Evolving Concepts and Controversies in Interstitial Lung diseases: Update in 2023
Main Article Content
Abstract
Recent studies elicited evolving concepts and controversies in interstitial lung diseases and proposed substantial changes in diagnostic and therapeutic approaches to interstitial lung diseases. Dr. Averill A. Liebow first coined the term usual interstitial pneumonia (UIP) in 1960’s as distinct pathologic pattern of fibrosis in idiopathic pulmonary fibrosis (IPF), the prototype of progressive fibrosing interstitial lung disease with poor prognosis. Advances in omics led to a better understanding of molecular pathogenesis of UIP and shed light on various types of familial pulmonary fibrosis as well as familial IPF. The concept of progressive pulmonary fibrosis was introduced to acknowledge additional types of progressive fibrosing interstitial lung diseases with the clinical and pathologic phenotypes very similar to those of UIP/IPF. As such, some authors have proposed a paradigm shift by considering UIP as a stand-alone diagnostic entity to encompass other fibrosing interstitial lung diseases that undergo the same relentless progression as IPF. Cicatricial organizing pneumonia is a variant of organizing pneumonia that can be reminiscent of UIP on histopathology but usually follows a stable clinical course unlike UIP. There has been significant disconnection in fundamental understanding as well as diagnostic criteria of lymphocytic interstitial pneumonia among pathologists, pulmonologists and radiologists, which needs to be resolved. The concept and histopathologic criteria of granulomatous and lymphocytic interstitial lung disease are also elusive and require clarification as well. In this review, these topics will be covered based on current literature.
Keywords:
idiopathic pulmonary fibrosis (IPF), usual interstitial pneumonia (UIP), familial pulmonary fibrosis, progressive pulmonary fibrosis, cicatricial organizing pneumonia (CiOP), lymphocytic interstitial pneumonia (LIP), granulomatous and lymphocytic interstitial lung disease (GLILD), American Thoracic Society (ATS), European Respiratory Society (ERS), Japanese Respiratory Society (JRS), Asociacion Latinoamericana de Torax (ALAT)
Article Details
How to Cite
WAWRYKO, Paul; YI, Eunhee S..
Evolving Concepts and Controversies in Interstitial Lung diseases: Update in 2023.
Medical Research Archives, [S.l.], v. 11, n. 8, aug. 2023.
ISSN 2375-1924.
Available at: <https://esmed.org/MRA/mra/article/view/4305>. Date accessed: 04 dec. 2024.
doi: https://doi.org/10.18103/mra.v11i8.4305.
Section
Research Articles
The Medical Research Archives grants authors the right to publish and reproduce the unrevised contribution in whole or in part at any time and in any form for any scholarly non-commercial purpose with the condition that all publications of the contribution include a full citation to the journal as published by the Medical Research Archives.
References
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7. Selman, M., A. Pardo, and A.U. Wells, Usual interstitial pneumonia as a stand-alone diagnostic entity: the case for a paradigm shift? Lancet Respir Med, 2023. 11(2): p. 188-196.
8. Collard, H.R., et al., Acute exacerbations of idiopathic pulmonary fibrosis. Am J Respir Crit Care Med, 2007. 176(7): p. 636-43.
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10. Richeldi, L., et al., Efficacy of a tyrosine kinase inhibitor in idiopathic pulmonary fibrosis. N Engl J Med, 2011. 365(12): p. 1079-87.
11. Richeldi, L., et al., Efficacy and safety of nintedanib in idiopathic pulmonary fibrosis. N Engl J Med, 2014. 370(22): p. 2071-82.
12. Azuma, A., et al., Double-blind, placebo-controlled trial of pirfenidone in patients with idiopathic pulmonary fibrosis. Am J Respir Crit Care Med, 2005. 171(9): p. 1040-7.
13. Taniguchi, H., et al., Pirfenidone in idiopathic pulmonary fibrosis. Eur Respir J, 2010. 35(4): p. 821-9.
14. Raghu, G., 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. 192(2): p. e3-19.
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16. Hambly, N., et al., Prevalence and characteristics of progressive fibrosing interstitial lung disease in a prospective registry. Eur Respir J, 2022. 60(4).
17. Hozumi, H., et al., Acute exacerbation in rheumatoid arthritis-associated interstitial lung disease: a retrospective case control study. BMJ Open, 2013. 3(9): p. e003132.
18. Kang, J., et al., Acute exacerbation of fibrotic hypersensitivity pneumonitis: incidence and outcomes. Respir Res, 2021. 22(1): p. 152.
19. Adegunsoye, A., et al., Computed Tomography Honeycombing Identifies a Progressive Fibrotic Phenotype with Increased Mortality across Diverse Interstitial Lung Diseases. Ann Am Thorac Soc, 2019. 16(5): p. 580-588.
20. Sandoz, E., Uber zwei falle von fotaler bronchektasie. Beitr Pathol Anat, 1907. 41: p. 496-517.
21. Marshall, R.P., et al., Adult familial cryptogenic fibrosing alveolitis in the United Kingdom. Thorax, 2000. 55(2): p. 143-6.
22. Hodgson, U., T. Laitinen, and P. Tukiainen, Nationwide prevalence of sporadic and familial idiopathic pulmonary fibrosis: evidence of founder effect among multiplex families in Finland. Thorax, 2002. 57(4): p. 338-42.
23. Barlo, N.P., et al., Surfactant protein-D predicts survival in patients with idiopathic pulmonary fibrosis. Sarcoidosis Vasc Diffuse Lung Dis, 2009. 26(2): p. 155-61.
24. Garcia-Sancho, C., et al., Familial pulmonary fibrosis is the strongest risk factor for idiopathic pulmonary fibrosis. Respir Med, 2011. 105(12): p. 1902-7.
25. Loyd, J.E., Pulmonary fibrosis in families. Am J Respir Cell Mol Biol, 2003. 29(3 Suppl): p. S47-50.
26. Fernandez, B.A., et al., A Newfoundland cohort of familial and sporadic idiopathic pulmonary fibrosis patients: clinical and genetic features. Respir Res, 2012. 13(1): p. 64.
27. Ley, B., et al., The MUC5B promoter polymorphism and telomere length in patients with chronic hypersensitivity pneumonitis: an observational cohort-control study. Lancet Respir Med, 2017. 5(8): p. 639-647.
28. Newton, C.A., et al., Telomere length and genetic variant associations with interstitial lung disease progression and survival. Eur Respir J, 2019. 53(4).
29. Cutting, C.C., et al., Family History of Pulmonary Fibrosis Predicts Worse Survival in Patients With Interstitial Lung Disease. Chest, 2021. 159(5): p. 1913-1921.
30. Steele, M.P., et al., Clinical and pathologic features of familial interstitial pneumonia. Am J Respir Crit Care Med, 2005. 172(9): p. 1146-52.
31. Newton, C.A., et al., Telomere-related lung fibrosis is diagnostically heterogeneous but uniformly progressive. Eur Respir J, 2016. 48(6): p. 1710-1720.
32. Mathai, S.K., et al., MUC5B variant is associated with visually and quantitatively detected preclinical pulmonary fibrosis. Thorax, 2019. 74(12): p. 1131-1139.
33. Hunninghake, G.M., et al., Interstitial Lung Disease in Relatives of Patients with Pulmonary Fibrosis. Am J Respir Crit Care Med, 2020. 201(10): p. 1240-1248.
34. Salisbury, M.L., et al., Development and Progression of Radiologic Abnormalities in Individuals at Risk for Familial Interstitial Lung Disease. Am J Respir Crit Care Med, 2020. 201(10): p. 1230-1239.
35. Mathai, S.K., et al., Pulmonary fibrosis in the era of stratified medicine. Thorax, 2016. 71(12): p. 1154-1160.
36. Seibold, M.A., et al., A common MUC5B promoter polymorphism and pulmonary fibrosis. N Engl J Med, 2011. 364(16): p. 1503-12.
37. Nogee, L.M., et al., A mutation in the surfactant protein C gene associated with familial interstitial lung disease. N Engl J Med, 2001. 344(8): p. 573-9.
38. van Moorsel, C.H.M., J.J. van der Vis, and J.C. Grutters, Genetic disorders of the surfactant system: focus on adult disease. Eur Respir Rev, 2021. 30(159).
39. Thomas, A.Q., et al., Heterozygosity for a surfactant protein C gene mutation associated with usual interstitial pneumonitis and cellular nonspecific interstitial pneumonitis in one kindred. Am J Respir Crit Care Med, 2002. 165(9): p. 1322-8.
40. van Moorsel, C.H., et al., SFTPA2 Mutations in Familial and Sporadic Idiopathic Interstitial Pneumonia. Am J Respir Crit Care Med, 2015. 192(10): p. 1249-52.
41. Armanios, M.Y., et al., Telomerase mutations in families with idiopathic pulmonary fibrosis. N Engl J Med, 2007. 356(13): p. 1317-26.
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