Patient Reported Experiences and Delays During the Diagnostic Pathway for Pulmonary Fibrosis: A Multinational European Survey

Iris G. van der Sar, Steve Jones, Deborah L. Clarke, Francesco Bonella, Jean Michel Fourrier, Katarzyna Lewandowska, Guadalupe Bermudo, Alexander Simidchiev, Irina R. Strambu, Marlies S. Wijsenbeek, Helen Parfrey*

*Corresponding author for this work

Research output: Contribution to journalArticleAcademicpeer-review

8 Citations (Scopus)


Introduction: Pulmonary fibrosis includes a spectrum of diseases and is incurable. There is a variation in disease course, but it is often progressive leading to increased breathlessness, impaired quality of life, and decreased life expectancy. Detection of pulmonary fibrosis is challenging, which contributes to considerable delays in diagnosis and treatment. More knowledge about the diagnostic journey from patients' perspective is needed to improve the diagnostic pathway. The aims of this study were to evaluate the time to diagnosis of pulmonary fibrosis, identify potential reasons for delays, and document patients emotions. Methods: Members of European patient organisations, with a self-reported diagnosis of pulmonary fibrosis, were invited to participate in an online survey. The survey assessed the diagnostic pathway retrospectively, focusing on four stages: (1) time from initial symptoms to first appointment in primary care; (2) time to hospital referral; (3) time to first hospital appointment; (4) time to final diagnosis. It comprised open-ended and closed questions focusing on time to diagnosis, factors contributing to delays, diagnostic tests, patient emotions, and information provision. Results: Two hundred and seventy three participants (214 idiopathic pulmonary fibrosis, 28 sarcoidosis, 31 other) from 13 countries responded. Forty percent of individuals took ≥1 year to receive a final diagnosis. Greatest delays were reported in stage 1, with only 50.2% making an appointment within 3 months. For stage 2, 73.3% reported a hospital referral within three primary care visits. However, 9.9% reported six or more visits. After referral, 76.9% of patients were assessed by a specialist within 3 months (stage 3) and 62.6% received a final diagnosis within 3 months of their first hospital visit (stage 4). Emotions during the journey were overall negative. A major need for more information and support during and after the diagnostic process was identified. Conclusion: The time to diagnose pulmonary fibrosis varies widely across Europe. Delays occur at each stage of the diagnostic pathway. Raising awareness about pulmonary fibrosis amongst the general population and healthcare workers is essential to shorten the time to diagnosis. Furthermore, there remains a need to provide patients with sufficient information and support at all stages of their diagnostic journey.

Original languageEnglish
Article number711194
JournalFrontiers in Medicine
Publication statusPublished - 4 Aug 2021

Bibliographical note

Funding Information:
The authors wish to thank W. M. Bramer from the Erasmus Medical Center Medical Library for developing the search strategies. The authors also wish to thank all patients and their carers for participating in the survey. Funding. Galapagos financially supported the survey, paid the open access publication fees, and had direct involvement in developing the questionnaire and report in collaboration with the EU-IPFF.

Publisher Copyright:
© Copyright © 2021 van der Sar, Jones, Clarke, Bonella, Fourrier, Lewandowska, Bermudo, Simidchiev, Strambu, Wijsenbeek and Parfrey.


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