Interstitial lung diseases (ILDs) represent a diverse group of diffuse parenchymal lung disorders characterized by significant morbidity and mortality worldwide. Recent advances have led to the development of a new classification system for idiopathic interstitial pneumonias, dividing them into major, rare, and unclassified groups, with a novel approach based on disease behavior. Idiopathic pulmonary fibrosis (IPF) remains the most fatal ILD, demonstrating considerable clinical heterogeneity and poor prognosis. Early diagnosis and disease stratification through biomarkers and imaging are critical for managing IPF and other ILDs. ILDs impact an estimated 650,000 individuals in the United States. Among the various forms, IPF, hypersensitivity pneumonitis, and ILD linked to connective tissue disorders are the most frequently diagnosed subtypes. Connective tissue disease-associated ILDs pose diagnostic and therapeutic challenges, with ongoing research focusing on pathogenesis and treatment strategies. Environmental and occupational exposures contribute significantly to ILD development, necessitating thorough history-taking and antigen avoidance in hypersensitivity pneumonitis. The recognition of progressive-fibrosing ILD (PF-ILD) has further expanded treatment options, including antifibrotic therapies. Additionally, ILD has emerged as an adverse effect associated with novel therapies, such as antibody-drug conjugates, underscoring the need for vigilant monitoring. Despite progress in classification, diagnosis, and management, ILDs remain complex conditions requiring multidisciplinary approaches and continued research to improve outcomes. The global burden of ILD and pulmonary sarcoidosis is substantial, with prevalence rates highlighting the need for enhanced awareness and intervention.

Keywords: Interstitial lung disease, idiopathic pulmonary fibrosis, connective tissue disease, hypersensitivity pneumonitis, progressive-fibrosing ILD

🔒 This is a subscription article

Full text is available to subscribers and institutional members. Please choose an option below to access it.

1. Interstitial lung disease (ILD) ppt slideshare. Available at

       https://www.slideshare.net/slideshow/interstitial-lung-disease-ild-ppt-slideshare/249315412.

2. Hinkle JL, Cheever KH. Brunner & Suddarth’s Textbook of Medical-Surgical Nursing. 13th ed. New Delhi: Reed Elsevier India Pvt. Ltd.; 2014. pp. 360–395.
3. Lewis SL, Dirksen SR, Heitkemper MM, Bucher L, Harding MM. Medical-Surgical Nursing: Assessment and Management of Clinical Problems. 9th ed. New Delhi: Elsevier; 2013. pp. 461–493.
4. Mueller-Mang C, Ringl H, Herold C. Interstitial lung diseases. Multislice CT. 2017:261–288. doi: 10.1007/174_2017_151.
5. Althobiani MA, Russell AM, Jacob J, Ranjan Y, Folarin AA, Hurst JR, et al. Interstitial lung disease: A review of classification, etiology, epidemiology, clinical diagnosis, pharmacological and non-pharmacological treatment. Front Med (Lausanne). 2024;11:1296890. doi: 10.3389/fmed.2024.1296890.
6. Blanc PD, Annesi-Maesano I, Balmes JR, Cummings KJ, Fishwick D, Miedinger D, et al. The occupational burden of nonmalignant respiratory diseases. An Official American Thoracic Society and European Respiratory Society Statement. Am J Respir Crit Care Med. 2019;199(11):1312–1334. doi: 10.1164/rccm.201904-0717ST.
7. Flaherty KR, Wells AU, Cottin V, Devaraj A, Walsh SLF, Inoue Y, et al. INBUILD Trial Investigators. Nintedanib in progressive fibrosing interstitial lung diseases. N Engl J Med. 2019;381(18):1718–1727. doi: 10.1056/NEJMoa1908681.
8. Skeoch S, Weatherley N, Swift AJ, Oldroyd A, Johns C, Hayton C, et al. Drug-induced interstitial lung disease: A systematic review. J Clin Med. 2018;7(10):356. doi: 10.3390/jcm7100356.
9. Capron F. Nouvelle classification des pneumopathies interstitielles diffuses [New classification of interstitial lung disease]. Rev Pneumol Clin. 2005;61(3):133–140. French. doi: 10.1016/s0761-8417(05)84804-7.
10. Raghu G, Nyberg F, Morgan G. The epidemiology of interstitial lung disease and its association with lung cancer. Br J Cancer. 2004;91 Suppl 2(Suppl 2):S3–10. doi: 10.1038/sj.bjc.6602061.
11. Coultas DB, Zumwalt RE, Black WC, Sobonya RE. The epidemiology of interstitial lung diseases. Am J Respir Crit Care Med. 1994;150(4):967–972. doi: 10.1164/ajrccm.150.4.7921471.
12. Olson A, Hartmann N, Patnaik P, Wallace L, Schlenker-Herceg R, Nasser M, et al. Estimation of the prevalence of progressive fibrosing interstitial lung diseases: Systematic literature review and data from a physician survey. Adv Ther. 2021;38(2):854–867. doi: 10.1007/s12325-020-01578-6.

Interstitial lung diseases (ILDs) represent a diverse group of diffuse parenchymal lung disorders characterized by significant morbidity and mortality worldwide. Recent advances have led to the development of a new classification system for idiopathic interstitial pneumonias, dividing them into major, rare, and unclassified groups, with a novel approach based on disease behavior. Idiopathic pulmonary fibrosis (IPF) remains the most fatal ILD, demonstrating considerable clinical heterogeneity and poor prognosis. Early diagnosis and disease stratification through biomarkers and imaging are critical for managing IPF and other ILDs. ILDs impact an estimated 650,000 individuals in the United States. Among the various forms, IPF, hypersensitivity pneumonitis, and ILD linked to connective tissue disorders are the most frequently diagnosed subtypes. Connective tissue disease-associated ILDs pose diagnostic and therapeutic challenges, with ongoing research focusing on pathogenesis and treatment strategies. Environmental and occupational exposures contribute significantly to ILD development, necessitating thorough history-taking and antigen avoidance in hypersensitivity pneumonitis. The recognition of progressive-fibrosing ILD (PF-ILD) has further expanded treatment options, including antifibrotic therapies. Additionally, ILD has emerged as an adverse effect associated with novel therapies, such as antibody-drug conjugates, underscoring the need for vigilant monitoring. Despite progress in classification, diagnosis, and management, ILDs remain complex conditions requiring multidisciplinary approaches and continued research to improve outcomes. The global burden of ILD and pulmonary sarcoidosis is substantial, with prevalence rates highlighting the need for enhanced awareness and intervention.

Keywords: Interstitial lung disease, idiopathic pulmonary fibrosis, connective tissue disease, hypersensitivity pneumonitis, progressive-fibrosing ILD

🔒 This is a subscription article

Full text is available to subscribers and institutional members. Please choose an option below to access it.

1. Interstitial lung disease (ILD) ppt slideshare. Available at

       https://www.slideshare.net/slideshow/interstitial-lung-disease-ild-ppt-slideshare/249315412.

2. Hinkle JL, Cheever KH. Brunner & Suddarth’s Textbook of Medical-Surgical Nursing. 13th ed. New Delhi: Reed Elsevier India Pvt. Ltd.; 2014. pp. 360–395.
3. Lewis SL, Dirksen SR, Heitkemper MM, Bucher L, Harding MM. Medical-Surgical Nursing: Assessment and Management of Clinical Problems. 9th ed. New Delhi: Elsevier; 2013. pp. 461–493.
4. Mueller-Mang C, Ringl H, Herold C. Interstitial lung diseases. Multislice CT. 2017:261–288. doi: 10.1007/174_2017_151.
5. Althobiani MA, Russell AM, Jacob J, Ranjan Y, Folarin AA, Hurst JR, et al. Interstitial lung disease: A review of classification, etiology, epidemiology, clinical diagnosis, pharmacological and non-pharmacological treatment. Front Med (Lausanne). 2024;11:1296890. doi: 10.3389/fmed.2024.1296890.
6. Blanc PD, Annesi-Maesano I, Balmes JR, Cummings KJ, Fishwick D, Miedinger D, et al. The occupational burden of nonmalignant respiratory diseases. An Official American Thoracic Society and European Respiratory Society Statement. Am J Respir Crit Care Med. 2019;199(11):1312–1334. doi: 10.1164/rccm.201904-0717ST.
7. Flaherty KR, Wells AU, Cottin V, Devaraj A, Walsh SLF, Inoue Y, et al. INBUILD Trial Investigators. Nintedanib in progressive fibrosing interstitial lung diseases. N Engl J Med. 2019;381(18):1718–1727. doi: 10.1056/NEJMoa1908681.
8. Skeoch S, Weatherley N, Swift AJ, Oldroyd A, Johns C, Hayton C, et al. Drug-induced interstitial lung disease: A systematic review. J Clin Med. 2018;7(10):356. doi: 10.3390/jcm7100356.
9. Capron F. Nouvelle classification des pneumopathies interstitielles diffuses [New classification of interstitial lung disease]. Rev Pneumol Clin. 2005;61(3):133–140. French. doi: 10.1016/s0761-8417(05)84804-7.
10. Raghu G, Nyberg F, Morgan G. The epidemiology of interstitial lung disease and its association with lung cancer. Br J Cancer. 2004;91 Suppl 2(Suppl 2):S3–10. doi: 10.1038/sj.bjc.6602061.
11. Coultas DB, Zumwalt RE, Black WC, Sobonya RE. The epidemiology of interstitial lung diseases. Am J Respir Crit Care Med. 1994;150(4):967–972. doi: 10.1164/ajrccm.150.4.7921471.
12. Olson A, Hartmann N, Patnaik P, Wallace L, Schlenker-Herceg R, Nasser M, et al. Estimation of the prevalence of progressive fibrosing interstitial lung diseases: Systematic literature review and data from a physician survey. Adv Ther. 2021;38(2):854–867. doi: 10.1007/s12325-020-01578-6.