Interstitial lung diseases.

Over 200 interstitial lung diseases, from ultra rare to relatively common, are recognised. Most interstitial lung diseases are characterised by inflammation or fibrosis within the interstitial space, the primary consequence of which is impaired gas exchange, resulting in breathlessness, diminished exercise tolerance, and decreased quality of life. Outcomes vary considerably for each of the different interstitial lung diseases. In some conditions, spontaneous reversibility or stabilisation can occur, but unfortunately in many people with interstitial lung disease, especially in those manifesting progressive pulmonary fibrosis, respiratory failure and death are a sad reality. Over the past 3 years, the field of interstitial lung disease has had important advances, with the approval of drugs to treat systemic sclerosis-associated interstitial lung disease, interstitial lung disease-associated pulmonary hypertension, and different forms of progressive pulmonary fibrosis. This Seminar provides an update on epidemiology, pathogenesis, presentation, diagnosis, disease course, and management of the interstitial lung diseases that are most frequently encountered in clinical practice. Furthermore, we describe how developments have led to a shift in the classification and treatment of interstitial lung diseases that exhibit progressive pulmonary fibrosis and summarise the latest practice-changing guidelines. We conclude with an outline of controversies, uncertainties, and future directions.

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Declaration of interests MW reports grants from The Netherlands Organisation for Health Research and Development, The Dutch Lung Foundation, Boehringer Ingelheim, The Dutch Pulmonary Fibrosis Patient Association, The Thorax Foundation Erasmus MC, and Sarcoidosis.nl; consulting fees from Boehringer Ingelheim, Hoffman la Roche, Galapagos, Bristol Myers Squibb, Galecto, PureTech Health, Kinevant Sciences, Molecure, Respivant, Nerretherapeutics, Horizontherapeutics, and CLS Behring; lecture fees from Boehringer Ingelheim, Hoffman la Roche, and Novartis; travel support from Boehringer Ingelheim, Hoffman la Roche, and Galapagos; and participation in data safety monitoring boards for Savara and Galapagos. All grants and fees were paid to her institution. AS reports lecture fees from Boehringer Ingelheim and GSK. TMM reports a grant from GSK paid to his institution; consulting fees from Boehringer Ingelheim, Roche, Galapagos, Bristol Myers Squibb, AstraZeneca, GSK, IQVIA, Pliant, Therevance, Galecto, Blade Therapeutics, Respivant, and Fibrogen; lecture honoraria from Boehringer Ingelheim, Roche, and Galapagos; travel support from Boehringer Ingelheim and Roche; and participation in data safety monitoring boards for Blade Therapeutics, Fibrogen, IQVIA, and Bergen Biosciences.