Early diagnosis of fibrotic interstitial lung disease: challenges and opportunities

Paolo Spagnolo*, Christopher J. Ryerson, Rachel Putman, Justin Oldham, Margaret Salisbury, Nicola Sverzellati, Claudia Valenzuela, Sabina Guler, Steve Jones, Marlies Wijsenbeek, Vincent Cottin

*Corresponding author for this work

Research output: Contribution to journalReview articlePopular

16 Citations (Scopus)

Abstract

Many patients with interstitial lung disease (ILD) develop pulmonary fibrosis, which can lead to reduced quality of life and early mortality. Patients with fibrotic ILD often have considerable diagnostic delay, and are exposed to unnecessary and costly diagnostic procedures, and ineffective and potentially harmful treatments. Non-specific and insidious presenting symptoms, along with scarce knowledge of fibrotic ILD among primary care physicians and non-ILD experts, are some of the main causes of diagnostic delay. Here, we outline and discuss the challenges facing both patients and physicians in making an early diagnosis of fibrotic ILD, and explore strategies to facilitate early identification of patients with fibrotic ILD, both in the general population and among individuals at highest risk of developing the disease. Finally, we discuss controversies and key uncertainties in screening programmes for fibrotic ILD. Timely identification and accurate diagnosis of patients with fibrotic ILD poses several substantial clinical challenges, but could potentially improve outcomes through early initiation of appropriate management.

Original languageEnglish
Pages (from-to)1065-1076
Number of pages12
JournalThe Lancet Respiratory Medicine
Volume9
Issue number9
DOIs
Publication statusPublished - 1 Sep 2021

Bibliographical note

Funding Information:
PS reports grants, personal fees, and non-financial support from Roche, PPM Services, and Boehringer Ingelheim, and personal fees from Galapagos, Chiesi, and Santhera Pharmaceuticals, outside of the submitted work; his wife is an employee of Novartis. CJR reports grants and personal fees from Boehringer Ingelheim and Hoffmann-La Roche, outside of the submitted work. RP reports grants from National Institutes of Health (NIH), during the conduct of the study. JO reports grants from NIH and personal fees from Genentech and Boehringer Ingelheim, outside of the submitted work. MS reports grants from NIH, during the conduct of the study; and personal fees from Boehringer Ingelheim, Orinove, and Roche, outside of the submitted work. NS reports personal fees from Roche and Boehringer Ingelheim, and grants from Chiesi, outside of the submitted work. CV reports personal fees from Boehringer Ingelheim, Hoffmann-La Roche, Galapagos, and BMS, outside of the submitted work. SG reports personal fees from Boehringer Ingelheim and Hoffmann-La Roche, outside of the submitted work. MW reports grants, speaker fees, and consultancy fees from Boehringer Ingelheim and Hoffman la Roche, consultancy fees from Galapagos and Respivant, data safety monitoring fees from Savara, and speaker fees from Novartis, outside of the submitted work; all grants and fees were paid to her institution. VC reports personal fees and non-financial support from Actelion and Roche/Promedior, grants, personal fees, and non-financial support from Boehringer Ingelheim, and personal fees from Bayer/MSD, Novartis, Sanofi, Celgene, Galapagos, Galecto, Shionogi, AstraZeneca, and Fibrogen, outside of the submitted work. SJ declares no competing interests.

Publisher Copyright:
© 2021 Elsevier Ltd

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