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Table 1 Study characteristics

From: Treatment of idiopathic pulmonary fibrosis: a network meta-analysis

  Number of randomized patients ATS/ERS/JRS/ALAT clinical criteria used for diagnosis Pulmonary function tests/blood gas criteria for enrolment Intervention (all compared to placebo) Follow-up used for analysis Risk of bias Mortality Severe adverse events Industry sponsor
Noth 2012 [21] Multicenter (22) USA n = 146 Yes – no bronchoscopy required Progressive decline in FVC (>10 % in last year) or DLCO (>15 % in last year) Warfarin 12 months High (trial stopped early for harm) VKA 14/72 Placebo 3/74 21/72 12/74 None
PANTHER 2012 [22] Multicenter (25) USA n = 155 Yes FVC >50 % predicted DLCO >30 % predicted Triple therapy (NAC, azathioprine, predinisone) 12 months High (trial stopped early for harm) Triple Rx 8/77 Placebo 1/78 24/77 8/78 None
Jackson 2010 [23] Single Center USA n = 29 Yes – no bronchoscopy required FVC 40–90 % predicted DLCO 30–90 % predicted Sildenafil 6 months Low Sildenafil 0/14 Placebo 0/15 0/14 0/15 Pfizer UK
STEP-IPF 2010 [24] Multicenter (14) USA n = 180 Yes DLCO <35 % predicted Sildenafil 6 months Low Sildenafil 3/89 Placebo 9/91 13/89 15/91 Pfizer
Homma 2012 [25] Multicenter (27) Japan n = 88 Yes Also required elevated markers pneumocyte injury (KL-6, surfactant protein A & D) Partial arterial oxygen concentration >70 mmHg at rest NAC monotherapy 12 months Low NAC 0/44 Placebo 0/46 Not reported None
Tomioka 2005 [26] Single Center Japan n = 30 Yes None NAC monotherapy 12 months Low NAC 2/15 Placebo 2/15 0/15 0/15 None
IPF Network 2014 [3] Multicenter (25) USA n = 264 Yes FVC >50 % predicted DLCO >30 % predicted NAC monotherapy 12 months Low NAC 6/133 Placebo 3/131 25/133 20/131 None
IFIGENIA 2005 [36] Multicenter (36) Europe n = 182 No Histologic or radiologic pattern of UIP with other causes ruled out. Mandatory biopsy in patients <50 years old Mandatory bronchoscopy and duration >3 months FVC <80 % predicted TLC <90 % predicted DLCO <80 % predicted NAC monotherapy 12 months Low NAC 7/92 Placebo 8/90 Not reported Zambon
Azuma 2005 [27] Multicenter (25) Japan n = 108 Yes None Pirfenidone 9 months High (trial stopped early for benefit) Pirfenidone 0/73 Placebo 1/35 Not reported Shinogi & Co.
Taniguchi 2010 [29] Multicenter (73) Japan n = 212 Yes None Pirfenidone 12 months Low Pirfenidone 3/108 Placebo 4/104 Not reported None
CAPACITY 2011 [28] Multicenter (110) Worldwide n = 692 Yes FVC >50 % predicted DLCO >35 % predicted Pirfenidone 24 months Low Pirfenidone 27/345 Placebo 34/347 113/345 109/347 Intermune
King Jr 2014 [4] Multicenter (127) Worldwide n = 555 Yes FVC 50–90 % predicted DLCO 30–90 % predicted FEV1/FVC >80 % Pirfenidone 12 months Low Pirfenidone 11/278 Placebo 20/277 52/278 56/277 Intermune
BUILD-1 2008 [30] Multicenter (29) Europe, N. America n = 158 Yes FVC >50 % predicted DLCO >30 % predicted Bosentan 12 months Low Bosentan 3/74 Placebo 3/84 22/74 29/84 Actelion Pharmaceuticals
BUILD-3 2011 [31] Multicenter (119) Worldwide n = 616 Yes None Bosentan 12 months Low Bosentan 17/407 Placebo 7/209 129/407 74/209 Actelion Pharmaceuticals
MUSIC 2013 [33] Multicenter (48) Worldwide n = 178 Yes, with positive biopsy FVC >50 % predicted DLCO >30 % predicted FEV1/FVC >70 % Macitentan 12 months Low Macitentan 3/119 Placebo 2/59 37/119 20/59 Actelion Pharmaceuticals
ARTEMIS 2013 [32] Mulitcenter (136) Worldwide n = 492 Yes None Ambrisentan 12 months High (trial stopped early for harm) Ambrisentan 26/329 Placebo 6/163 73/329 25/163 Gilead Sciences
Daniels 2010 [34] Multicenter (13) USA & Mexico n = 119 Yes FVC >55 % predicted DLCO >35 % predicted FEV1/FVC >60 % Progressive decline in FVC (>10 % in last year) Imatinib 24 months Low Imatinib 8/59 Placebo 10/60 18/59 19/60 Novartis Pharmaceuticals
Richeldi 2011 [35] Multicenter (92) Worldwide n = 428 Yes FVC >50 % predicted DLCO 30–79 % predicted Nintedanib 12 months Low Nintedanib 25/343 Placebo 9/85 90/343 26/85 Boehringer Ingelheim
INPULSUS 2014 [5] Multicenter (205) Worldwide n = 1061 Yes FVC >50 % predicted DLCO 30–79 % predicted Nintedanib 12 months Low Nintedanib 35/638 Placebo 33/423 194/638 127/423 Boehringer Ingelheim
  1. n, Number; VKA, Vitamin K antagonist; FVC, Forced vital capacity; DLCO, Diffusion capacity of lung for carbon monoxide; TLC, Total lung capacity; NAC, N-acetylcysteine