Interstitial lung diseases

Marlies Wijsenbeek*, Atsushi Suzuki, Toby M. Maher

*Corresponding author for this work

Research output: Contribution to journalReview articleAcademicpeer-review

106 Citations (Scopus)


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.

Original languageEnglish
Pages (from-to)769-786
Number of pages18
JournalThe Lancet
Issue number10354
Publication statusPublished - 3 Sept 2022

Bibliographical note

We thank Jan von der Thüssen (pathologist), Arlette Odink (radiologist), and Jente Klok for their help with the images.

Publisher Copyright: © 2022 Elsevier Ltd


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