Identification of progressive pulmonary fibrosis: consensus findings from a modified Delphi study

Abstract Background We sought consensus among practising respiratory physicians on the prediction, identification and monitoring of progression in patients with fibrosing interstitial lung disease (ILD) using a modified Delphi process. Methods Following a literature review, statements on the predict...

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Main Authors: Athol U. Wells, Simon L. F. Walsh, Ayodeji Adegunsoye, Vincent Cottin, Sonye K. Danoff, Anand Devaraj, Kevin R. Flaherty, Peter M. George, Kerri A. Johannson, Martin Kolb, Yasuhiro Kondoh, Andrew G. Nicholson, Sara Tomassetti, Elizabeth R. Volkmann, Kevin K. Brown
Format: Article
Language:English
Published: BMC 2024-12-01
Series:Respiratory Research
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Online Access:https://doi.org/10.1186/s12931-024-03070-z
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author Athol U. Wells
Simon L. F. Walsh
Ayodeji Adegunsoye
Vincent Cottin
Sonye K. Danoff
Anand Devaraj
Kevin R. Flaherty
Peter M. George
Kerri A. Johannson
Martin Kolb
Yasuhiro Kondoh
Andrew G. Nicholson
Sara Tomassetti
Elizabeth R. Volkmann
Kevin K. Brown
author_facet Athol U. Wells
Simon L. F. Walsh
Ayodeji Adegunsoye
Vincent Cottin
Sonye K. Danoff
Anand Devaraj
Kevin R. Flaherty
Peter M. George
Kerri A. Johannson
Martin Kolb
Yasuhiro Kondoh
Andrew G. Nicholson
Sara Tomassetti
Elizabeth R. Volkmann
Kevin K. Brown
author_sort Athol U. Wells
collection DOAJ
description Abstract Background We sought consensus among practising respiratory physicians on the prediction, identification and monitoring of progression in patients with fibrosing interstitial lung disease (ILD) using a modified Delphi process. Methods Following a literature review, statements on the prediction, identification and monitoring of progression of ILD were developed by a panel of physicians with specialist expertise. Practising respiratory physicians were sent a survey asking them to indicate their level of agreement with these statements on a binary scale or 7-point Likert scale (− 3 to 3), or to select answers from a list. Consensus was considered to be achieved if ≥ 70% of respondents selected the same answer, or, for responses on a Likert scale, the median score was ≤ –2 (disagree/not important) or ≥ 2 (agree/important) with an interquartile range ≤ 1. There were three rounds of the survey. Results Surveys 1, 2 and 3 were completed by 207, 131 and 94 physicians, respectively, between March 2022 and July 2023. Decline in forced vital capacity (FVC), decline in diffusing capacity of the lungs for carbon monoxide, and increased fibrosis on high-resolution computed tomography (HRCT) were ranked as the most important endpoints for determining progression. Consensus was reached that progression on HRCT or a decline in FVC ≥ 10% from baseline is sufficient to determine progression, and that small declines in multiple endpoints indicates progression. Consensus was reached that a histological pattern of usual interstitial pneumonia (UIP) is a risk factor for progression of ILD, but that a biopsy to look for a UIP pattern should not be performed solely for prognostic reasons. Consensus was not reached on the time period over which progression should be defined. There was consensus that appropriate management of ILD depends on the type of ILD, and that ‘despite adequate management’ or ‘despite usual management’ should be included in the definition of progression. Conclusions This modified Delphi process provided consensus statements on the identification of ILD progression that were supported by a broad group of clinicians and may help to inform clinical practice until robust evidence-based guidelines are available.
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spelling doaj-art-af422ffbaddb4557a6bc556de9bd91712025-01-05T12:43:50ZengBMCRespiratory Research1465-993X2024-12-0125111310.1186/s12931-024-03070-zIdentification of progressive pulmonary fibrosis: consensus findings from a modified Delphi studyAthol U. Wells0Simon L. F. Walsh1Ayodeji Adegunsoye2Vincent Cottin3Sonye K. Danoff4Anand Devaraj5Kevin R. Flaherty6Peter M. George7Kerri A. Johannson8Martin Kolb9Yasuhiro Kondoh10Andrew G. Nicholson11Sara Tomassetti12Elizabeth R. Volkmann13Kevin K. Brown14Royal Brompton and Harefield Hospitals, Guy’s and St Thomas’ NHS Foundation TrustNational Heart and Lung Institute, Imperial College LondonUniversity of ChicagoNational Reference Center for Rare Pulmonary Diseases, Louis Pradel Hospital, Claude Bernard University Lyon 1Johns Hopkins MedicineRoyal Brompton and Harefield Hospitals, Guy’s and St Thomas’ NHS Foundation TrustUniversity of MichiganRoyal Brompton and Harefield Hospitals, Guy’s and St Thomas’ NHS Foundation TrustUniversity of CalgaryMcMaster University and St. Joseph’s HealthcareTosei General HospitalRoyal Brompton and Harefield Hospitals, Guy’s and St Thomas’ NHS Foundation TrustFlorence UniversityUniversity of California, David Geffen School of MedicineNational Jewish HealthAbstract Background We sought consensus among practising respiratory physicians on the prediction, identification and monitoring of progression in patients with fibrosing interstitial lung disease (ILD) using a modified Delphi process. Methods Following a literature review, statements on the prediction, identification and monitoring of progression of ILD were developed by a panel of physicians with specialist expertise. Practising respiratory physicians were sent a survey asking them to indicate their level of agreement with these statements on a binary scale or 7-point Likert scale (− 3 to 3), or to select answers from a list. Consensus was considered to be achieved if ≥ 70% of respondents selected the same answer, or, for responses on a Likert scale, the median score was ≤ –2 (disagree/not important) or ≥ 2 (agree/important) with an interquartile range ≤ 1. There were three rounds of the survey. Results Surveys 1, 2 and 3 were completed by 207, 131 and 94 physicians, respectively, between March 2022 and July 2023. Decline in forced vital capacity (FVC), decline in diffusing capacity of the lungs for carbon monoxide, and increased fibrosis on high-resolution computed tomography (HRCT) were ranked as the most important endpoints for determining progression. Consensus was reached that progression on HRCT or a decline in FVC ≥ 10% from baseline is sufficient to determine progression, and that small declines in multiple endpoints indicates progression. Consensus was reached that a histological pattern of usual interstitial pneumonia (UIP) is a risk factor for progression of ILD, but that a biopsy to look for a UIP pattern should not be performed solely for prognostic reasons. Consensus was not reached on the time period over which progression should be defined. There was consensus that appropriate management of ILD depends on the type of ILD, and that ‘despite adequate management’ or ‘despite usual management’ should be included in the definition of progression. Conclusions This modified Delphi process provided consensus statements on the identification of ILD progression that were supported by a broad group of clinicians and may help to inform clinical practice until robust evidence-based guidelines are available.https://doi.org/10.1186/s12931-024-03070-zDisease progressionFibrosis, pulmonaryInterstitial lung diseaseMonitoring, physiologicPulmonary function tests
spellingShingle Athol U. Wells
Simon L. F. Walsh
Ayodeji Adegunsoye
Vincent Cottin
Sonye K. Danoff
Anand Devaraj
Kevin R. Flaherty
Peter M. George
Kerri A. Johannson
Martin Kolb
Yasuhiro Kondoh
Andrew G. Nicholson
Sara Tomassetti
Elizabeth R. Volkmann
Kevin K. Brown
Identification of progressive pulmonary fibrosis: consensus findings from a modified Delphi study
Respiratory Research
Disease progression
Fibrosis, pulmonary
Interstitial lung disease
Monitoring, physiologic
Pulmonary function tests
title Identification of progressive pulmonary fibrosis: consensus findings from a modified Delphi study
title_full Identification of progressive pulmonary fibrosis: consensus findings from a modified Delphi study
title_fullStr Identification of progressive pulmonary fibrosis: consensus findings from a modified Delphi study
title_full_unstemmed Identification of progressive pulmonary fibrosis: consensus findings from a modified Delphi study
title_short Identification of progressive pulmonary fibrosis: consensus findings from a modified Delphi study
title_sort identification of progressive pulmonary fibrosis consensus findings from a modified delphi study
topic Disease progression
Fibrosis, pulmonary
Interstitial lung disease
Monitoring, physiologic
Pulmonary function tests
url https://doi.org/10.1186/s12931-024-03070-z
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