Time for a change: is idiopathic pulmonary fibrosis still idiopathic and only fibrotic?

doi:10.1016/S2213-2600(18)30007-9

Review

. 2018 Feb;6(2):154-160.

doi: 10.1016/S2213-2600(18)30007-9.

Time for a change: is idiopathic pulmonary fibrosis still idiopathic and only fibrotic?

Affiliations

¹ Division of Pulmonary and Critical Care Medicine, Department of Medicine, University of California, San Francisco, CA, USA. Electronic address: paul.wolters@ucsf.edu.
² Department of Medicine, Vanderbilt University, Nashville, TN, USA.
³ Division of Pulmonary Sciences and Critical Care Medicine, Department of Medicine, University of Colorado Denver, Aurora, CO, USA.
⁴ Section of Pulmonary, Critical Care, and Sleep Medicine, Yale University School of Medicine, New Haven, CT, USA.
⁵ Division of Respiratory Medicine, University of Nottingham, Nottingham University Hospitals, Nottingham, UK.
⁶ Fibrosis Research Group, National Heart and Lung Institute, Imperial College London, London, UK.
⁷ Department of Pneumology, Unit of Interstitial Lung Diseases, University Hospital of Bellvitge Institute for Biomedical Research (IDIBELL), Barcelona, Spain.
⁸ Department of Medicine, Cedars-Sinai Medical Center, Los Angeles, CA, USA.
⁹ Center for Interstitial Lung Disease, University of Washington, Seattle, WA, USA.
¹⁰ Division of Pulmonary Medicine, A Gemelli University Hospital, Catholic University of the Sacred Heart, Rome, Italy.
¹¹ Instituto Nacional de Enfermedades Respiratorias Ismael Cosío Villegas, Mexico City, Mexico.
¹² Department of Pulmonary Medicine, Unit for Interstitial Lung diseases. University Hospitals Leuven, Leuven, Belgium.

PMID: 29413083
PMCID: PMC5903445
DOI: 10.1016/S2213-2600(18)30007-9

Review

Time for a change: is idiopathic pulmonary fibrosis still idiopathic and only fibrotic?

Paul J Wolters et al. Lancet Respir Med. 2018 Feb.

. 2018 Feb;6(2):154-160.

doi: 10.1016/S2213-2600(18)30007-9.

Authors

Affiliations

¹ Division of Pulmonary and Critical Care Medicine, Department of Medicine, University of California, San Francisco, CA, USA. Electronic address: paul.wolters@ucsf.edu.
² Department of Medicine, Vanderbilt University, Nashville, TN, USA.
³ Division of Pulmonary Sciences and Critical Care Medicine, Department of Medicine, University of Colorado Denver, Aurora, CO, USA.
⁴ Section of Pulmonary, Critical Care, and Sleep Medicine, Yale University School of Medicine, New Haven, CT, USA.
⁵ Division of Respiratory Medicine, University of Nottingham, Nottingham University Hospitals, Nottingham, UK.
⁶ Fibrosis Research Group, National Heart and Lung Institute, Imperial College London, London, UK.
⁷ Department of Pneumology, Unit of Interstitial Lung Diseases, University Hospital of Bellvitge Institute for Biomedical Research (IDIBELL), Barcelona, Spain.
⁸ Department of Medicine, Cedars-Sinai Medical Center, Los Angeles, CA, USA.
⁹ Center for Interstitial Lung Disease, University of Washington, Seattle, WA, USA.
¹⁰ Division of Pulmonary Medicine, A Gemelli University Hospital, Catholic University of the Sacred Heart, Rome, Italy.
¹¹ Instituto Nacional de Enfermedades Respiratorias Ismael Cosío Villegas, Mexico City, Mexico.
¹² Department of Pulmonary Medicine, Unit for Interstitial Lung diseases. University Hospitals Leuven, Leuven, Belgium.

PMID: 29413083
PMCID: PMC5903445
DOI: 10.1016/S2213-2600(18)30007-9

Abstract

Idiopathic pulmonary fibrosis (IPF) is a progressive, irreversible, and typically fatal lung disease characterised by subpleural fibrosis, subepithelial fibroblast foci, and microscopic honeycombing. Although understanding of the pathogenic mechanisms continues to evolve, evidence indicates that distal airway and alveolar epithelial cells are central drivers of the disease. In this Viewpoint, we review the history of naming and classifications used to define the disease now referred to as IPF, in the context of understanding the clinical presentation, causes, and pathogenesis of the disease. We aim to generate discussion on whether, given the substantial progress made in understanding the clinical, genetic, cellular, and molecular mechanisms involved in the development of IPF, a change of name should be considered. To initiate this discussion, we offer new suggestions to update the name of this disease and new approaches to classify all forms of pulmonary fibrosis.

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Conflict of interest statement

Declaration of interests

The other authors declare no competing interests.

Figures

**Figure. Three-stage description of the pathogenesis of idiopathic pulmonary fibrosis**
In the predisposition stage, recurrent environmental insults lead, in genetically predisposed individuals, to increased turnover of alveolar type II cells, ER-stress-mediated activation of UPR, apoptosis, and progressive telomere attrition. In the activation stage, accumulation of a lifetime insults leads to pathological alterations of the lung epithelium, such as senescence reprogramming, and release of profibrotic mediators (eg, TGFβ, Wnts, and PDGFβ) by the alveolar epithelium. These mediators, either directly or indirectly via leucocytes, activate fibroblasts to deposit pathological matrix. In the progression stage, the pathological matrix promotes additional differentiation of fibroblasts to myofibroblasts, which deposit more matrix and further activate fibroblasts in a feed-forward loop of lung remodelling. ER=endoplasmic reticulum. UPR=unfolded protein response. PDGF=platelet-derived growth factor. TGF=transforming growth factor.

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Comment in

Idiopathic pulmonary fibrosis: idiopathic no more?
Thannickal VJ, Wells A, Kolb M. Thannickal VJ, et al. Lancet Respir Med. 2018 Feb;6(2):84-85. doi: 10.1016/S2213-2600(18)30022-5. Lancet Respir Med. 2018. PMID: 29413084 No abstract available.

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References

1. Jones MG, Fabre A, Schneider P, et al. Three-dimensional characterization of fibroblast foci in idiopathic pulmonary fibrosis. JCI Insight. 2016;1:e86375. - PMC - PubMed
1. King TE, Jr, Pardo A, Selman M. Idiopathic pulmonary fibrosis. Lancet. 2011;378:1949–61. - PubMed
1. Liebow AA. Definition and classification of interstitial pneumonias in human pathology. Prog Respir Res. 1975;8:1–31.
1. Wolters PJ, Collard HR, Jones KD. Pathogenesis of idiopathic pulmonary fibrosis. Annu Rev Pathol. 2014;9:157–79. - PMC - PubMed
1. Selman M, Pardo A. Revealing the pathogenic and aging-related mechanisms of the enigmatic idiopathic pulmonary fibrosis an integral model. Am J Respir Crit Care Med. 2014;189:1161–72. - PubMed

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[1] Jones MG, Fabre A, Schneider P, et al. Three-dimensional characterization of fibroblast foci in idiopathic pulmonary fibrosis. JCI Insight. 2016;1:e86375. - PMC - PubMed

[2] Jones MG, Fabre A, Schneider P, et al. Three-dimensional characterization of fibroblast foci in idiopathic pulmonary fibrosis. JCI Insight. 2016;1:e86375. - PMC - PubMed

[3] King TE, Jr, Pardo A, Selman M. Idiopathic pulmonary fibrosis. Lancet. 2011;378:1949–61. - PubMed

[4] King TE, Jr, Pardo A, Selman M. Idiopathic pulmonary fibrosis. Lancet. 2011;378:1949–61. - PubMed

[5] Liebow AA. Definition and classification of interstitial pneumonias in human pathology. Prog Respir Res. 1975;8:1–31.

[6] Liebow AA. Definition and classification of interstitial pneumonias in human pathology. Prog Respir Res. 1975;8:1–31.

[7] Wolters PJ, Collard HR, Jones KD. Pathogenesis of idiopathic pulmonary fibrosis. Annu Rev Pathol. 2014;9:157–79. - PMC - PubMed

[8] Wolters PJ, Collard HR, Jones KD. Pathogenesis of idiopathic pulmonary fibrosis. Annu Rev Pathol. 2014;9:157–79. - PMC - PubMed

[9] Selman M, Pardo A. Revealing the pathogenic and aging-related mechanisms of the enigmatic idiopathic pulmonary fibrosis an integral model. Am J Respir Crit Care Med. 2014;189:1161–72. - PubMed

[10] Selman M, Pardo A. Revealing the pathogenic and aging-related mechanisms of the enigmatic idiopathic pulmonary fibrosis an integral model. Am J Respir Crit Care Med. 2014;189:1161–72. - PubMed

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Time for a change: is idiopathic pulmonary fibrosis still idiopathic and only fibrotic?

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Time for a change: is idiopathic pulmonary fibrosis still idiopathic and only fibrotic?

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