Treatment of idiopathic pulmonary fibrosis: a position paper from a Nordic expert group

Journal of Internal Medicine

Volumn 281, Issue 2, 2017, Pages 149-166

  Skold C M ^a,b   Bendstrup, E ^c   Myllarniemi M ^d   Gudmundsson, G ^e   Sjaheim T ^f   Hilberg, O ^c   Altraja, A ^g,h   Kaarteenaho, R ^i,j,k   Ferrara, G ^a,b  

^a KAROLINSKA INSTITUTE   (Sweden)

^b KAROLINSKA UNIVERSITY HOSPITAL   (Sweden)

^c AARHUS UNIVERSITY HOSPITAL   (Denmark)

^d HELSINKI UNIVERSITY CENTRAL HOSPITAL   (Finland)

^e LANDSPITALI UNIVERSITY HOSPITAL   (Iceland)

^f OSLO UNIVERSITY HOSPITAL   (Norway)

^g UNIVERSITY OF TARTU   (Estonia)

^h TARTU UNIVERSITY HOSPITAL   (Estonia)

ⁱ UNIVERSITY OF EASTERN FINLAND   (Finland)

^j UNIVERSITY OF OULU   (Finland)

^k KUOPIO UNIVERSITY HOSPITAL   (Finland)

more..

Author keywords

idiopathic interstitial pneumonias; lung transplantation; nintedanib; pirfenidone; pulmonary rehabilitation

Indexed keywords

ACETYLCYSTEINE; ANTACID AGENT; ANTICOAGULANT AGENT; CORTICOSTEROID; ENDOTHELIN RECEPTOR ANTAGONIST; GAMMA INTERFERON; IMATINIB; IMMUNOMODULATING AGENT; NINTEDANIB; PHOSPHODIESTERASE INHIBITOR; PIRFENIDONE; TUMOR NECROSIS FACTOR INHIBITOR; INDOLE DERIVATIVE; NONSTEROID ANTIINFLAMMATORY AGENT; PYRIDONE DERIVATIVE;

AIRWAY OBSTRUCTION; ALGORITHM; COUGHING; DISEASE COURSE; DYSPNEA; EMPHYSEMA; FIBROSING ALVEOLITIS; HUMAN; LUNG CANCER; LUNG TRANSPLANTATION; MEDLINE; MONOTHERAPY; OXYGEN THERAPY; PALLIATIVE THERAPY; PRACTICE GUIDELINE; PRIORITY JOURNAL; PULMONARY HYPERTENSION; PULMONARY REHABILITATION; RANDOMIZED CONTROLLED TRIAL (TOPIC); RESPIRATORY FAILURE; REVIEW; SYSTEMATIC REVIEW; VENTILATOR; COMPLICATION; IDIOPATHIC PULMONARY FIBROSIS;

ALGORITHMS; ANTI-INFLAMMATORY AGENTS, NON-STEROIDAL; HUMANS; IDIOPATHIC PULMONARY FIBROSIS; INDOLES; PYRIDONES;

EID: 85003946087     PISSN: 09546820     EISSN: 13652796     Source Type: Journal    
DOI: 10.1111/joim.12571     Document Type: Review

References (134)

1. Raghu G, Weycker D, Edelsberg J, Bradford WZ, Oster G. Incidence and prevalence of idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 2006; 174: 810–6.
2. Kaunisto J, Salomaa ER, Hodgson U, Kaarteenaho R, Myllarniemi M. Idiopathic pulmonary fibrosis–a systematic review on methodology for the collection of epidemiological data. BMC Pulm Med 2013; 13: 53.
3. Hyldgaard C, Hilberg O, Muller A, Bendstrup E. A cohort study of interstitial lung diseases in central Denmark. Respir Med 2014; 108: 793–9.
4. du Bois RM. An earlier and more confident diagnosis of idiopathic pulmonary fibrosis. Eur Respir Rev 2012; 21: 141–6.
5. American Thoracic Society, European Respiratory Society. American Thoracic Society/European Respiratory Society International Multidisciplinary Consensus Classification of the Idiopathic Interstitial Pneumonias. This joint statement of the American Thoracic Society (ATS), and the European Respiratory Society (ERS) was adopted by the ATS board of directors, June 2001 and by the ERS Executive Committee, June 2001. Am J Respir Crit Care Med 2002; 165: 277–304.
6. Travis WD, Costabel U, Hansell DM et al. An official American Thoracic Society/European Respiratory Society statement: update of the international multidisciplinary classification of the idiopathic interstitial pneumonias. Am J Respir Crit Care Med 2013; 188: 733–48.
7. Raghu G, Collard HR, Egan JJ et al. An official ATS/ERS/JRS/ALAT statement: idiopathic pulmonary fibrosis: evidence-based guidelines for diagnosis and management. Am J Respir Crit Care Med 2011; 183: 788–824.
8. Raghu G, Rochwerg B, Zhang Y et al. An Official ATS/ERS/JRS/ALAT clinical practice guideline: treatment of idiopathic pulmonary fibrosis. an update of the 2011 clinical practice guideline. Am J Respir Crit Care Med 2015; 192: e3–19.
9. Bendstrup E, Hyldgaard C, Altraja A et al. Organisation of diagnosis and treatment of idiopathic pulmonary fibrosis and other interstitial lung diseases in the Nordic countries. Eur Clin Respir J 2015; 2: 28348. http://dx.doi.org/10.3402/ecrj.v2.28348.
10. Thabut G, Mal H, Castier Y et al. Survival benefit of lung transplantation for patients with idiopathic pulmonary fibrosis. J Thorac Cardiovasc Surg 2003; 126: 469–75.
11. Lederer DJ, Arcasoy SM, Wilt JS, D'Ovidio F, Sonett JR, Kawut SM. Six-minute-walk distance predicts waiting list survival in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 2006; 174: 659–64.
12. Weill D, Benden C, Corris PA et al. A consensus document for the selection of lung transplant candidates: 2014–an update from the Pulmonary Transplantation Council of the International Society for Heart and Lung Transplantation. J Heart Lung Transplant 2015; 34: 1–15.
13. Gottlieb J, Greer M, Sommerwerck U et al. Introduction of the lung allocation score in Germany. Am J Transplant 2014; 14: 1318–27.
14. Brown AW, Kaya H, Nathan SD. Lung transplantation in IIP: a review. Respirology 2016; 21: 1173–84.
15. Christie JD, Edwards LB, Kucheryavaya AY et al. The registry of the INTERNATIONAL Society for Heart and Lung Transplantation: twenty-eighth Adult Lung and Heart-Lung Transplant Report–2011. J Heart Lung Transplant 2011; 30: 1104–22.
16. Yusen RD, Edwards LB, Kucheryavaya AY et al. The registry of the International Society for Heart and Lung Transplantation: thirty-second official Adult Lung and Heart-Lung Transplantation Report–2015; Focus Theme: Early Graft Failure. J Heart Lung Transplant 2015; 34: 1264–77.
17. Force SD, Kilgo P, Neujahr DC et al. Bilateral lung transplantation offers better long-term survival, compared with single-lung transplantation, for younger patients with idiopathic pulmonary fibrosis. Ann Thorac Surg 2011; 91: 244–9.
18. Mason DP, Brizzio ME, Alster JM et al. Lung transplantation for idiopathic pulmonary fibrosis. Ann Thorac Surg 2007; 84: 1121–8.
19. Neurohr C, Huppmann P, Thum D et al. Potential functional and survival benefit of double over single lung transplantation for selected patients with idiopathic pulmonary fibrosis. Transpl Int 2010; 23: 887–96.
20. Kistler KD, Nalysnyk L, Rotella P, Esser D. Lung transplantation in idiopathic pulmonary fibrosis: a systematic review of the literature. BMC Pulm Med 2014; 14: 139.
21. Schaffer JM, Singh SK, Reitz BA, Zamanian RT, Mallidi HR. Single- vs double-lung transplantation in patients with chronic obstructive pulmonary disease and idiopathic pulmonary fibrosis since the implementation of lung allocation based on medical need. JAMA 2015; 313: 936–48.
22. Grunnet N, Bodvarsson M, Jakobsen A et al. Aspects of present and future data presentation in Scandiatransplant. Transplant Proc 2009; 41: 732–5.
23. Dellgren G, Riise GC, Sward K et al. Extracorporeal membrane oxygenation as a bridge to lung transplantation: a long-term study. Eur J Cardiothorac Surg 2015; 47: 95–100; discussion.
24. Nishiyama O, Miyajima H, Fukai Y et al. Effect of ambulatory oxygen on exertional dyspnea in IPF patients without resting hypoxemia. Respir Med 2013; 107: 1241–6.
25. Douglas WW, Ryu JH, Schroeder DR. Idiopathic pulmonary fibrosis: impact of oxygen and colchicine, prednisone, or no therapy on survival. Am J Respir Crit Care Med 2000; 161: 1172–8.
26. Long term domiciliary oxygen therapy in chronic hypoxic cor pulmonale complicating chronic bronchitis and emphysema. Report of the Medical Research Council Working Party. Lancet 1981; 1: 681–6.
27. Continuous or nocturnal oxygen therapy in hypoxemic chronic obstructive lung disease: a clinical trial. Nocturnal Oxygen Therapy Trial Group. Ann Intern Med 1980; 93: 391–8.
28. Mallick S. Outcome of patients with idiopathic pulmonary fibrosis (IPF) ventilated in intensive care unit. Respir Med 2008; 102: 1355–9.
29. Gaudry S, Vincent F, Rabbat A et al. Invasive mechanical ventilation in patients with fibrosing interstitial pneumonia. J Thorac Cardiovasc Surg 2014; 147: 47–53.
30. Dreher M, Ekkernkamp E, Schmoor C, Schoenheit-Kenn U, Winterkamp S, Kenn K. Pulmonary rehabilitation and noninvasive ventilation in patients with hypercapnic interstitial lung disease. Respiration 2015; 89: 208–13.
31. Dowman L, Hill CJ, Holland AE. Pulmonary rehabilitation for interstitial lung disease. Cochrane Database Syst Rev 2014; 10: CD006322.
32. Ferreira A, Garvey C, Connors GL et al. Pulmonary rehabilitation in interstitial lung disease: benefits and predictors of response. Chest 2009; 135: 442–7.
33. Gaunaurd IA, Gomez-Marin OW, Ramos CF et al. Physical activity and quality of life improvements of patients with idiopathic pulmonary fibrosis completing a pulmonary rehabilitation program. Respir Care 2014; 59: 1872–9.
34. Vainshelboim B, Oliveira J, Fox BD, Soreck Y, Fruchter O, Kramer MR. Long-term effects of a 12-week exercise training program on clinical outcomes in idiopathic pulmonary fibrosis. Lung 2015; 193: 345–54.
35. Holland AE, Dowman LM, Hill CJ. Principles of rehabilitation and reactivation: interstitial lung disease, sarcoidosis and rheumatoid disease with respiratory involvement. Respiration 2015; 89: 89–99.
36. Kenn K, Gloeckl R, Behr J. Pulmonary rehabilitation in patients with idiopathic pulmonary fibrosis–a review. Respiration 2013; 86: 89–99.
37. Jackson RM, Gomez-Marin OW, Ramos CF et al. Exercise limitation in IPF patients: a randomized trial of pulmonary rehabilitation. Lung 2014; 192: 367–76.
38. Holland AE, Hill CJ, Conron M, Munro P, McDonald CF. Short term improvement in exercise capacity and symptoms following exercise training in interstitial lung disease. Thorax 2008; 63: 549–54.
39. Ryerson CJ, Cayou C, Topp F et al. Pulmonary rehabilitation improves long-term outcomes in interstitial lung disease: a prospective cohort study. Respir Med 2014; 108: 203–10.
40. Ryerson CJ, Abbritti M, Ley B, Elicker BM, Jones KD, Collard HR. Cough predicts prognosis in idiopathic pulmonary fibrosis. Respirology 2011; 16: 969–75.
41. Kohberg C, Andersen CU, Bendstrup E. Opioids: an unexplored option for treatment of dyspnea in IPF. Eur Clin Respir J 2016; 3: 30629.
42. Horton MR, Santopietro V, Mathew L et al. Thalidomide for the treatment of cough in idiopathic pulmonary fibrosis: a randomized trial. Ann Intern Med 2012; 157: 398–406.
43. Chung KF. Currently available cough suppressants for chronic cough. Lung 2008; 186(Suppl 1): S82–7.
44. Ryan NM, Birring SS, Gibson PG. Gabapentin for refractory chronic cough: a randomised, double-blind, placebo-controlled trial. Lancet 2012; 380: 1583–9.
45. Ekstrom MP, Bornefalk-Hermansson A, Abernethy AP, Currow DC. Safety of benzodiazepines and opioids in very severe respiratory disease: national prospective study. BMJ 2014; 348: g445.
46. Sgalla G, Cerri S, Ferrari R et al. Mindfulness-based stress reduction in patients with interstitial lung diseases: a pilot, single-centre observational study on safety and efficacy. BMJ Open Respir Res 2015; 2: e000065.
47. Wijsenbeek M, Bendstrup E, Ross J, Wells A. Cultural Differences in Palliative Care in Patients With Idiopathic Pulmonary Fibrosis. Chest 2015; 148: e56.
48. Lindell KO, Liang Z, Hoffman LA et al. Palliative care and location of death in decedents with idiopathic pulmonary fibrosis. Chest 2015; 147: 423–9.
49. du Bois RM, Weycker D, Albera C et al. Forced vital capacity in patients with idiopathic pulmonary fibrosis: test properties and minimal clinically important difference. Am J Respir Crit Care Med 2011; 184: 1382–9.
50. Taniguchi H, Kondoh Y, Ebina M et al. The clinical significance of 5% change in vital capacity in patients with idiopathic pulmonary fibrosis: extended analysis of the pirfenidone trial. Respir Res 2011; 12: 93.
51. Zappala CJ, Latsi PI, Nicholson AG et al. Marginal decline in forced vital capacity is associated with a poor outcome in idiopathic pulmonary fibrosis. Eur Respir J 2010; 35: 830–6.
52. Richeldi L, Ryerson CJ, Lee JS et al. Relative versus absolute change in forced vital capacity in idiopathic pulmonary fibrosis. Thorax 2012; 67: 407–11.
53. Richeldi L, Davies HR, Ferrara G, Franco F. Corticosteroids for idiopathic pulmonary fibrosis. Cochrane Database Syst Rev 2003; 3: CD002880.
54. Raghu G, Depaso WJ, Cain K et al. Azathioprine combined with prednisone in the treatment of idiopathic pulmonary fibrosis: a prospective double-blind, randomized, placebo-controlled clinical trial. Am Rev Respir Dis 1991; 144: 291–6.
55. Johnson MA, Kwan S, Snell NJ, Nunn AJ, Darbyshire JH, Turner-Warwick M. Randomised controlled trial comparing prednisolone alone with cyclophosphamide and low dose prednisolone in combination in cryptogenic fibrosing alveolitis. Thorax 1989; 44: 280–8.
56. Pereira CA, Malheiros T, Coletta EM et al. Survival in idiopathic pulmonary fibrosis-cytotoxic agents compared to corticosteroids. Respir Med 2006; 100: 340–7.
57. Demedts M, Behr J, Buhl R et al. High-dose acetylcysteine in idiopathic pulmonary fibrosis. N Engl J Med 2005; 353: 2229–42.
58. Idiopathic Pulmonary Fibrosis Clinical Research Network, Raghu G, Anstrom KJ, King TE Jr, Lasky JA, Martinez FJ. Prednisone, azathioprine, and N-acetylcysteine for pulmonary fibrosis. N Engl J Med 2012; 366: 1968–77.
59. Myllärniemi MKR. Pharmacological treatment of idiopathic pulmonary fibrosis - preclinical and clinical studies of pirfenidone, nintedanib and N-acetylcysteine. Eur Clin Resp J 2015; 2: 1–10.
60. Idiopathic Pulmonary Fibrosis Clinical Research Network, Martinez FJ, de Andrade JA, Anstrom KJ, King TE Jr, Raghu GRandomized trial of acetylcysteine in idiopathic pulmonary fibrosis. N Engl J Med 2014; 370: 2093–101.
61. Behr J, Bendstrup E, Crestani B et al. Safety and tolerability of acetylcysteine and pirfenidone combination therapy in idiopathic pulmonary fibrosis: a randomised, double-blind, placebo-controlled, phase 2 trial. Lancet Respir Med 2016; 4: 445–53.
62. Homma S, Azuma A, Taniguchi H et al. Efficacy of inhaled N-acetylcysteine monotherapy in patients with early stage idiopathic pulmonary fibrosis. Respirology 2012; 17: 467–77.
63. Sakamoto S, Muramatsu Y, Satoh K et al. Effectiveness of combined therapy with pirfenidone and inhaled N-acetylcysteine for advanced idiopathic pulmonary fibrosis: a case-control study. Respirology 2015; 20: 445–52.
64. Oldham JM, Ma SF, Martinez FJ et al. TOLLIP, MUC5B and the Response to N-acetylcysteine Among Individuals with Idiopathic Pulmonary Fibrosis. Am J Respir Crit Care Med 2015; 192: 1475–82.
65. Noth I, Zhang Y, Ma SF et al. Genetic variants associated with idiopathic pulmonary fibrosis susceptibility and mortality: a genome-wide association study. Lancet Respir Med 2013; 1: 309–17.
66. Fingerlin TE, Murphy E, Zhang W et al. Genome-wide association study identifies multiple susceptibility loci for pulmonary fibrosis. Nat Genet 2013; 45: 613–20.
67. Raghu G, Brown KK, Bradford WZ et al. A placebo-controlled trial of interferon gamma-1b in patients with idiopathic pulmonary fibrosis. N Engl J Med 2004; 350: 125–33.
68. King TE Jr, Albera C, Bradford WZ et al. Effect of interferon gamma-1b on survival in patients with idiopathic pulmonary fibrosis (INSPIRE): a multicentre, randomised, placebo-controlled trial. Lancet 2009; 374: 222–8.
69. King TE Jr, Behr J, Brown KK et al. BUILD-1: a randomized placebo-controlled trial of bosentan in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 2008; 177: 75–81.
70. King TE Jr, Brown KK, Raghu G et al. BUILD-3: a randomized, controlled trial of bosentan in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 2011; 184: 92–9.
71. Raghu G, Million-Rousseau R, Morganti A, Perchenet L, Behr J; MUSIC Study Group. Macitentan for the treatment of idiopathic pulmonary fibrosis: the randomised controlled MUSIC trial. Eur Respir J 2013; 42: 1622–32.
72. Corte TJ, Keir GJ, Dimopoulos K et al. Bosentan in pulmonary hypertension associated with fibrotic idiopathic interstitial pneumonia. Am J Respir Crit Care Med 2014; 190: 208–17.
73. Raghu G, Behr J, Brown KK et al. Treatment of idiopathic pulmonary fibrosis with ambrisentan: a parallel, randomized trial. Ann Intern Med 2013; 158: 641–9.
74. Idiopathic Pulmonary Fibrosis Clinical Research Network, Zisman DA, Schwarz M, Anstrom KJ, Collard HR, Flaherty KR et al. A controlled trial of sildenafil in advanced idiopathic pulmonary fibrosis. N Engl J Med 2010; 363: 620–8.
75. Collard HR, Anstrom KJ, Schwarz MI, Zisman DA. Sildenafil improves walk distance in idiopathic pulmonary fibrosis. Chest 2007; 131: 897–9.
76. Han MK, Bach DS, Hagan PG et al. Sildenafil preserves exercise capacity in patients with idiopathic pulmonary fibrosis and right-sided ventricular dysfunction. Chest 2013; 143: 1699–708.
77. Raghu G, Brown KK, Costabel U et al. Treatment of idiopathic pulmonary fibrosis with etanercept: an exploratory, placebo-controlled trial. Am J Respir Crit Care Med 2008; 178: 948–55.
78. Kubo H, Nakayama K, Yanai M et al. Anticoagulant therapy for idiopathic pulmonary fibrosis. Chest 2005; 128: 1475–82.
79. Noth I, Anstrom KJ, Calvert SB et al. A placebo-controlled randomized trial of warfarin in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 2012; 186: 88–95.
80. Tomassetti S, Ruy JH, Gurioli C et al. The effect of anticoagulant therapy for idiopathic pulmonary fibrosis in real life practice. Sarcoidosis Vasc Diffuse Lung Dish 2013; 30: 121–7.
81. Hyldgaard C, Hilberg O, Bendstrup E. How does comorbidity influence survival in idiopathic pulmonary fibrosis? Respir Med 2014; 108: 647–53.
82. Kreuter M, Wijsenbeek MS, Vasakova M et al. Unfavourable effects of medically indicated oral anticoagulants on survival in idiopathic pulmonary fibrosis. Eur Respir J 2016; 47: 1776–84.
83. Lee JS, Collard HR, Anstrom KJ et al. Anti-acid treatment and disease progression in idiopathic pulmonary fibrosis: an analysis of data from three randomised controlled trials. Lancet Respir Med 2013; 1: 369–76.
84. Kreuter M, Wuyts W, Renzoni E et al. Antacid therapy and disease outcomes in idiopathic pulmonary fibrosis: a pooled analysis. Lancet Respir Med 2016; 4: 381–9.
85. Lee JS, Ryu JH, Elicker BM et al. Gastroesophageal reflux therapy is associated with longer survival in patients with idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 2011; 184: 1390–4.
86. Raghu G. Idiopathic pulmonary fibrosis: increased survival with “gastroesophageal reflux therapy”: fact or fallacy? Am J Respir Crit Care Med 2011; 184: 1330–2.
87. Daniels CE, Lasky JA, Limper AH et al. Imatinib treatment for idiopathic pulmonary fibrosis: randomized placebo-controlled trial results. Am J Respir Crit Care Med 2010; 181: 604–10.
88. Azuma A, Nukiwa T, Tsuboi E et al. Double-blind, placebo-controlled trial of pirfenidone in patients with idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 2005; 171: 1040–7.
89. Taniguchi H, Ebina M, Kondoh Y et al. Pirfenidone in idiopathic pulmonary fibrosis. Eur Respir J 2010; 35: 821–9.
90. Noble PW, Albera C, Bradford WZ et al. Pirfenidone in patients with idiopathic pulmonary fibrosis (CAPACITY): two randomised trials. Lancet 2011; 377: 1760–9.
91. King TE Jr, Bradford WZ, Castro-Bernardini S et al. A phase 3 trial of pirfenidone in patients with idiopathic pulmonary fibrosis. N Engl J Med 2014; 370: 2083–92.
92. Loeh B, Drakopanagiotakis F, Bandelli GP et al. Intraindividual response to treatment with pirfenidone in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 2015; 191: 110–3.
93. Harari S, Caminati A. Idiopathic pulmonary fibrosis: from clinical trials to real-life experiences. Eur Respir Rev 2015; 24: 420–7.
94. Noble PW, Albera C, Bradford WZ et al. Pirfenidone for idiopathic pulmonary fibrosis: analysis of pooled data from three multinational phase 3 trials. Eur Respir J 2016; 47: 243–53.
95. Hilberg F, Roth GJ, Krssak M et al. BIBF 1120: triple angiokinase inhibitor with sustained receptor blockade and good antitumor efficacy. Cancer Res 2008; 68: 4774–82.
96. Myllarniemi M, Kaarteenaho R. Pharmacological treatment of idiopathic pulmonary fibrosis - preclinical and clinical studies of pirfenidone, nintedanib, and N-acetylcysteine. Eur Clin Respir J 2015; 2: doi: 10.3402/ecrj.v2.26385.
97. Wollin L, Wex E, Pautsch A et al. Mode of action of nintedanib in the treatment of idiopathic pulmonary fibrosis. Eur Respir J 2015; 45: 1434–45.
98. Awasthi N, Schwarz RE. Profile of nintedanib in the treatment of solid tumors: the evidence to date. Onco Targets Ther 2015; 8: 3691–701.
99. Richeldi L, Costabel U, Selman M et al. Efficacy of a tyrosine kinase inhibitor in idiopathic pulmonary fibrosis. N Engl J Med 2011; 365: 1079–87.
100. Richeldi L, du Bois RM, Raghu G et al. Efficacy and safety of nintedanib in idiopathic pulmonary fibrosis. N Engl J Med 2014; 370: 2071–82.
101. Costabel U, Inoue Y, Richeldi L et al. Efficacy of Nintedanib in Idiopathic Pulmonary Fibrosis across Prespecified Subgroups in INPULSIS. Am J Respir Crit Care Med 2016; 193: 178–85.
102. Bonella F, Stowasser S, Wollin L. Idiopathic pulmonary fibrosis: current treatment options and critical appraisal of nintedanib. Drug Des Devel Ther 2015; 9: 6407–19.
103. Suissa S, Ernst P. The INPULSIS enigma: exacerbations in idiopathic pulmonary fibrosis. Thorax 2015; 70: 508–10.
104. Cottin V, Nunes H, Brillet PY et al. Combined pulmonary fibrosis and emphysema: a distinct underrecognised entity. Eur Respir J 2005; 26: 586–93.
105. Mejia M, Carrillo G, Rojas-Serrano J et al. Idiopathic pulmonary fibrosis and emphysema: decreased survival associated with severe pulmonary arterial hypertension. Chest 2009; 136: 10–5.
106. Ryerson CJ, Hartman T, Elicker BM et al. Clinical features and outcomes in combined pulmonary fibrosis and emphysema in idiopathic pulmonary fibrosis. Chest 2013; 144: 234–40.
107. Kwak N, Park CM, Lee J et al. Lung cancer risk among patients with combined pulmonary fibrosis and emphysema. Respir Med 2014; 108: 524–30.
108. Andersen CU, Mellemkjaer S, Hilberg O, Nielsen-Kudsk JE, Simonsen U, Bendstrup E. Pulmonary hypertension in interstitial lung disease: prevalence, prognosis and 6 min walk test. Respir Med 2012; 106: 875–82.
109. Galie N, Humbert M, Vachiery JL et al. 2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension: the Joint Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS): Endorsed by: Association for European Paediatric and Congenital Cardiology (AEPC), International Society for Heart and Lung Transplantation (ISHLT). Eur Heart J 2016; 37: 67–119.
110. Kimura M, Taniguchi H, Kondoh Y et al. Pulmonary hypertension as a prognostic indicator at the initial evaluation in idiopathic pulmonary fibrosis. Respiration 2013; 85: 456–63.
111. Seeger W, Adir Y, Barbera JA et al. Pulmonary hypertension in chronic lung diseases. J Am Coll Cardiol 2013; 62(25 Suppl): D109–16.
112. Cottin V, Le Pavec J, Prevot G et al. Pulmonary hypertension in patients with combined pulmonary fibrosis and emphysema syndrome. Eur Respir J 2010; 35: 105–11.
113. Vancheri C. Common pathways in idiopathic pulmonary fibrosis and cancer. Eur Respir Rev 2013; 22: 265–72.
114. Turner-Warwick M, Lebowitz M, Burrows B, Johnson A. Cryptogenic fibrosing alveolitis and lung cancer. Thorax 1980; 35: 496–9.
115. Harris JM, Johnston ID, Rudd R, Taylor AJ, Cullinan P. Cryptogenic fibrosing alveolitis and lung cancer: the BTS study. Thorax 2010; 65: 70–6.
116. Usui K, Tanai C, Tanaka Y, Noda H, Ishihara T. The prevalence of pulmonary fibrosis combined with emphysema in patients with lung cancer. Respirology 2011; 16: 326–31.
117. Goto T, Maeshima A, Oyamada Y, Kato R. Idiopathic pulmonary fibrosis as a prognostic factor in non-small cell lung cancer. Int J Clin Oncol 2014; 19: 266–73.
118. Watanabe N, Taniguchi H, Kondoh Y et al. Efficacy of chemotherapy for advanced non-small cell lung cancer with idiopathic pulmonary fibrosis. Respiration 2013; 85: 326–31.
119. Watanabe N, Taniguchi H, Kondoh Y et al. Chemotherapy for extensive-stage small-cell lung cancer with idiopathic pulmonary fibrosis. Int J Clin Oncol 2014; 19: 260–5.
120. Reck M, Kaiser R, Mellemgaard A et al. Docetaxel plus nintedanib versus docetaxel plus placebo in patients with previously treated non-small-cell lung cancer (LUME-Lung 1): a phase 3, double-blind, randomised controlled trial. Lancet Oncol 2014; 15: 143–55.
121. Ryerson CJ, Cottin V, Brown KK, Collard HR. Acute exacerbation of idiopathic pulmonary fibrosis: shifting the paradigm. Eur Respir J 2015; 46: 512–20.
122. Song JW, Hong SB, Lim CM, Koh Y, Kim DS. Acute exacerbation of idiopathic pulmonary fibrosis: incidence, risk factors and outcome. Eur Respir J 2011; 37: 356–63.
123. Martinez FJ, Safrin S, Weycker D et al. The clinical course of patients with idiopathic pulmonary fibrosis. Ann Intern Med 2005; 142: 963–7.
124. Daniels CE, Yi ES, Ryu JH. Autopsy findings in 42 consecutive patients with idiopathic pulmonary fibrosis. Eur Respir J 2008; 32: 170–4.
125. Natsuizaka M, Chiba H, Kuronuma K et al. Epidemiologic survey of Japanese patients with idiopathic pulmonary fibrosis and investigation of ethnic differences. Am J Respir Crit Care Med 2014; 190: 773–9.
126. Enomoto N, Suda T, Uto T et al. Possible therapeutic effect of direct haemoperfusion with a polymyxin B immobilized fibre column (PMX-DHP) on pulmonary oxygenation in acute exacerbations of interstitial pneumonia. Respirology 2008; 13: 452–60.
127. Oishi K, Mimura-Kimura Y, Miyasho T et al. Association between cytokine removal by polymyxin B hemoperfusion and improved pulmonary oxygenation in patients with acute exacerbation of idiopathic pulmonary fibrosis. Cytokine 2013; 61: 84–9.
128. Kono M, Nakamura Y, Enomoto N et al. Usual interstitial pneumonia preceding collagen vascular disease: a retrospective case control study of patients initially diagnosed with idiopathic pulmonary fibrosis. PLoS One 2014; 9: e94775.
129. Nathan SD, Albera C, Bradford WZ et al. Effect of continued treatment with pirfenidone following clinically meaningful declines in forced vital capacity: analysis of data from three phase 3 trials in patients with idiopathic pulmonary fibrosis. Thorax 2016; 71: 429–35.
130. Schmidt SL, Tayob N, Han MK et al. Predicting pulmonary fibrosis disease course from past trends in pulmonary function. Chest 2014; 145: 579–85.
131. Wuyts WA, Antoniou KM, Borensztajn K et al. Combination therapy: the future of management for idiopathic pulmonary fibrosis? Lancet Respir Med 2014; 2: 933–42.
132. Ogura T, Taniguchi H, Azuma A et al. Safety and pharmacokinetics of nintedanib and pirfenidone in idiopathic pulmonary fibrosis. Eur Respir J 2015; 45: 1382–92.
133. Lancaster L, Albera C, Bradford WZ et al. Safety of pirfenidone in patients with idiopathic pulmonary fibrosis: integrated analysis of cumulative data from 5 clinical trials. BMJ Open Respir Res 2016; 3: e000105.
134. Corte T, Bonella F, Crestani B et al. Safety, tolerability and appropriate use of nintedanib in idiopathic pulmonary fibrosis. Respir Res 2015; 16: 116.