A review on Interstitial Lung Diseases: an overview from diagnosis to innovative therapies


Submitted: 29 June 2023
Accepted: 20 July 2023
Published: 29 September 2023
Abstract Views: 806
PDF: 102
Publisher's note
All claims expressed in this article are solely those of the authors and do not necessarily represent those of their affiliated organizations, or those of the publisher, the editors and the reviewers. Any product that may be evaluated in this article or claim that may be made by its manufacturer is not guaranteed or endorsed by the publisher.

Authors

Interstitial Lung Diseases (ILDs) enclose a wide heterogeneous group of more than 200 pathological disorders, which are characterized by an inflammatory and/or fibrotic pattern in the respiratory tract. In recent years, significant developments in the understanding of their pathogenesis have led to considerable progress in the therapeutic field and in improving clinical outcomes. COVID-19 pandemic has also had an important impact on the course of interstitial disease, particularly that which developed following infection in both symptomatic and asymptomatic patients, as well as in patients with pre-existing interstitial disease prior to infection. The purpose of this review is to describe clinical, radiological and therapeutic features of some of the most common interstitial lung diseases, as well as the multidisciplinary diagnostic procedure that underlies an increasingly accurate diagnosis.


Wijsenbeek M, Suzuki A, Maher TM. Interstitial lung diseases. Lancet. 2022;400:769-86. DOI: https://doi.org/10.1016/S0140-6736(22)01052-2

Bagnato G, Harari S. Cellular interactions in the pathogenesis of interstitial lung diseases. Eur Respir Rev. 2015;24:102-14. DOI: https://doi.org/10.1183/09059180.00003214

Shumar JN, Chandel A, King CS. Antifibrotic Therapies and Progressive Fibrosing Interstitial Lung Disease (PF-ILD): Building on INBUILD. J Clin Med. 2021;10:2285. DOI: https://doi.org/10.3390/jcm10112285

Behr J, Kreuter M, Hoeper MM, et al. Management of patients with idiopathic pulmonary fibrosis in clinical practice: the INSIGHTS-IPF registry. Eur Respir J. 2015;46:186-96. DOI: https://doi.org/10.1183/09031936.00217614

Raghu G, Amatto VC, Behr J, Stowasser S. Comorbidities in idiopathic pulmonary fibrosis patients: a systematic literature review. Eur Respir J. 2015;46:1113-30. DOI: https://doi.org/10.1183/13993003.02316-2014

Mikolasch TA, Garthwaite HS, Porter JC. Update in diagnosis and management of interstitial lung disease. Clin Med (Lond). 2017;17:146-53. DOI: https://doi.org/10.7861/clinmedicine.17-2-146

Raghu G, Remy-Jardin M, Myers JL, et al. An Official ATS/ERS/JRS/ALAT Clinical Practice Guideline. Am J Respir Crit Care Med. 2018;198:e44-68. DOI: https://doi.org/10.1164/rccm.201807-1255ST

Fernández Pérez ER, Swigris JJ, Forssén AV, et al. Identifying an inciting antigen is associated with improved survival in patients with chronic hypersensitivity pneumonitis. Chest. 2013;144:1644-51. DOI: https://doi.org/10.1378/chest.12-2685

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:e18-47. DOI: https://doi.org/10.1164/rccm.202202-0399ST

Maher TM, Bendstrup E, Dron L, et al. Global incidence and prevalence of idiopathic pulmonary fibrosis. Respir Res. 2021;22:197. DOI: https://doi.org/10.1186/s12931-021-01791-z

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. DOI: https://doi.org/10.1164/rccm.201308-1483ST

Sève P, Pacheco Y, Durupt F, et al. Sarcoidosis: A Clinical Overview from Symptoms to Diagnosis. Cells. 2021;10:766. DOI: https://doi.org/10.3390/cells10040766

Ketchersid K. A review of organizing pneumonia. JAAPA. 2023;36:16-9. DOI: https://doi.org/10.1097/01.JAA.0000918776.59717.eb

Qi XM, Luo Y, Song MY, et al. Pneumoconiosis: current status and future prospects. Chin Med J (Engl). 2021;134:898-907. DOI: https://doi.org/10.1097/CM9.0000000000001461

Raghu G, Remy-Jardin M, Ryerson CJ, et al. Diagnosis of Hypersensitivity Pneumonitis in Adults. An Official ATS/JRS/ALAT Clinical Practice Guideline. Am J Respir Crit Care Med. 2020;202:e36-69. Erratum in: Am J Respir Crit Care Med. 2021;203:150-1. Erratum in: Am J Respir Crit Care Med. 2022;206:518. DOI: https://doi.org/10.1164/rccm.v203erratum1

Kadura S, Raghu G. Rheumatoid arthritis-interstitial lung disease: manifestations and current concepts in pathogenesis and management. Eur Respir Rev. 2021;30:210011. DOI: https://doi.org/10.1183/16000617.0011-2021

Cavagna L, Monti S, Grosso V, et al. The multifaceted aspects of interstitial lung disease in rheumatoid arthritis. Biomed Res Int. 2013;2013:759760. DOI: https://doi.org/10.1155/2013/759760

Juge PA, Lee JS, Ebstein E, et al. MUC5B Promoter Variant and Rheumatoid Arthritis with Interstitial Lung Disease. N Engl J Med. 2018;379:2209-19. DOI: https://doi.org/10.1056/NEJMoa1801562

Wang D, Zhang J, Lau J, et al. Mechanisms of lung disease development in rheumatoid arthritis. Nat Rev Rheumatol. 2019;15:581-96. DOI: https://doi.org/10.1038/s41584-019-0275-x

Young A, Vummidi D, Visovatti S, et al. Prevalence, Treatment, and Outcomes of Coexistent Pulmonary Hypertension and Interstitial Lung Disease in Systemic Sclerosis. Arthritis Rheumatol. 2019;71:1339-49. DOI: https://doi.org/10.1002/art.40862

Perelas A, Silver RM, Arrossi AV, Highland KB. Systemic sclerosis-associated interstitial lung disease. Lancet Respir Med. 2020;8:304-20. DOI: https://doi.org/10.1016/S2213-2600(19)30480-1

Fujisawa T. Management of Myositis-Associated Interstitial Lung Disease. Medicina (Kaunas). 2021;57:347. DOI: https://doi.org/10.3390/medicina57040347

Fujisawa T, Hozumi H, Kono M, et al. Prognostic factors for myositis-associated interstitial lung disease. PLoS One. 2014;9:e98824. DOI: https://doi.org/10.1371/journal.pone.0098824

Lee CT, Strek ME. The other connective tissue disease-associated interstitial lung diseases: Sjogren's syndrome, mixed connective tissue disease, and systemic lupus erythematosus. Curr Opin Pulm Med. 2021;27:388-95. DOI: https://doi.org/10.1097/MCP.0000000000000791

Sogkas G, Hirsch S, Olsson KM, et al. Lung Involvement in Primary Sjögren's Syndrome-An Under-Diagnosed Entity. Front Med (Lausanne). 2020;7:332. DOI: https://doi.org/10.3389/fmed.2020.00332

Reiseter S, Gunnarsson R, Mogens Aaløkken T, et al. Progression and mortality of interstitial lung disease in mixed connective tissue disease: a long-term observational nationwide cohort study. Rheumatology (Oxford). 2018;57:255-62. DOI: https://doi.org/10.1093/rheumatology/kex077

Narváez J, Borrell H, Sánchez-Alonso F, et al. Primary respiratory disease in patients with systemic lupus erythematosus: data from the Spanish rheumatology society lupus registry (RELESSER) cohort. Arthritis Res Ther. 2018;20:280. DOI: https://doi.org/10.1186/s13075-018-1776-8

Toyoda Y, Koyama K, Kawano H, et al. Clinical features of interstitial pneumonia associated with systemic lupus erythematosus. Respir Investig. 2019;57:435-43. DOI: https://doi.org/10.1016/j.resinv.2019.04.005

Guler SA, Ryerson CJ. Unclassifiable interstitial lung disease: from phenotyping to possible treatments. Curr Opin Pulm Med. 2018;24:461-8. DOI: https://doi.org/10.1097/MCP.0000000000000509

O'Callaghan M, Bonella F, McCarthy C. Unclassifiable, or simply unclassified interstitial lung disease? Curr Opin Pulm Med. 2021;27:405-13. DOI: https://doi.org/10.1097/MCP.0000000000000801

Skeoch S, Weatherley N, Swift AJ, et al. Drug-Induced Interstitial Lung Disease: A Systematic Review. J Clin Med. 2018;7:356. DOI: https://doi.org/10.3390/jcm7100356

Conte P, Ascierto PA, Patelli G, et al. Drug-induced interstitial lung disease during cancer therapies: expert opinion on diagnosis and treatment. ESMO Open. 2022;7:100404. DOI: https://doi.org/10.1016/j.esmoop.2022.100404

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. DOI: https://doi.org/10.1056/NEJMoa1103690

Distler O, Highland KB, Gahlemann M, et al. SENSCIS Trial Investigators. Nintedanib for Systemic Sclerosis-Associated Interstitial Lung Disease. N Engl J Med. 2019;380:2518-28. DOI: https://doi.org/10.1056/NEJMoa1903076

Wells AU, Flaherty KR, Brown KK, et al. INBUILD trial investigators. Nintedanib in patients with progressive fibrosing interstitial lung diseases-subgroup analyses by interstitial lung disease diagnosis in the INBUILD trial: a randomised, double-blind, placebo-controlled, parallel-group trial. Lancet Respir Med. 2020;8:453-60. DOI: https://doi.org/10.1016/S2213-2600(20)30036-9

Noble PW, Albera C, Bradford WZ, et al. CAPACITY Study Group. Pirfenidone in patients with idiopathic pulmonary fibrosis (CAPACITY): two randomised trials. Lancet. 2011;377:1760-9. DOI: https://doi.org/10.1016/S0140-6736(11)60405-4

Khanna D, Albera C, Fischer A, et al. An Open-label, Phase II Study of the Safety and Tolerability of Pirfenidone in Patients with Scleroderma-associated Interstitial Lung Disease: the LOTUSS Trial. J Rheumatol. 2016;43:1672-9. DOI: https://doi.org/10.3899/jrheum.151322

Behr J, Prasse A, Kreuter M, et al. RELIEF investigators. Pirfenidone in patients with progressive fibrotic interstitial lung diseases other than idiopathic pulmonary fibrosis (RELIEF): a double-blind, randomised, placebo-controlled, phase 2b trial. Lancet Respir Med. 2021;9:476-86. DOI: https://doi.org/10.1016/S2213-2600(20)30554-3

Podolanczuk AJ, Wong AW, Saito S, et al. Update in Interstitial Lung Disease 2020. Am J Respir Crit Care Med. 2021;203:1343-52. DOI: https://doi.org/10.1164/rccm.202103-0559UP

John AE, Joseph C, Jenkins G, Tatler AL. COVID-19 and pulmonary fibrosis: A potential role for lung epithelial cells and fibroblasts. Immunol Rev. 2021;302:228-40. DOI: https://doi.org/10.1111/imr.12977

Shi H, Han X, Jiang N, et al. Radiological findings from 81 patients with COVID-19 pneumonia in Wuhan, China: a descriptive study. Lancet Infect Dis. 2020;20:425-34. DOI: https://doi.org/10.1016/S1473-3099(20)30086-4

Valenzuela C, Waterer G, Raghu G. Interstitial lung disease before and after COVID-19: a double threat? Eur Respir J. 2021;58:2101956. DOI: https://doi.org/10.1183/13993003.01956-2021

Bertolina, C., Bertolotti, M., Farotto, M., Giacchero, F., Ravera, S., Salio, M., Pasquali, M., & Maconi, A. (2023). A review on Interstitial Lung Diseases: an overview from diagnosis to innovative therapies. Working Paper of Public Health, 11(1). https://doi.org/10.4081/wpph.2023.9785

Downloads

Download data is not yet available.

Citations