = 43.6 (20.9) | 70.4 (19.4) | 41.5 (16.2) |
Zisman et al., 2010 [41] | RCT | Sildenafil | US | 180 | 180 | T = 24.4, C = 22.4 | T-Total = 54.55 (16.46), C-Total = 51.72 (15.86) | T = 54.9 (14.00), C = 58.7 (14.12) | -- |