Diagnosis and management of idiopathic pulmonary fibrosis: Thoracic Society of Australia and New Zealand and Lung Foundation Australia position statements summary

Jo*, Helen E; Prasad*, Jyotika D; Troy, Lauren K; Mahar, Annabelle; Bleasel, Jane; Ellis, Samantha J; Chambers, Daniel C; Holland, Anne E; Lake, Fiona R; Keir, Gregory; Goh, Nicole S; Wilsher, Margaret; de Boer, Sally; Moodley, Yuben; Grainge, Christopher; Whitford, Helen M; Chapman, Sally A; Reynolds, Paul N; Beatson**, David; Jones, Leonie J; Hopkins, Peter; Allan, Heather M; Glaspole***, Ian; Corte***, Tamera J

doi:10.5694/mja17.00799

Topics

Abstract

Introduction: Idiopathic pulmonary fibrosis (IPF) is a fibrosing interstitial lung disease associated with debilitating symptoms of dyspnoea and cough, resulting in respiratory failure, impaired quality of life and ultimately death. Diagnosing IPF can be challenging, as it often shares many features with other interstitial lung diseases. In this article, we summarise recent joint position statements on the diagnosis and management of IPF from the Thoracic Society of Australia and New Zealand and Lung Foundation Australia, specifically tailored for physicians across Australia and New Zealand.

Main suggestions:

A comprehensive multidisciplinary team meeting is suggested to establish a prompt and precise IPF diagnosis.
Antifibrotic therapies should be considered to slow disease progression. However, enthusiasm should be tempered by the lack of evidence in many IPF subgroups, particularly the broader disease severity spectrum.
Non-pharmacological interventions including pulmonary rehabilitation, supplemental oxygen, appropriate treatment of comorbidities and disease-related symptoms remain crucial to optimal management.
Despite recent advances, IPF remains a fatal disease and suitable patients should be referred for lung transplantation assessment.

1 Royal Prince Alfred Hospital, Sydney, NSW
2 Alfred Hospital, Melbourne, VIC
3 Royal Melbourne Hospital, Melbourne, VIC
4 University of Sydney, Sydney, NSW
5 University of Queensland, Brisbane, QLD
6 Prince Charles Hospital, Brisbane, QLD
7 La Trobe University, Melbourne, VIC
8 University of Western Australia, Perth, WA
9 Princess Alexandra Hospital, Brisbane, QLD
10 Austin Health, Melbourne, VIC
11 Auckland District Health Board, Auckland, NZ
12 University of Auckland, Auckland, NZ
13 Auckland City Hospital, Auckland, NZ
14 John Hunter Hospital, Newcastle, NSW
15 Royal Adelaide Hospital, Adelaide, SA
16 Auckland, NZ
17 Queensland Lung Transplant Service, Prince Charles Hospital, Brisbane, QLD
18 Lung Foundation Australia, Brisbane, QLD
19 Monash University, Melbourne, VIC

Correspondence: helen.jo@sydney.edu.au

* Joint first authors

** Deceased

*** Joint senior authors

Acknowledgements:

David Beatson was a well known New Zealand journalist, broadcaster and editor. Among his many roles, he served as Prime Minister’s chief press officer, and chair of NZ on Air. A thoughtful, erudite and engaging patient, David died from IPF on 21 September 2017. We acknowledge his important contribution as patient author on this article.

Competing interests:

For competing interest declarations, see online .

1. Ley B, Collard HR, King TE Jr. Clinical course and prediction of survival in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 2011; 183: 431-440.
2. Raghu G, Amatto VC, Behr J, Stowasser S. Comorbidities in idiopathic pulmonary fibrosis patients: a systematic literature review. Eur Respir J 2015; 46: 1113-1130.
3. Kim HJ, Perlman D, Tomic R. Natural history of idiopathic pulmonary fibrosis. Respir Med 2015; 109: 661-670.
4. Aggarwal R, McBurney C, Schneider F, et al. Myositis-associated usual interstitial pneumonia has a better survival than idiopathic pulmonary fibrosis. Rheumatology (Oxford) 2017; 56: 384-389.
5. Miyazaki Y, Tateishi T, Akashi T, et al. Clinical predictors and histologic appearance of acute exacerbations in chronic hypersensitivity pneumonitis. Chest 2008; 134: 1265-1270.
6. Lake F, Proudman S. Rheumatoid arthritis and lung disease: from mechanisms to a practical approach. Semin Respir Crit Care Med 2014; 35: 222-238.
7. Raghu G. An Official ATS/ERS/JRS/ALAT Statement: idiopathic pulmonary fibrosis: evidence-based guidelines for diagnosis and management. Am J Crit Care Med 2011; 183: 788-824.
8. 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: 2083-2092.
9. Richeldi L, du Bois RM, Raghu G, et al. Efficacy and safety of nintedanib in idiopathic pulmonary fibrosis. N Engl J Med 2014; 370: 2071-2082.
10. Prasad JD, Mahar A, Bleasel J, et al. The interstitial lung disease multidisciplinary meeting: a position statement from the Thoracic Society of Australia and New Zealand and the Lung Foundation Australia. Respirology 2017; 22: 1459-1472.
11. Jo HE, Troy LK, Keir G, et al. Treatment of idiopathic pulmonary fibrosis in Australia and New Zealand: a position statement from the Thoracic Society of Australia and New Zealand and the Lung Foundation Australia. Respirology 2017; 22: 1436-1458.
12. Sgalla G, Biffi A, Richeldi L. Idiopathic pulmonary fibrosis: Diagnosis, epidemiology and natural history. Respirology 2016; 21: 427-437.
13. Lee AS. The burden of idiopathic pulmonary fibrosis: an unmet public health need. Respir Med 2014; 108: 955-967.
14. Kim DS, Collard HR, King TE Jr. Classification and natural history of the idiopathic interstitial pneumonias. Proc Am Thorac Soc 2006; 3: 285-292.
15. Sarkar M, Mahesh DM, Madabhavi I. Digital clubbing. Lung India 2012; 29: 354-362.
16. Grenier P, Valeyre D, Cluzel P, et al. Chronic diffuse interstitial lung disease: diagnostic value of chest radiography and high-resolution CT. Radiology 1991; 179: 123-132.
17. Swensen SJ, Aughenbaugh GL, Myers JL. Diffuse lung disease: diagnostic accuracy of CT in patients undergoing surgical biopsy of the lung. Radiology 1997; 205: 229-234.
18. Flaherty KR, King TE, Jr, Raghu G, et al. Idiopathic interstitial pneumonia: what is the effect of a multidisciplinary approach to diagnosis? Am J Respir Crit Care Med 2004; 170: 904-910.
19. Flaherty KR, Andrei AC, King TE Jr, et al. Idiopathic interstitial pneumonia: do community and academic physicians agree on diagnosis? Am J Respir Crit Care Med 2007; 175: 1054-1060.
20. Walsh SL, Wells AU, Desai SR, et al. Multicentre evaluation of multidisciplinary team meeting agreement on diagnosis in diffuse parenchymal lung disease: a case-cohort study. Lancet Respir Med 2016; 4: 557-565.
21. Jo HE, Glaspole IN, Levin KC, et al. Clinical impact of the interstitial lung disease multidisciplinary service. Respirology 2016; 21: 1438-1444.
22. Walsh SLF, Hansell D, Flaherty KR, et al. Accuracy of a clinical diagnosis of idiopathic pulmonary fibrosis: a study of 275 pulmonologists from 54 countries [abstract]. Am J Respir Crit Care Med 2017; 195: A1130.
23. Noble PW, Albera C, Bradford WZ, et al. Pirfenidone in patients with idiopathic pulmonary fibrosis (CAPACITY): two randomised trials. Lancet 2011; 377: 1760-1769.
24. Albera C, Bradford WZ, Costabel U, et al. Pirfenidone is efficacious in patients with idiopathic pulmonary fibrosis (IPF) and mild restrictive disease [abstract]. Am J Respir Crit Care Med 2015; 191: A1018.
25. Kolb M, Richeldi L, Behr J, Maher TM. Nintedanib in patients with idiopathic pulmonary fibrosis and preserved lung volume. 2017; 72: 340-346.
26. Sakamoto S, Itoh T, Muramatsu Y, et al. Efficacy of pirfenidone in patients with advanced-stage idiopathic pulmonary fibrosis. Intern Med 2013; 52: 2495-2501.
27. Okuda R, Hagiwara E, Baba T, et al. Safety and efficacy of pirfenidone in idiopathic pulmonary fibrosis in clinical practice. Respir Med 2013; 107: 1431-1437.
28. Troy L, Glaspole I, Goh N, et al. Prevalence and prognosis of unclassifiable interstitial lung disease. Eur Respir J 2014; 43: 1529-1530.
29. Ryerson CJ, Urbania TH, Richeldi L, et al. Prevalence and prognosis of unclassifiable interstitial lung disease. Eur Respir J 2013; 42: 750-757.
30. 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: 1390-1394.
31. 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: 369-376.
32. Raghu G, Crestani B, Bailes Z, et al. Effect of anti-acid medication on reduction in FVC decline with nintedanib. Eur Respir J 2015; 46 (Suppl 59): OA4502.
33. Patel NM, Lederer DJ, Borczuk AC, Kawut SM. Pulmonary hypertension in idiopathic pulmonary fibrosis. Chest 2007; 132: 998-1006.
34. Raghu G, Behr J, Brown KK, et al. Treatment of idiopathic pulmonary fibrosis with ambrisentan: a parallel, randomized trial. Ann Intern Med 2013; 158: 641-649.
35. Raghu G, Million-Rousseau R, Morganti A, et al. Macitentan for the treatment of idiopathic pulmonary fibrosis: the randomised controlled MUSIC trial. Eur Respir J 2013; 42: 1622-1632.
36. King TE Jr. Bosentan for idiopathic pulmonary fibrosis. Curr Opin Investig Drugs 2008; 9: 1171-1179.
37. Zisman DA, Schwarz M, Anstrom KJ, et al. A controlled trial of sildenafil in advanced idiopathic pulmonary fibrosis. N Engl J Med 2010; 363: 620-628.
38. Rochwerg B, Neupane B, Zhang Y, et al. Treatment of idiopathic pulmonary fibrosis: a network meta-analysis. BMC Med 2016; 14: 18.
39. Kolilekas L, Manali E, Vlami KA, et al. Sleep oxygen desaturation predicts survival in idiopathic pulmonary fibrosis. J Clin Sleep Med 2013; 9: 593-601.
40. Pihtili A, Bingol Z, Kiyan E, et al. Obstructive sleep apnea is common in patients with interstitial lung disease. Sleep Breathing 2013; 17: 1281-1288.
41. Corte T, Cottin V, Taniguchi H, et al. Effect of baseline emphysema on reduction in FVC decline with nintedanib in the INPULSIS trials. Respirology 2015; 20: 57.
42. 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; 317: 681-686.
43. Nocturnal Oxygen Therapy Trial Group. Continuous or nocturnal oxygen therapy in hypoxemic chronic obstructive lung disease: a clinical trial. Ann Intern Med 1980; 93: 391-398.
44. Dowman L, Hill CJ, Holland AE. Pulmonary rehabilitation for interstitial lung disease. Cochrane Database Syst Rev 2014; 10: CD006322.
45. Holland AE, Hill CJ, Glaspole I, et al. Predictors of benefit following pulmonary rehabilitation for interstitial lung disease. Respir Med 2012; 106: 429-435.
46. 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.
47. Bennett D, Fossi A, Bargagli E, et al. Mortality on the waiting list for lung transplantation in patients with idiopathic pulmonary fibrosis: a single-centre experience. Lung 2015; 193: 677-681.

Online responses are no longer available. Please refer to our instructions for authors page for more information.