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

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Time for a change: is idiopathic pulmonary fibrosis still idiopathic and only fibrotic?
The Lancet ( IF 98.4 ) Pub Date : 2018-02-01 , DOI: 10.1016/s2213-2600(18)30007-9
Paul J Wolters ₁ , Timothy S Blackwell ₂ , Oliver Eickelberg ₃ , James E Loyd ₂ , Naftali Kaminski ₄ , Gisli Jenkins ₅ , Toby M Maher ₆ , Maria Molina-Molina ₇ , Paul W Noble ₈ , Ganesh Raghu ₉ , Luca Richeldi ₁₀ , Marvin I Schwarz ₃ , Moises Selman ₁₁ , Wim A Wuyts ₁₂ , David A Schwartz ₃

Affiliation

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

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.

中文翻译：

是时候改变了：特发性肺纤维化仍然是特发性的并且只是纤维化吗？

特发性肺纤维化 (IPF) 是一种进行性、不可逆且通常致命的肺部疾病，其特征是胸膜下纤维化、上皮下成纤维细胞病灶和微观蜂窝状。尽管对致病机制的理解不断发展，但有证据表明远端气道和肺泡上皮细胞是该疾病的主要驱动因素。在这个观点中，我们回顾了用于定义现在称为 IPF 的疾病的命名和分类的历史，以了解该疾病的临床表现、原因和发病机制。鉴于在了解 IPF 发展所涉及的临床、遗传、细胞和分子机制方面取得的实质性进展，我们旨在就是否应考虑更改名称进行讨论。为了发起这个讨论，

更新日期：2018-01-26

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