A systematic approach to the management of patients with advanced interstitial lung disease
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Keywords
fibrotic lung disease, interstitial lung disease, advanced interstitial lung disease
Abstract
Background: Interstitial lung disease (ILD) is a heterogeneous group of diseases characterized by clinical, physiologic, and radiologic abnormalities. Advanced ILD (sometimes referred to as end-stage interstitial lung disease) is characterized by either gradual or rapid progressive decline of pre-existing or newly diagnosed ILD with worsening of symptoms, pulmonary function tests, six-minute walk test (6MWT) distance, and/or imaging findings, specifically high-resolution computed tomography (HRCT) imaging of the chest affecting patient’s quality of life. Special considerations are required for the management of patients with these advanced ILDs, particularly those who will ultimately require lung transplantation. Though several society and expert panels provide guidance on ILD management, there are none focused specifically on the approach to patients with advanced ILD.
Objectives: In this review, we attempt to define advanced ILD and identify the clinical considerations required for diagnosis and management of patients with advanced ILD.
Discussion: Pathophysiologic mechanisms of ILD and complications are not completely understood but include inflammation, fibrosis, pulmonary hypertension, venous thromboembolism, recurrent infections, and aspiration. Management of these patients includes consideration of anti-fibrotic medications, anti-inflammatory/immunosuppressive medications and treatment of concurrent pulmonary hypertension as indicated on a case-by-case basis. Non-pharmacologic management with supplemental oxygen, pulmonary rehabilitation, and palliative care are tailored to each patient’s clinical status. Finally, early referral to lung transplantation is critical, as advanced ILD can be fatal, and sometimes rapidly so.
Conclusion: Advanced ILD is an oftentimes fatal group of heterogeneous diseases that have varying managements depending on underlying subtype. Identification of progressive disease and complications are the first steps to management of these patients to optimize lung function and quality of life and improve outcomes before lung transplantation.
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2. Walsh SLF, Devaraj A, Enghelmayer JI, et al. Role of imaging in progressive-fibrosing interstitial lung diseases. Eur Respir Rev 2018;27(150):180073. doi: 10.1183/16000617.0073-2018.
3. Leong SW, Bos S, Lordan JL, Nair A, Fisher AJ, Meachery G. Lung transplantation for interstitial lung disease: evolution over three decades. BMJ Open Respir Res 2023;10(1):e001387. doi: 10.1136/bmjresp-2022-001387.
4. Zhang N, Liu S, Zhang Z, Liu Y, Mi L, Xu K. Lung Transplantation: A Viable Option for Connective Tissue Disease? Arthritis Care Res (Hoboken) 2023;75(11):2389-2398.
5. 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(4):431-40. doi: 10.1164/rccm.201006-0894CI.
6. Gupta R, Judson MA, Baughman RP. Management of Advanced Pulmonary Sarcoidosis. Am J Respir Crit Care Med 2022;205(5):495-506. doi: 10.1164/rccm.202106-1366CI.
7. Cottin V, Hirani NA, Hotchkin DL, et al. Presentation, diagnosis and clinical course of the spectrum of progressive-fibrosing interstitial lung diseases. Eur Respir Rev 2018;27(150):180076. doi: 10.1183/16000617.0076-2018.
8. Raghu G, Remy-Jardin M, Richeldi L, et al. 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;205(9):e18-e47.
9. Faverio P, De Giacomi F, Bonaiti G, et al. Management of Chronic Respiratory Failure in Interstitial Lung Diseases: Overview and Clinical Insights. Int J Med Sci 2019; 16(7): 967-980.
10. Podolanczuk AJ, Wong AW, Saito S, Lasky JA, Ryerson CJ, Eickelberg O. Update in Interstitial Lung Disease 2020. Am J Respir Crit Care Med 2021;203(11):1343-1352. doi: 10.1164/rccm.202103-0559UP.
11. Raghu G, Remy-Jardin M, Myers JL, et al. Diagnosis of idiopathic pulmonary fibrosis: an official ATS/ERS/JRS/ALAT clinical practice guideline. Am J Respir Crit Care Med 2018; 198(5):e44-e68.
12. Fernandez-Perez ER, Travis WD, et al. Diagnosis and Evaluation of Hypersensitivity Pneumonitis: CHEST Guideline and Expert Panel Report. Chest 2021;160(2):e97-e156. doi: 10.1016/j.chest.2021.03.066.
13. Briganti DF, D'Ovidio F. Long-term management of patients with end-stage lung diseases. Best Pract Res Clin Anaesthesiol 2017;31(2):167-178. doi: 10.1016/j.bpa.2017.07.007.
14. Flaherty KR, Thwaite EL, Kazerooni EA, et al. Radiological versus histological diagnosis in UIP and NSIP: survival implications. Thorax 2003; 58(2):143-8. doi: 10.1136/thorax.58.2.143.
15. Bonham CA, Strek ME, Patterson KC. From granuloma to fibrosis: sarcoidosis associated pulmonary fibrosis. Curr Opin Pulm Med 2016;22(5):484-91. doi: 10.1097/MCP.0000000000000301.
16. Trivieri MG, Spagnolo P, Birnie D, Liu P, Drake W, Kovacic JC, Baughman R, Fayad ZA, Judson MA. Challenges in Cardiac and Pulmonary Sarcoidosis: JACC State-of-the-Art Review. J Am Coll Cardiol 2020;76(16):1878-1901.
17. Park JH, Kim DS, Park IN, et al. Prognosis of fibrotic interstitial pneumonia: idiopathic versus collagen vascular disease-related subtypes. Am J Respir Crit Care Med 2007;175(7):705-11. doi: 10.1164/rccm.200607-912OC.
18. Khush KK, Cherikh WS, Chambers DC, et al; International Society for Heart and Lung Transplantation. The International Thoracic Organ Transplant Registry of the International Society for Heart and Lung Transplantation: Thirty-sixth adult heart transplantation report - 2019; focus theme: Donor and recipient size match. J Heart Lung Transplant 2019;38(10):1056-1066. doi: 10.1016/j.healun.2019.08.004.
19. Hsich E, Singh TP, Cherikh WS, et al; International Society for Heart and Lung Transplantation. The International thoracic organ transplant registry of the international society for heart and lung transplantation: Thirty-ninth adult heart transplantation report-2022; focus on transplant for restrictive heart disease. J Heart Lung Transplant 2022;41(10):1366-1375.
20. Harari S, Wells AU, Wuyts WA, et al. The 6-min walk test as a primary end-point in interstitial lung disease. Eur Respir Rev 2022;31(165):220087.
21. Callejas-Moraga EL, Guillén-Del-Castillo A, Perurena-Prieto J, et al. Anti-RNPC-3 antibody predicts poor prognosis in patients with interstitial lung disease associated to systemic sclerosis. Rheumatology (Oxford) 2021;61(1):154-162. doi: 10.1093/rheumatology/keab279.
22. Barratt SL, Davis R, Sharp C, Pauling JD. The prognostic value of cardiopulmonary exercise testing in interstitial lung disease: a systematic review. ERJ Open Res2020 Aug;6(3):00027-2020.
23. Ley B, Ryerson CJ, Vittinghoff E, et al. A multidimensional index and staging system for idiopathic pulmonary fibrosis. Ann Intern Med 2012;156(10):684-91. doi: 10.7326/0003-4819-156-10-201205150-00004.
24. Ryerson CJ, Vittinghoff E, Ley B, et al. Predicting survival across chronic interstitial lung disease: the ILD-GAP model. Chest 2014;145(4):723-728. doi: 10.1378/chest.13-1474.
25. Chandel A, Pastre J, Valery S, King CS, Nathan SD. Derivation and validation of a simple multidimensional index incorporating exercise capacity parameters for survival prediction in idiopathic pulmonary fibrosis. Thorax 2023;78(4):368-375.
26. Cao H, Huan C, Wang Q, Xu G, Lin J, Zhou J. Predicting Survival Across Acute Exacerbation of Interstitial Lung Disease in Patients with Idiopathic Inflammatory Myositis: The GAP-ILD Model. Rheumatol Ther 2020;7(4):967-978. doi: 10.1007/s40744-020-00244-1.
27. Mango RL, Matteson EL, Crowson CS, Ryu JH, Makol A. Assessing Mortality Models in Systemic Sclerosis-Related Interstitial Lung Disease. Lung 2018;196(4):409-416. doi: 10.1007/s00408-018-0126-6.
28. Nurmi HM, Purokivi MK, Kärkkäinen MS, Kettunen HP, Selander TA, Kaarteenaho RL. Are risk predicting models useful for estimating survival of patients with rheumatoid arthritis-associated interstitial lung disease? BMC Pulm Med 2017;17(1):16. doi: 10.1186/s12890-016-0358-2.
29. Kobayashi H, Naito T, Omae K, et al. ILD-NSCLC-GAP index scoring and staging system for patients with non-small cell lung cancer and interstitial lung disease. Lung Cancer 2018;121:48-53.
30. Brusca RM, Pinal-Fernandez I, Psoter K, et al. The ILD-GAP risk prediction model performs poorly in myositis-associated interstitial lung disease. Respir Med 2019;150:63-65. doi: 10.1016/j.rmed.2019.02.015.
31. Patel DC, Valeyre D. Advanced pulmonary sarcoidosis. Curr Opin Pulm Med 2020;26(5):574-581.
32. Yu D, Xiang Y, Gou T, Tong R, Xu C, Chen L, Zhong L, Shi J. New therapeutic approaches against pulmonary fibrosis. Bioorg Chem 2023;138:106592.
33. Richeldi L, du Bois RM, Raghu G, et al. Efficacy and safety of nintedanib in idiopathic pulmonary fibrosis. N Engl J Med 2014;370(22):2071-82. doi: 10.1056/NEJMoa1402584.
34. Shumar JN, Chandel A, King CS. Antifibrotic Therapies and Progressive Fibrosing Interstitial Lung Disease (PF-ILD): Building on INBUILD. J Clin Med 2021 May;10(11):2285.
35. King TE, 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(22): 2083–2092. doi:10.1056/nejmoa1402582.
36. Nathan SD, Costabel U, Glaspole I et al. Efficacy of Pirfenidone in the Context of Multiple Disease Progression Events in Patients With Idiopathic Pulmonary Fibrosis. Chest 2019; 155(4): 712-719.
37. Vancheri C, Kreuter M, Richeldi L, et al. Nintedanib with Add-on Pirfenidone in Idiopathic Pulmonary Fibrosis. Results of the INJOURNEY Trial. Am J Respir Crit Care Med 2018;197(3):356-363.
38. 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(21):1968-77. doi: 10.1056/NEJMoa1113354.
39. Morisset J, Johannson KA, Vittinghoff E, et al. Use of Mycophenolate Mofetil or Azathioprine for the Management of Chronic Hypersensitivity Pneumonitis. Chest 2017;151(3):619-625. doi: 10.1016/j.chest.2016.10.029.
40. Interstitial Lung Disease Guideline. ACR. Published August 22, 2023. https://rheumatology.org/interstitial-lung-disease-guideline
41. Zekić T. Rituximab as the first-line therapy in anti-synthetase syndrome-related interstitial lung disease. Rheumatol Int. 2023 Jun;43(6):1015-1021.
42. Tashkin DP, Roth MD, Clements PJ, et al; Sclerodema Lung Study II Investigators. Mycophenolate mofetil versus oral cyclophosphamide in scleroderma-related interstitial lung disease (SLS II): a randomised controlled, double-blind, parallel group trial. Lancet Respir Med 2016;4(9):708-719. doi: 10.1016/S2213-2600(16)30152-7.
43. Khanna D, Lin CJF, Furst DE, et al. Long-Term Safety and Efficacy of Tocilizumab in Early Systemic Sclerosis-Interstitial Lung Disease: Open-Label Extension of a Phase 3 Randomized Controlled Trial. Am J Respir Crit Care Med 2022;205(6):674-684. doi: 10.1164/rccm.202103-0714OC.
44. Comes A, Sofia C, Richeldi L. Novel insights in fibrotic pulmonary sarcoidosis. Curr Opin Pulm Med 2022;28(5):478-484.
45. Baughman RP, Valeyre D, Korsten P, et al. ERS clinical practice guidelines on treatment of sarcoidosis. Eur Respir J 2021;58(6):2004079.
46. Clinicaltrials.gov
47. Posner S, Zheng J, Wood RK, et al. Gastroesophageal reflux symptoms are not sufficient to guide esophageal function testing in lung transplant candidates. Dis Esophagus 2018;31(5). doi: 10.1093/dote/dox157.
48. Herbella FAM, Patti MG. Gastroesophageal Reflux Disease and Idiopathic Lung Fibrosis. From Heartburn to Lung Transplant, and Beyond. Am Surg. 2022 Feb;88(2):297-302. doi: 10.1177/0003134821998686.
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Kattih Z, Greenstein Z, Makkar P, Gupta R. A systematic approach to the management of patients with advanced interstitial lung disease. Sarcoidosis Vasc Diffuse Lung Dis [Internet]. [cited 2025 Jan. 22];42(2). Available from: https://mattioli1885journals.com/index.php/sarcoidosis/article/view/16206
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How to Cite
1.
Kattih Z, Greenstein Z, Makkar P, Gupta R. A systematic approach to the management of patients with advanced interstitial lung disease. Sarcoidosis Vasc Diffuse Lung Dis [Internet]. [cited 2025 Jan. 22];42(2). Available from: https://mattioli1885journals.com/index.php/sarcoidosis/article/view/16206
