PULMONARY FIBROSIS IS A COMMON THREAT IN FIBROTIC ILDs1–4
There are a wide range of ILDs that are characterized by pulmonary fibrosis, such as IPF, iNSIP, unclassifiable ILD, cHP, as well as CTD-ILDs.1–4,14 ILD occurs in ~15% of CTD patients and at higher rates in certain conditions, such as SSc and RA.10
ILDs make up a diverse group of more than 200 heterogeneous lung disorders, mostly classified as rare or only infrequently seen in clinical practice1,10,11
Although individual fibrotic ILDs can be rare compared to other conditions, collectively they affect a considerable number of patients, representing a substantial burden of disease14
Relative prevalence of ILDs, and proportion estimates of patients who develop a progressive fibrosing phenotype, accompanied by detailed equivalent information on CTD-ILDs14
Adapted from: Wijsenbeek M, Cottin V. N Engl J Med. 2020;383:958–968.
FIND OUT MORE ABOUT THE CRITICAL THREAT OF PULMONARY FIBROSIS IN ILDs
See what Vincent Cottin, Professor of Respiratory Medicine, has to say about the importance of pulmonary fibrosis in IPF and in a range of non-IPF ILDs
Fibrosis is an excessive deposition of extracellular matrix within the lungs. Pulmonary fibrosis is actually characterised histologically, and pulmonary fibrosis occurs in a number of conditions, including idiopathic pulmonary fibrosis, which is most typical of these conditions characterised by progressive onset, but what we have learned in recent years is the importance also of pulmonary fibrosis in other conditions. What we call pulmonary fibrosis may also occur in other conditions, for example, in connective tissue disease, scleroderma, rheumatoid arthritis with interstitial lung disease, and other connective tissue disease. In chronic HP, chronic hypersensitivity pneumonitis, which is related to exposure to an antigen, there are acute forms that are reversible, but there are also chronic forms that often include some fibrosis.
What could the threat posed by fibrotic ILDs mean for your patients?
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- Wells AU, Brown KK, Flaherty KR, Kolb M, Thannickal VJ, on behalf of the IPF Consensus Working Group. Eur Resp J. 2018;51:1800692.
- Kolb M, Bondue B, Pesci A, et al. Acute exacerbations of progressive-fibrosing interstitial lung diseases. Eur Respir Rev. 2018;27(150):pii:180071.
- Collard HR, Ryerson CJ, Corte TJ, et al. Acute exacerbation of idiopathic pulmonary fibrosis: an international working group report. Am J Respir Crit Care Med. 2016;194(3):265–275.
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- Song JW, Lee HK, Lee CK, et al. Clinical course and outcome of rheumatoid arthritis-related usual interstitial pneumonia. Sarcoidosis Vasc Diffuse Lung Dis. 2013;30(2):103-112.
- Wijsenbeek M, Cottin V. Spectrum of Fibrotic Lung Diseases. N Engl J Med. 2020;383:958–968.
- Cottin V, Hirani N, Hotchkin D, et al. Presentation, diagnosis and clinical course of the spectrum of progressive-fibrosing interstitial lung diseases. Eur Respir Rev. 2018;27(150):180076.
- Demedts M, Wells AU, AntÓ JM, et al. Interstitial lung diseases: an epidemiological overview. Eur Respir J Suppl. 2001;18(suppl32):2s-16s.
- Cottin V, Wollin L, Fischer A, et al. Fibrosing interstitial lung diseases: knowns and unknowns. Eur Respir Rev. 2019b;28(151):pii:180100.
- Kolb M, Vašáková M. The natural history of progressive fibrosing interstitial lung diseases. Respir Res. 2019;20(1).
- Wijsenbeek M, Cottin V. Spectrum of Fibrotic Lung Diseases. N Engl J Med. 2020;383:958–968. Supplementary Appendix.
- Fischer A and Distler J. Progressive fibrosing interstitial lung disease associated with systemic autoimmune diseases. Clin Rheumatol. 2019;38(10):2673–2681.
- Mathai SC and Danoff SK. Management of interstitial lung disease associated with connective tissue disease. BMJ. 2016;352:h6819.
- Wallace B, Vummidi D, Khanna D. Management of connective tissue diseases associated interstitial lung disease: a review of the published literature. Curr Opin Rheumatol. 2016;28(3):236–245.
- Spagnolo P, Cordier JF, Cottin V. Connective tissue diseases, multimorbidity and the ageing lung. Eur Respir J. 2016;47(5):1535–1558.
- Tomiyama F, Watanabe R, Ishii T, et al. High Prevalence of Acute Exacerbation of Interstitial Lung Disease in Japanese Patients with Systemic Sclerosis. Tohoku J Exp Med. 2016;239, 297–305.
- Okamoto M, Fujimoto K, Sadohara J, et al. A retrospective cohort study of outcome in systemic sclerosis-associated interstitial lung disease. Respiratory Investigation. 2016;54, 445–453.