References
Key articles
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 Mar 15;183(6):788-824.Full text Abstract
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 Jul 15;192(2):e3-19.Full text Abstract
Raghu G, Remy-Jardin M, Richeldi L, et al; American Thoracic Society, European Respiratory Society, Japanese Respiratory Society, and Latin American Thoracic Society. Idiopathic pulmonary fibrosis (an update) and progressive pulmonary fibrosis in adults: an official ATS/ERS/JRS/ALAT clinical practice guideline. Am J Respir Crit Care Med. 2022 May 1;205(9):e18-e47.Full text Abstract
Collard HR, Ryerson CJ, Corte TJ, et al. Acute exacerbation of idiopathic pulmonary fibrosis. an international working group report. Am J Respir Crit Care Med. 2016 Aug 1;194(3):265-75.Full text Abstract
Borie R, Kannengiesser C, Antoniou K, et al. European Respiratory Society statement on familial pulmonary fibrosis. Eur Respir J. 2023 Mar;61(3):2201383.Full text Abstract
Reference articles
1. Raghu G, Remy-Jardin M, Myers JL, et al; American Thoracic Society, European Respiratory Society, Japanese Respiratory Society, and Latin American Thoracic Society. Diagnosis of idiopathic pulmonary fibrosis. An official ATS/ERS/JRS/ALAT clinical practice guideline. Am J Respir Crit Care Med. 2018 Sep 1;198(5):e44-68.Full text Abstract
2. Liu GY, Budinger GRS, Dematte JE. Advances in the management of idiopathic pulmonary fibrosis and progressive pulmonary fibrosis. BMJ. 2022 Jun 29;377:e066354.Full text Abstract
3. 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 Mar 15;183(6):788-824.Full text Abstract
4. 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 Jul 15;192(2):e3-19.Full text Abstract
5. Raghu G, Remy-Jardin M, Richeldi L, et al; American Thoracic Society, European Respiratory Society, Japanese Respiratory Society, and Latin American Thoracic Society. Idiopathic pulmonary fibrosis (an update) and progressive pulmonary fibrosis in adults: an official ATS/ERS/JRS/ALAT clinical practice guideline. Am J Respir Crit Care Med. 2022 May 1;205(9):e18-e47.Full text Abstract
6. Hutchinson J, Fogarty A, Hubbard R, et al. Global incidence and mortality of idiopathic pulmonary fibrosis: a systematic review. Eur Respir J. 2015 Sep;46(3):795-806.Full text Abstract
7. Caminati A, Madotto F, Cesana G, et al. Epidemiological studies in idiopathic pulmonary fibrosis: pitfalls in methodologies and data interpretation. Eur Respir Rev. 2015 Sep;24(137):436-44.Full text Abstract
8. Olson AL, Gifford AH, Inase N, et al. The epidemiology of idiopathic pulmonary fibrosis and interstitial lung diseases at risk of a progressive-fibrosing phenotype. Eur Respir Rev. 2018 Dec 31;27(150):180077.Full text Abstract
9. Raghu G, Chen SY, Yeh WS, et al. Idiopathic pulmonary fibrosis in US Medicare beneficiaries aged 65 years and older: incidence, prevalence, and survival, 2001-11. Lancet Respir Med. 2014 Jul;2(7):566-72. Abstract
10. Nathan N, Sileo C, Thouvenin G, et al. Pulmonary fibrosis in children. J Clin Med. 2019 Aug 26;8(9):1312.Full text Abstract
11. Navaratnam V, Fleming KM, West J, et al. The rising incidence of idiopathic pulmonary fibrosis in the U.K. Thorax. 2011 Jun;66(6):462-7. Abstract
12. Gupta R, Morgan AD, George PM, et al. Incidence, prevalence and mortality of idiopathic pulmonary fibrosis in England from 2008 to 2018: a cohort study. Thorax. 2024 Jun 14;79(7):624-31. Abstract
13. Harari S, Davì M, Biffi A, et al. Epidemiology of idiopathic pulmonary fibrosis: a population-based study in primary care. Intern Emerg Med. 2020 Apr;15(3):437-45. Abstract
14. Smith-Bindman R, Kwan ML, Marlow EC, et al. Trends in use of medical imaging in US health care systems and in Ontario, Canada, 2000-2016. JAMA. 2019 Sep 3;322(9):843-56.Full text Abstract
15. Collard HR, Ryerson CJ, Corte TJ, et al. Acute exacerbation of idiopathic pulmonary fibrosis. an international working group report. Am J Respir Crit Care Med. 2016 Aug 1;194(3):265-75.Full text Abstract
16. Borie R, Kannengiesser C, Antoniou K, et al. European Respiratory Society statement on familial pulmonary fibrosis. Eur Respir J. 2023 Mar;61(3):2201383.Full text Abstract
17. Marshall RP, Puddicombe A, Cookson WO, et al. Adult familial cryptogenic fibrosing alveolitis in the United Kingdom. Thorax. 2000 Feb;55(2):143-6. Abstract
18. Lee HL, Ryu JH, Wittmer MH, et al. Familial idiopathic pulmonary fibrosis: clinical features and outcome. Chest. 2005 Jun;127(6):2034-41. Abstract
19. Borie R, Kannengiesser C, Sicre de Fontbrune F, et al. Management of suspected monogenic lung fibrosis in a specialised centre. Eur Respir Rev. 2017 Jun 30;26(144):160122.Full text Abstract
20. Tirelli C, Pesenti C, Miozzo M, et al. The genetic and epigenetic footprint in idiopathic pulmonary fibrosis and familial pulmonary fibrosis: a state-of-the-art review. Diagnostics (Basel). 2022 Dec 9;12(12):3107.Full text Abstract
21. Meltzer EB, Noble PW. Idiopathic pulmonary fibrosis. Orphanet J Rare Dis. 2008 Mar 26;3:8.Full text Abstract
22. Streiter RM. Pathogenesis and natural history of usual interstitial pneumonia: the whole story or the last chapter of a long novel. Chest. 2005 Nov;128(5 suppl 1):526-32S. Abstract
23. Collopy LC, Walne AJ, Cardoso S, et al. Triallelic and epigenetic-like inheritance in human disorders of telomerase. Blood. 2015 Jul 9;126(2):176-84.Full text Abstract
24. Lawson WE, Loyd JE. The genetic approach in pulmonary fibrosis: can it provide clues to this complex disease? Proc Am Thorac Soc. 2006 Jun;3(4):345-9.Full text Abstract
25. Siebold MA, Wise AL, Speer MC, et al. A common MUC5B promotor polymorphism and pulmonary fibrosis. N Engl J Med. 2011 Apr 21;364(16):1503-12. Abstract
26. Bellou V, Belbasis L, Evangelou E. Tobacco smoking and risk for pulmonary fibrosis: a prospective cohort study from the UK biobank. Chest. 2021 Sep;160(3):983-93. Abstract
27. Zhu J, Zhou D, Yu M, et al. Appraising the causal role of smoking in idiopathic pulmonary fibrosis: a Mendelian randomization study. Thorax. 2024 Jan 18;79(2):179-81.Full text Abstract
28. Baumgartner KB, Samet JM, Coultas DB, et al. Occupational and environmental risk factors for idiopathic pulmonary fibrosis: a multicenter case-control study. Am J Epidemiol. 2000 Aug 15;152(4):307-15.Full text Abstract
29. Gandhi SA, Min B, Fazio JC, et al. The impact of occupational exposures on the risk of idiopathic pulmonary fibrosis: a systematic review and meta-analysis. Ann Am Thorac Soc. 2024 Mar;21(3):486-98. Abstract
30. Bédard Méthot D, Leblanc É, Lacasse Y. Meta-analysis of gastroesophageal reflux disease and idiopathic pulmonary fibrosis. Chest. 2019 Jan;155(1):33-43. Abstract
31. Moore BB, Moore TA. Viruses in idiopathic pulmonary fibrosis. Etiology and exacerbation. Ann Am Thorac Soc. 2015 Nov;12 Suppl 2(suppl 2):S186-92.Full text Abstract
32. Tang YW, Johnson JE, Browning PJ, et al. Herpesvirus DNA is consistently detected in lungs of patients with idiopathic pulmonary fibrosis. J Clin Microbiol. 2003 Jun;41(6):2633-40.Full text Abstract
33. Sheng G, Chen P, Wei Y, et al. Viral infection increases the risk of idiopathic pulmonary fibrosis: a meta-analysis. Chest. 2020 May;157(5):1175-87.Full text Abstract
34. Moghoofei M, Mostafaei S, Kondori N, et al. Bacterial and viral coinfection in idiopathic pulmonary fibrosis patients: the prevalence and possible role in disease progression. BMC Pulm Med. 2022 Feb 11;22(1):60.Full text Abstract
35. Mostafaei S, Sayad B, Azar MEF, et al. The role of viral and bacterial infections in the pathogenesis of IPF: a systematic review and meta-analysis. Respir Res. 2021 Feb 12;22(1):53.Full text Abstract
36. Kopf S, Groener JB, Kender Z, et al. Breathlessness and restrictive lung disease: an important diabetes-related feature in patients with type 2 diabetes. Respiration. 2018;96(1):29-40.Full text Abstract
37. Rajasurya V, Gunasekaran K, Surani S. Interstitial lung disease and diabetes. World J Diabetes. 2020 Aug 15;11(8):351-7.Full text Abstract
38. Bai L, Zhang L, Pan T, et al. Idiopathic pulmonary fibrosis and diabetes mellitus: a meta-analysis and systematic review. Respir Res. 2021 Jun 8;22(1):175.Full text Abstract
39. Gribbin J, Hubbard R, Smith C. Role of diabetes mellitus and gastro-oesophageal reflux in the aetiology of idiopathic pulmonary fibrosis. Respir Med. 2009 Jun;103(6):927-31. Abstract
40. Navaratnam V, Davis TME, Hubbard R, et al. Incidence and predictors of idiopathic pulmonary fibrosis complicating type 2 diabetes: the Fremantle Diabetes Study Phase I. Intern Med J. 2021 Feb;51(2):276-9. Abstract
41. Kang Q, Ren J, Cong J, et al. Diabetes mellitus and idiopathic pulmonary fibrosis: a Mendelian randomization study. BMC Pulm Med. 2024 Mar 20;24(1):142.Full text Abstract
42. Hunninghake GW, Zimmerman MB, Schwartz DA, et al. Utility of a lung biopsy for the diagnosis of idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 2001 Jul 15;164(2):193-6.Full text Abstract
43. Travis WD, Costabel U, Hansell DM, et al; ATS/ERS Committee on Idiopathic Interstitial Pneumonias. 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 Sep 15;188(6):733-48.Full text Abstract
44. Hobbs S, Chung JH, Leb J, et al. Practical imaging interpretation in patients suspected of having idiopathic pulmonary fibrosis: official recommendations from the radiology working group of the pulmonary fibrosis foundation. Radiol Cardiothorac Imaging. 2021 Feb;3(1):e200279.Full text Abstract
45. Aveyard P, Gao M, Lindson N, et al. Association between pre-existing respiratory disease and its treatment, and severe COVID-19: a population cohort study. Lancet Respir Med. 2021 Aug;9(8):909-23.Full text Abstract
46. Wong AW, Shapera S, Morisset J, et al. Optimizing care for patients with interstitial lung disease during the COVID-19 pandemic. Can J Respir Crit Care Sleep Med. 2020;4:226-8.Full text
47. Lynch DA, Sverzellati N, Travis WD, et al. Diagnostic criteria for idiopathic pulmonary fibrosis: a Fleischner Society White Paper. Lancet Respir Med. 2017 Nov 15;6(2):138-53.Full text Abstract
48. Meyer KC, Raghu G, Baughman RP, et al. An official American Thoracic Society clinical practice guideline: the clinical utility of bronchoalveolar lavage cellular analysis in interstitial lung disease. Am J Respir Crit Care Med. 2012 May 1;185(9):1004-14. Abstract
49. Korevaar DA, Colella S, Fally M, et al. European Respiratory Society guidelines on transbronchial lung cryobiopsy in the diagnosis of interstitial lung diseases. Eur Respir J. 2022 Nov;60(5):2200425.Full text Abstract
50. Kim SY, Diggans J, Pankratz D, et al. Classification of usual interstitial pneumonia in patients with interstitial lung disease: assessment of a machine learning approach using high-dimensional transcriptional data. Lancet Respir Med. 2015 Jun;3(6):473-82. Abstract
51. Lasky JA, Case A, Unterman A, et al. The impact of the Envisia genomic classifier in the diagnosis and management of patients with idiopathic pulmonary fibrosis. Ann Am Thorac Soc. 2022 Jun;19(6):916-24.Full text Abstract
52. Katzenstein AL, Myers JL. Idiopathic pulmonary fibrosis: clinical relevance of pathologic classification. Am J Respir Crit Care Med. 1998 Apr;157(4 Pt 1):1301-15.Full text Abstract
53. Bando M, Ohno S, Hosono T, et al. Risk of acute exacerbation after video-assisted thoracoscopic lung biopsy for interstitial lung disease. J Bronchology Interv Pulmonol. 2009 Oct;16(4):229-35. Abstract
54. Ghatol A, Ruhl AP, Danoff SK. Exacerbations in idiopathic pulmonary fibrosis triggered by pulmonary and nonpulmonary surgery: a case series and comprehensive review of the literature. Lung. 2012 Aug;190(4):373-80. Abstract
55. American Thoracic Society; European Respiratory Society. Idiopathic pulmonary fibrosis: diagnosis and treatment. International consensus statement. Am J Respir Crit Care Med. 2000 Feb;161(2 Pt 1):646-64.Full text Abstract
56. Berbescu EA, Katzenstein AL, Snow JL, et al. Transbronchial biopsy in usual interstitial pneumonia. Chest. 2006 May;129(5):1126-31.Full text Abstract
57. Cordier JF. Cryptogenic organising pneumonia. Eur Respir J. 2006 Aug;28(2):422-46.Full text Abstract
58. American Thoracic Society (ATS), European Respiratory Society (ERS). American Thoracic Society/European Respiratory Society international multidisciplinary consensus classification of the idiopathic interstitial pneumonias. Am J Respir Crit Care Med. 2002 Jan 15;165(2):277-304.Full text Abstract
59. Chan-Yeung M, Müller NL. Cryptogenic fibrosing alveolitis. Lancet. 1997 Aug 30;350(9078):651-6. Abstract
60. Cottin V, Selman M, Inoue Y, et al. Syndrome of combined pulmonary fibrosis and emphysema: an official ATS/ERS/JRS/ALAT research statement. Am J Respir Crit Care Med. 2022 Aug 15;206(4):e7-41.Full text Abstract
61. National Institute for Health and Care Excellence. Idiopathic pulmonary fibrosis in adults: diagnosis and management. May 2017 [internet publication].Full text
62. Martinez FJ, Safrin S, Weycker D, et al. The clinical course of patients with idiopathic pulmonary fibrosis. Ann Intern Med. 2005 Jun 21;142(12 Pt 1):963-7. Abstract
63. Agarwal R, Jindal SK. Acute exacerbation of idiopathic pulmonary fibrosis: a systematic review. Eur J Intern Med. 2008 Jun;19(4):227-35. Abstract
64. Kreuter M, Polke M, Walsh SLF, et al. Acute exacerbation of idiopathic pulmonary fibrosis: international survey and call for harmonisation. Eur Respir J. 2020 Apr;55(4):1901760.Full text Abstract
65. Naccache JM, Jouneau S, Didier M, et al. Cyclophosphamide added to glucocorticoids in acute exacerbation of idiopathic pulmonary fibrosis (EXAFIP): a randomised, double-blind, placebo-controlled, phase 3 trial. Lancet Respir Med. 2022 Jan;10(1):26-34. Abstract
66. Petnak T, Lertjitbanjong P, Thongprayoon C, et al. Impact of antifibrotic therapy on mortality and acute exacerbation in idiopathic pulmonary fibrosis: a systematic review and meta-analysis. Chest. 2021 Nov;160(5):1751-63. Abstract
67. Zhao C, Yin Y, Zhu C, et al. Drug therapies for treatment of idiopathic pulmonary fibrosis: a systematic review, Bayesian network meta-analysis, and cost-effectiveness analysis. EClinicalMedicine. 2023 Jul;61:102071.Full text Abstract
68. Flaherty KR, Fell CD, Huggins JT, et al. Safety of nintedanib added to pirfenidone treatment for idiopathic pulmonary fibrosis. Eur Respir J. 2018 Aug 2;52(2):1800230. [Erratum in: Eur Respir J. 2018 Oct 4;52(4):1850230.]Full text Abstract
69. Rahaghi F, Belperio JA, Fitzgerald J, et al. Delphi consensus recommendations on management of dosing, adverse events, and comorbidities in the treatment of idiopathic pulmonary fibrosis with nintedanib. Clin Med Insights Circ Respir Pulm Med. 2021;15:11795484211006050.Full text Abstract
70. Marcos Ribes B, Sancho-Chust JN, Talens A, et al. Effectiveness and safety of pirfenidone for idiopathic pulmonary fibrosis. Eur J Hosp Pharm. 2020 Nov;27(6):350-4. Abstract
71. Noble PW, Albera C, Bradford WZ, et al; CAPACITY Study Group. Pirfenidone in patients with idiopathic pulmonary fibrosis (CAPACITY): two randomised trials. Lancet. 2011 May 21;377(9779):1760-9. Abstract
72. Spagnolo P, Del Giovane C, Luppi F, et al. Non-steroid agents for idiopathic pulmonary fibrosis. Cochrane Database Syst Rev. 2010 Sep 8;(9):CD003134. Abstract
73. King TE Jr, Bradford WZ, Castro-Bernardini S, et al; ASCEND Study Group. A phase 3 trial of pirfenidone in patients with idiopathic pulmonary fibrosis. N Engl J Med. 2014 May 29;370(22):2083-92. Abstract
74. Richeldi L, du Bois RM, Raghu G, et al; INPULSIS Trial Investigators. Efficacy and safety of nintedanib in idiopathic pulmonary fibrosis. N Engl J Med. 2014 May 29;370(22):2071-82.Full text Abstract
75. 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 Jan 15;193(2):178-85.Full text Abstract
76. Crestani B, Huggins JT, Kaye M, et al. Long-term safety and tolerability of nintedanib in patients with idiopathic pulmonary fibrosis: results from the open-label extension study, INPULSIS-ON. Lancet Respir Med. 2018 Sep 14;7(1):60-8. Abstract
77. Pitre T, Mah J, Helmeczi W, et al. Medical treatments for idiopathic pulmonary fibrosis: a systematic review and network meta-analysis. Thorax. 2022 Dec;77(12):1243-50. Abstract
78. Canestaro WJ, Forrester SH, Raghu G, et al. Drug treatment of idiopathic pulmonary fibrosis: systematic review and network meta-analysis. Chest. 2016 Mar;149(3):756-66. Abstract
79. Richeldi L, Davies HR, Ferrara G, et al. Corticosteroids for idiopathic pulmonary fibrosis. Cochrane Database Syst Rev. 2003 Jul 21;(3):CD002880. Abstract
80. 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 Jul 18;374(9685):222-8. Abstract
81. 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 Mar 15;181(6):604-10. Abstract
82. King TE, Brown KK, Raghu G. BUILD-3: a randomized, controlled trial of bosentan in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 2011 Jul 1;184(1):92-9. Abstract
83. Raghu G, Behr J, Brown KK, et al; ARTEMIS-IPF Investigators. Treatment of idiopathic pulmonary fibrosis with ambrisentan: a parallel, randomized trial. Ann Intern Med. 2013 May 7;158(9):641-9. Abstract
84. Raghu G, Million-Rousseau R, Morganti A, et al; MUSIC Study Group. Macitentan for the treatment of idiopathic pulmonary fibrosis: the randomised controlled MUSIC trial. Eur Respir J. 2013 Dec;42(6):1622-32. Abstract
85. Raghu G, Anstrom KJ, King TE Jr, et al; Idiopathic Pulmonary Fibrosis Clinical Research Network. Prednisone, azathioprine, and N-acetylcysteine for pulmonary fibrosis. N Engl J Med. 2012 May 24;366(21):1968-77.Full text Abstract
86. Martinez FJ, de Andrade JA, Anstrom KJ, et al; Idiopathic Pulmonary Fibrosis Clinical Research Network. Randomized trial of acetylcysteine in idiopathic pulmonary fibrosis. N Engl J Med. 2014 May 29;370(22):2093-101. Abstract
87. Fujimoto H, Gabazza EC, Hataji O, et al. Thrombin-activatable fibrinolysis inhibitor and protein C inhibitor in interstitial lung disease. Am J Respir Crit Care Med. 2003 Jun 15;167(12):1687-94.Full text Abstract
88. Kubo H, Nakayama K, Yanai M, et al. Anticoagulant therapy for idiopathic pulmonary fibrosis. Chest. 2005 Sep;128(3):1475-82. Abstract
89. 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 Jul 1;186(1):88-95. Abstract
90. Distefano G, Fanzone L, Palermo M, et al. HRCT patterns of drug-induced interstitial lung diseases: a review. Diagnostics (Basel). 2020 Apr 22;10(4):244.Full text Abstract
91. Spruit MA, Singh SJ, Garvey C, et al. An official American Thoracic Society/European Respiratory Society statement: key concepts and advances in pulmonary rehabilitation. Am J Respir Crit Care Med. 2013 Oct 15;188(8):e13-64.Full text Abstract
92. Rochester CL, Alison JA, Carlin B, et al. Pulmonary rehabilitation for adults with chronic respiratory disease: an official American Thoracic Society Clinical Practice Guideline. Am J Respir Crit Care Med. 2023 Aug 15;208(4):e7-e26.Full text Abstract
93. Dowman L, Hill CJ, May A, et al. Pulmonary rehabilitation for interstitial lung disease. Cochrane Database Syst Rev. 2021 Feb 1;2(2):CD006322.Full text Abstract
94. Jacobs SS, Krishnan JA, Lederer DJ, et al. Home oxygen therapy for adults with chronic lung disease. An official American Thoracic Society clinical practice guideline. Am J Respir Crit Care Med. 2020 Nov 15;202(10):e121-41.Full text Abstract
95. Sharp C, Adamali H, Millar AB. Ambulatory and short-burst oxygen for interstitial lung disease. Cochrane Database Syst Rev. 2016 Jul 6;(7):CD011716.Full text Abstract
96. Khor YH, Bissell B, Ghazipura M, et al. Antacid medication and antireflux surgery in patients with idiopathic pulmonary fibrosis: a systematic review and meta-analysis. Ann Am Thorac Soc. 2022 May;19(5):833-44.Full text Abstract
97. Janssen DJA, Bajwah S, Boon MH, et al. European Respiratory Society clinical practice guideline: palliative care for people with COPD or interstitial lung disease. Eur Respir J. 2023 Aug;62(2):2202014.Full text Abstract
98. Kim JW, Atkins C, Wilson AM. Barriers to specialist palliative care in interstitial lung disease: a systematic review. BMJ Support Palliat Care. 2019 Jun;9(2):130-8. Abstract
99. Barnes H, McDonald J, Smallwood N, et al. Opioids for the palliation of refractory breathlessness in adults with advanced disease and terminal illness. Cochrane Database Syst Rev. 2016 Mar 31;(3):CD011008.Full text Abstract
100. Wu Z, Spencer LG, Banya W, et al. Morphine for treatment of cough in idiopathic pulmonary fibrosis (PACIFY COUGH): a prospective, multicentre, randomised, double-blind, placebo-controlled, two-way crossover trial. Lancet Respir Med. 2024 Apr;12(4):273-80.Full text Abstract
101. Bajwah S, Ross JR, Wells AU, et al. Palliative care for patients with advanced fibrotic lung disease: a randomised controlled phase II and feasibility trial of a community case conference intervention. Thorax. 2015 Sep;70(9):830-9. Abstract
102. Leard LE, Holm AM, Valapour M, et al. Consensus document for the selection of lung transplant candidates: an update from the International Society for Heart and Lung Transplantation. J Heart Lung Transplant. 2021 Nov;40(11):1349-79.Full text Abstract
103. Richeldi L, Azuma A, Cottin V, et al. Trial of a preferential phosphodiesterase 4B inhibitor for idiopathic pulmonary fibrosis. N Engl J Med. 2022 Jun 9;386(23):2178-87.Full text Abstract
104. Richeldi L, Azuma A, Cottin V, et al. Design of a phase III, double-blind, randomised, placebo-controlled trial of BI 1015550 in patients with idiopathic pulmonary fibrosis (FIBRONEER-IPF). BMJ Open Respir Res. 2023 Aug;10(1):e001563.Full text Abstract
105. Maher TM, Assassi S, Azuma A, et al. Design of a phase III, double-blind, randomised, placebo-controlled trial of BI 1015550 in patients with progressive pulmonary fibrosis (FIBRONEER-ILD). BMJ Open Respir Res. 2023 Sep;10(1):e001580. Abstract
106. ClinicalTrials.gov. Study to evaluate axatilimab in participants with idiopathic pulmonary fibrosis (IPF). Jun 2024 [interent publication].Full text
107. Willette RN, Mangrolia P, Pondell SM, et al. Modulation of oxidative phosphorylation with IM156 attenuates mitochondrial metabolic reprogramming and inhibits pulmonary fibrosis. J Pharmacol Exp Ther. 2021 Nov;379(3):290-300.Full text Abstract
108. Salgado-Polo F, Borza R, Matsoukas MT, et al. Autotaxin facilitates selective LPA receptor signaling. Cell Chem Biol. 2023 Jan 19;30(1):69-84.e14.Full text Abstract
109. ClinicalTrials.gov. To evaluate the efficacy, safety, and tolerability of BBT-877 in patients with IPF. Jul 2024 [interent publication].Full text
110. ClinicalTrials.gov. RESPIRARE - efficacy and safety of cudetaxestat in patients with idiopathic pulmonary fibrosis (IPF). May 2022 [interent publication].Full text Abstract
111. Decaris ML, Schaub JR, Chen C, et al. Dual inhibition of α(v)β(6) and α(v)β(1) reduces fibrogenesis in lung tissue explants from patients with IPF. Respir Res. 2021 Oct 19;22(1):265.Full text Abstract
112. ClinicalTrials.gov. Evaluation of efficacy and safety of PLN-74809 in patients with idiopathic pulmonary fibrosi. Jun 2024 [interent publication].Full text
113. Kim DS, Collard HR, King TE Jr. Classification and natural history of the idiopathic interstitial pneumonias. Proc Am Thorac Soc. 2006 Jun;3(4):285-92.Full text Abstract
114. Flaherty KR, Thwaite EL, Kazerooni EA, et al. Radiological versus histological diagnosis in UIP and NSIP: survival implications. Thorax. 2003 Feb;58(2):143-8.Full text Abstract
115. Collard HR, King TE Jr, Bartelson BB, et al. Changes in clinical and physiologic variables predict survival in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 2003 Sep 1;168(5):538-42.Full text Abstract
116. Latsi PI, du Bois RM, Nicholson AG, et al. Fibrotic idiopathic interstitial pneumonia. The prognostic value of longitudinal functional trends. Am J Respir Crit Care Med. 2003 Sep 1;168(5):531-7.Full text Abstract
117. Flaherty KR, Mumford JA, Murray S, et al. Prognostic implications of physiologic and radiographic changes in idiopathic interstitial pneumonia. Am J Respir Crit Care Med. 2003 Sep 1;168(5):543-8.Full text Abstract
118. King TE Jr, Tooze JA, Schwarz MI, et al. Predicting survival in idiopathic pulmonary fibrosis: scoring system and survival model. Am J Respir Crit Care Med. 2001 Oct 1;164(7):1171-81.Full text Abstract
119. Antoniou KM, Hansell DM, Rubens MB, et al. Idiopathic pulmonary fibrosis: outcome in relation to smoking status. Am J Resp Crit Care Med. 2008 Jan 15;177(2):190-4. Abstract
120. Song JW, Hong SB, Lim CM, et al. Acute exacerbation of idiopathic pulmonary fibrosis: incidence, risk factors, and outcome. Eur Respir J. 2011 Feb;37(2):356-63. Abstract
121. 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 Apr;35(4):830-6. Abstract
122. Swigris JJ, Swick J, Wamboldt FS, et al. Heart rate recovery after 6-min walk test predicts survival in patients with idiopathic pulmonary fibrosis. Chest. 2009 Sep;136(3):841-8.Full text Abstract
123. Lama VN, Flaherty KR, Toews GB, et al. Prognostic value of desaturation during a 6-minute walk test in idiopathic interstitial pneumonia. Am J Respir Crit Care Med. 2003 Nov 1;168(9):1084-90.Full text Abstract
124. Scholand MB, Coon H, Wolff R, et al. Use of a genealogical database demonstrates heritability of pulmonary fibrosis. Lung. 2013 Oct;191(5):475-81.Full text Abstract
125. Atkins CP, Loke YK, Wilson AM. Outcomes in idiopathic pulmonary fibrosis: a meta-analysis from placebo controlled trials. Respir Med. 2014 Feb;108(2):376-87.Full text Abstract
126. Peljto AL, Zhang Y, Fingerlin TE, et al. Association between the MUC5B promoter polymorphism and survival in patients with idiopathic pulmonary fibrosis. JAMA. 2013 Jun 5;309(21):2232-9. Abstract
127. Song JW, Do KH, Jang SJ, et al. Blood biomarkers MMP-7 and SP-A: predictors of outcome in idiopathic pulmonary fibrosis. Chest. 2013 May;143(5):1422-9. Abstract
128. Nathan SD, Reffett T, Brown AW, et al. The red cell distribution width as a prognostic indicator in idiopathic pulmonary fibrosis. Chest. 2013 Jun;143(6):1692-8. Abstract
129. Kahloon RA, Xue J, Bhargava A, et al. Patients with idiopathic pulmonary fibrosis with antibodies to heat shock protein 70 have poor prognoses. Am J Respir Crit Care Med. 2013 Apr 1;187(7):768-75. Abstract
130. Ley B, Ryerson CJ, Vittinghoff E, et al. A multidimensional index and staging system for idiopathic pulmonary fibrosis. Ann Intern Med. 2012 May 15;156(10):684-91. Abstract
131. Ryerson CJ, Vittinghoff E, Ley B, et al. Predicting survival across chronic interstitial lung disease: the ILD-GAP model. Chest. 2014 Apr;145(4):723-8. Abstract
132. Zhang X, Ren Y, Xie B, et al. External validation of the GAP model in Chinese patients with idiopathic pulmonary fibrosis. Clin Respir J. 2023 Sep;17(9):831-40.Full text Abstract
133. Lacedonia D, De Pace CC, Rea G, et al. Machine learning and BMI improve the prognostic value of GAP index in treated IPF patients. Bioengineering (Basel). 2023 Feb 14;10(2):251.Full text Abstract
134. Bocchino M, Bruzzese D, Scioscia G, et al. Disease stage-related survival in idiopathic pulmonary fibrosis patients treated with nintedanib and pirfenidone: An exploratory study. Respir Med Res. 2023 Nov;84:101013.Full text Abstract
135. Humbert M, Kovacs G, Hoeper MM, et al. 2022 ESC/ERS guidelines for the diagnosis and treatment of pulmonary hypertension. Eur Heart J. 2022 Oct 11;43(38):3618-731.Full text Abstract
136. Nathan SD, Barbera JA, Gaine SP, et al. Pulmonary hypertension in chronic lung disease and hypoxia. Eur Respir J. 2019 Jan;53(1):1801914.Full text Abstract
137. Nadrous HR, Pellikka PA, Krowka MJ, et al. Pulmonary hypertension in patients with idiopathic pulmonary fibrosis. Chest. 2005 Oct;128(4):2393-9. Abstract
138. Lettieri CJ, Nathan SD, Barnett SD, et al. Prevalence and outcomes of pulmonary arterial hypertension in advanced idiopathic pulmonary fibrosis. Chest. 2006 Mar;129(3):746-52. Abstract
139. Ryu JH, Krowka MJ, Swanson KL, et al. Pulmonary hypertension in patients with interstitial lung diseases. Mayo Clin Proc. 2007 Mar;82(3):342-50. Abstract
140. Kolb M, Raghu G, Wells AU, et al. Nintedanib plus sildenafil in patients with idiopathic pulmonary fibrosis. N Engl J Med. 2018 Sep 15;379(18):1722-31.Full text Abstract
141. Behr J, Nathan SD, Wuyts WA, et al. Efficacy and safety of sildenafil added to pirfenidone in patients with advanced idiopathic pulmonary fibrosis and risk of pulmonary hypertension: a double-blind, randomised, placebo-controlled, phase 2b trial. Lancet Respir Med. 2021 Jan;9(1):85-95. Abstract
142. Bongiovanni G, Tonutti A, Stainer A, et al. Vasoactive drugs for the treatment of pulmonary hypertension associated with interstitial lung diseases: a systematic review. BMJ Open Respir Res. 2024 Mar 13;11(1):e002161.Full text Abstract
143. El-Kersh K, Jalil BA. Pulmonary hypertension inhaled therapies: an updated review. Am J Med Sci. 2023 Jul;366(1):3-15. Abstract
144. Nathan SD, Behr J, Cottin V, et al. Study design and rationale for the TETON phase 3, randomised, controlled clinical trials of inhaled treprostinil in the treatment of idiopathic pulmonary fibrosis. BMJ Open Respir Res. 2022 Jul;9(1):e001310.Full text Abstract
145. Harris JM, Johnston ID, Rudd R, et al. Cryptogenic fibrosing alveolitis and lung cancer: the BTS study. Thorax. 2010 Jan;65(1):70-6. Abstract
146. Daniels CE, Jett JR. Does interstitial lung disease predispose to lung cancer? Curr Opin Pulm Med. 2005 Sep;11(5):431-7. Abstract
147. Naoi H, Suzuki Y, Mori K, et al. Impact of antifibrotic therapy on lung cancer development in idiopathic pulmonary fibrosis. Thorax. 2022 Jul;77(7):727-30. Abstract
148. Molyneaux PL, Cox MJ, Willis-Owen SA, et al. The role of bacteria in the pathogenesis and progression of idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 2014 Oct 15;190(8):906-13.Full text Abstract
149. Panos RJ, Mortenson R, Niccoli SA, et al. Clinical deterioration in patients with idiopathic pulmonary fibrosis. Causes and assessment. Am J Med. 1990 Apr;88(4):396-404. Abstract
150. Hubbard RB, Smith C, Le Jeune I, et al. The association between idiopathic pulmonary fibrosis and vascular disease: a population-based study. Am J Respir Crit Care Med. 2008 Dec 15;178(12):1257-61. Abstract
151. Nathan SD, Basavaraj A, Reichner C, et al. Prevalence and impact of coronary artery disease in idiopathic pulmonary fibrosis. Respir Med. 2010 Jul;104(7):1035-41. Abstract
152. Flaherty KR, Andrei AC, Murray S, et al. Idiopathic pulmonary fibrosis: prognostic value of changes in physiology and six-minute-walk test. Am J Respir Crit Care Med. 2006 Oct 1;174(7):803-9.Full text Abstract
153. Eaton T, Young P, Milne D, et al. Six-minute walk, maximal exercise tests: reproducibility in fibrotic interstitial pneumonia. Am J Respir Crit Care Med. 2005 May 15;171(10):1150-7.Full text Abstract
154. Hanson D, Winterbauer RH, Kirtland SH, et al. Changes in pulmonary function test results after 1 year of therapy as predictors of survival in patients with idiopathic pulmonary fibrosis. Chest. 1995 Aug;108(2):305-10. Abstract
155. 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 Dec 15;184(12):1382-9.Full text Abstract
156. Fardet L, Kassar A, Cabane J, et al. Corticosteroid-induced adverse events in adults: frequency, screening and prevention. Drug Saf. 2007;30(10):861-81. Abstract
157. Vainshelboim B, Oliveira J, Izhakian S, et al. Lifestyle behaviors and clinical outcomes in idiopathic pulmonary fibrosis. Respiration. 2018;95(1):27-34.Full text Abstract
158. British Thoracic Society. BTS clinical statement on air travel for passengers with respiratory disease. Mar 2022 [internet publication].Full text
159. UK Health Security Agency. Complete routine immunisation schedule: immunisation information for health professionals and immunisation practitioners. Jul 2024 [internet publication].Full text
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