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Progressive pulmonary fibrosis: all roads lead to Rome (but not all at the same speed)

Vincent Cottin, Claudia Valenzuela
European Respiratory Journal 2022 60: 2201449; DOI: 10.1183/13993003.01449-2022
Vincent Cottin
1Department of Respiratory Medicine, National Reference Centre for Rare Pulmonary Diseases, Louis Pradel Hospital, Hospices Civils de Lyon, UMR 754, Claude Bernard University Lyon 1, Lyon, France
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  • For correspondence: vincent.cottin@chu-lyon.fr
Claudia Valenzuela
2ILD Unit, Department of Respiratory Medicine, Hospital universitario de la Princesa, Universitad autónoma de Madrid, Madrid, Spain
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Extract

Fibrosing interstitial lung diseases (ILDs) encompass a number of diverse conditions, overlapping in their clinical presentations, and imaging and histopathological patterns [1]. Idiopathic pulmonary fibrosis (IPF) is the prototype of fibrosing ILD, and is characterised by irreversible progressive pulmonary disease, accounting for loss of lung function, exercise intolerance and complications, especially acute exacerbation and respiratory failure leading to early mortality [2]. A significant proportion of patients with fibrosing ILDs other than IPF will develop a progressive phenotype comparable to untreated IPF [3]. Such progression can occur despite conventional treatment which, depending on the underlying condition, may include close monitoring, antigen eviction, glucocorticoids, immunosuppressive therapy and pulmonary rehabilitation. Progressive pulmonary fibrosis (PPF) [1, 4], also referred to as “progressive fibrosing ILDs” (PF-ILD) or fibrosing ILDs with a progressive phenotype [5], is characterised by a disease course similar to that of IPF, with worsening respiratory symptoms, decline in lung function and early mortality [6, 7].

Abstract

Large observational cohorts show that eventually, a majority of patients with fibrotic ILD will experience disease progression. Highest rates of progression are seen in patients with idiopathic pulmonary fibrosis and fibrotic hypersensitivity pneumonitis. https://bit.ly/3QcnVKJ

Footnotes

  • Conflict of interest: V. Cottin reports grants from Boehringer Ingelheim; consulting fees from Boehringer Ingelheim, Roche, Shionogi, RedX, Pure Tech, Celgene/BMS, AstraZeneca, CSL Behring, Sanofi, United Therapeutics and Pliant; lecture fees and travel support from Boehringer Ingelheim and Roche; data and safety monitoring board participation with Galapagos and Galecto; adjudication committee role with Fibrogen; outside the submitted work. C. Valenzuela reports consulting fees, fees for lectures and support for attending meetings from Boehringer Ingelheim and Roche; outside the submitted work.

  • Received July 19, 2022.
  • Accepted July 25, 2022.
  • Copyright ©The authors 2022. For reproduction rights and permissions contact permissions{at}ersnet.org
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Progressive pulmonary fibrosis: all roads lead to Rome (but not all at the same speed)
Vincent Cottin, Claudia Valenzuela
European Respiratory Journal Oct 2022, 60 (4) 2201449; DOI: 10.1183/13993003.01449-2022

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Progressive pulmonary fibrosis: all roads lead to Rome (but not all at the same speed)
Vincent Cottin, Claudia Valenzuela
European Respiratory Journal Oct 2022, 60 (4) 2201449; DOI: 10.1183/13993003.01449-2022
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