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Review
. 2023 Mar 30;61(3):2103187.
doi: 10.1183/13993003.03187-2021. Print 2023 Mar.

Progressive pulmonary fibrosis: an expert group consensus statement

Sujeet K Rajan  1 , Vincent Cottin  2 , Raja Dhar  3 , Sonye Danoff  4 , Kevin R Flaherty  5 , Kevin K Brown  6 , Anant Mohan  7 , Elizabeth Renzoni  8 , Murali Mohan  9 , Zarir Udwadia  10 , Padmanabha Shenoy  11 , David Currow  12 , Anand Devraj  13 , Bhavin Jankharia  14 , Ritu Kulshrestha  15 , Steve Jones  16 , Claudia Ravaglia  17 , Silvia Quadrelli  18 , Rajam Iyer  19 , Sahajal Dhooria  20 , Martin Kolb  21   22 , Athol U Wells  23   22
Affiliations
Review

Progressive pulmonary fibrosis: an expert group consensus statement

Sujeet K Rajan et al. Eur Respir J. .

Abstract

This expert group consensus statement emphasises the need for standardising the definition of progressive fibrosing interstitial lung diseases (F-ILDs), with an accurate initial diagnosis being of paramount importance in ensuring appropriate initial management. Equally, case-by-case decisions on monitoring and management are essential, given the varying presentations of F-ILDs and the varying rates of progression. The value of diagnostic tests in risk stratification at presentation and, separately, the importance of a logical monitoring strategy, tailored to manage the risk of progression, are also stressed. The term "progressive pulmonary fibrosis" (PPF) exactly describes the entity that clinicians often face in practice. The importance of using antifibrotic therapy early in PPF (once initial management has failed to prevent progression) is increasingly supported by evidence. Artificial intelligence software for high-resolution computed tomography analysis, although an exciting tool for the future, awaits validation. Guidance is provided on pulmonary rehabilitation, oxygen and the use of non-invasive ventilation focused specifically on the needs of ILD patients with progressive disease. PPF should be differentiated from acute deterioration due to drug-induced lung toxicity or other forms of acute exacerbations. Referral criteria for a lung transplant are discussed and applied to patient needs in severe diseases where transplantation is not realistic, either due to access limitations or transplantation contraindications. In conclusion, expert group consensus guidance is provided on the diagnosis, treatment and monitoring of F-ILDs with specific focus on the recognition of PPF and the management of pulmonary fibrosis progressing despite initial management.

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Conflict of interest statement

Conflict of interest: S.K. Rajan reports grants to their institution, consulting fees and lecture fees from Cipla and Boehringer Ingelheim; speaker fees from the Indian Chest Society; and support from Cipla to attend a European Respiratory Society meeting, all in the 36 months prior to manuscript submission. V. Cottin reports an unrestricted grant to their institution from Boehringer Ingelheim; consulting fees from Boehringer Ingelheim, Roche, Galapagos, Galecto, Shionogi, Fibrogen, RedX Pharma and PureTech; payment or honoraria and support for attending meetings from Boehringer Ingelheim and Roche; and participation on a data safety monitoring board or advisory board for Roche/Promedior, Celgene/Bristol Myers Squibb and Galapagos, all in the 36 months prior to manuscript submission. R. Dhar reports external expert contracts from Cipla and Boehringer Ingelheim; speaker fees from the Indian Chest Society, Cipla and Boehringer Ingelheim; and support for attendance at a European Respiratory Society meeting from Cipla. S. Danoff reports grant funding from Bristol Myers Squibb (Myositis ILD Trial), Boehringer Ingelheim (INBUILD and INBUILD-ON Trials) and Roche/Genentech (TRAIL Trial); royalties from UpToDate; advisory board fees from Boehringer Ingelheim and Lupin Pharma; payment for presentations from the France Foundation; funding for travel to present lectures from Boehringer Ingelheim; and participation on data safety monitoring boards for Galecto and Galapagos, all in the 36 months prior to manuscript submission; as well as a role as senior medical adviser and interim CMO for the Pulmonary Fibrosis Foundation and membership of the board of directors of the American Thoracic Society. K.R. Flaherty reports research grants paid to their institution by Boehringer Ingelheim; and consulting fees paid to them by Boehringer Ingelheim, Roche/Genentech, Blade Therapeutics, Shionogi, DevPro, AstraZeneca, Pure Health, Fibrogen, Sun Pharmaceuticals, Pliant, United Therapeutics, Arrowhead, Lupin, Polarean and PureTech, all in the 36 months prior to manuscript submission. K.K. Brown reports lung fibrosis research grants from the National Heart, Lung, and Blood Institute; being an external science advisor to AbbVie, CSL Behring, Dispersol, Huitai Biomedicine, Lilly, RedX Pharma, Theravance and Translate Bio; being a DMC chair for Biogen; scientific advisory board membership for Blade Therapeutics, Boehringer Ingelheim, Bristol Myers Squibb, DevPro Biopharma, Galapagos NV, Galecto, Open Source Imaging Consortium, Pliant, Sanofi and Third Pole; DMC membership for Humanetics; and a scientific collaboration with Sekisui Medical Co., all in the 36 months prior to manuscript submission; as well as leadership or fiduciary roles for the Fleischner Society and Open Source Imaging Consortium. A. Mohan declares no competing interests. E. Renzoni declares a research contract payment and advisory board meeting payment to their institution by Boehringer Ingelheim; lecture fees paid to their institution by Boehringer Ingelheim, Roche and Chiesi; and registration and travel expenses for the American Thoracic Society conference, paid by Boehringer Ingelheim, all in the 36 months prior to manuscript submission. M. Mohan reports no competing interests. Z. Udwadia reports no competing interests. P. Shenoy reports no competing interests. D. Currow reports intellectual property payments and royalties from Mayne Pharma International Pty Ltd, a manufacturer of sustained-release morphine; and consulting fees from Helsinn Pharmaceuticals, in the 36 months prior to manuscript submission; as well as leadership or fiduciary roles for the Dust Diseases Board of New South Wales and the Board of the Cancer Institute NSW. A. Devraj reports consulting fees from Boehringer Ingelheim, Roche, Galecto, Galapagos, Brainomix and Vicore, all in the 36 months prior to manuscript submission. B. Jankharia reports payment or honoraria to their institution from Cipla India, Lupin India, Boehringer Ingelheim and German Remedies India, all in the 36 months prior to manuscript submission; as well as being a past president of the Indian Musculoskeletal Society and the Indian Radiology and Imaging Association. R. Kulshrestha reports no competing interests. S. Jones reports grants to their institution from Boehringer Ingelheim and Trevi for being Chair of Action for Pulmonary Fibrosis (APF) and President of the European Pulmonary Fibrosis Federation (EU-IPFF) (both these organisations implement projects in partnership with industry; the author is not involved in these projects and does not gain personally); reports payment or honoraria from Boehringer Ingelheim, Vicore and Roche (paid to their institution); and participation on a data safety monitoring board or advisory board as Galecto DSMB for an anti-fibrotic therapy, in the 36 months prior to manuscript submission. C. Ravaglia reports no competing interests. S. Quadrelli reports speaker fees from Boehringer Ingelheim in the 36 months prior to manuscript submission. R. Iyer reports no competing interests. S. Dhooria reports no competing interests. M. Kolb reports research funding for pre-clinical work from Boehringer Ingelheim and Pieris; research funding for a clinical project from Roche; consulting fees from Boehringer Ingelheim, Roche, Horizon, Cipla, AbbVie, Belerophon, Algernon, CSL Behring, United Therapeutics and AstraZeneca; payment or honoraria from Novartis, Boehringer Ingelheim and Roche; payment for court testimony from Roche; participation on a data safety monitoring board or advisory board for Covance and United Therapeutics; and an allowance paid to their institution from the European Respiratory Society relating to their duties as European Respiratory Journal Chief Editor, all in the 36 months prior to manuscript submission. A.U. Wells reports payments or honoraria from Boehringer Ingelheim and Roche; and participation on a data safety monitoring board or advisory board for Boehringer Ingelheim, Roche and Veracyte, all in the 36 months prior to manuscript submission.

Figures

FIGURE 1
FIGURE 1
Schematic representation of the prevalent spectrum of interstitial lung diseases (ILDs) that may be associated with “progressive pulmonary fibrosis (despite management)”. The lowest and highest prevalences across different countries are shown in brackets [14]. CTD: connective tissue disease; G/F PF: genetic and/or familial pulmonary fibrosis; HP: hypersensitivity pneumonitis; iNSIP: idiopathic non-specific interstitial pneumonia; IPAF: interstitial pneumonia with autoimmune features; IPF: idiopathic pulmonary fibrosis; uILD: unclassifiable ILD. Adapted with permission from [15].
See this image and copyright information in PMC

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