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The Vasculature in Pulmonary Fibrosis

  • Lung Injury & Fibrosis (CL Wilson, Section Editor)
  • Published:
Current Tissue Microenvironment Reports Aims and scope Submit manuscript

A Correction to this article was published on 28 September 2022

This article has been updated

Abstract

Purpose of review

The current paradigm of idiopathic pulmonary fibrosis (IPF) pathogenesis involves recurrent injury to a sensitive alveolar epithelium followed by impaired repair responses marked by fibroblast activation and deposition of extracellular matrix. Multiple cell types are involved in this response with potential roles suggested by advances in single-cell RNA sequencing and lung developmental biology. Notably, recent work has better characterized the cell types present in the pulmonary endothelium and identified vascular changes in patients with IPF.

Recent findings

Lung tissue from patients with IPF has been examined at single-cell resolution, revealing reductions in lung capillary cells and expansion of a population of vascular cells expressing markers associated with bronchial endothelium. In addition, pre-clinical models have demonstrated a fundamental role for aging and vascular permeability in the development of pulmonary fibrosis.

Summary

Mounting evidence suggests that the endothelium undergoes changes in the context of fibrosis, and these changes may contribute to the development and/or progression of pulmonary fibrosis. Additional studies will be needed to further define the functional role of these vascular changes.

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Authors and Affiliations

  1. University of Louisville School of Medicine, Louisville, KY, USA

    Eric Engelbrecht

  2. Division of Pulmonary and Critical Care Medicine, Massachusetts General Hospital, Harvard Medical School, 149 13th Street, Charlestown, Boston, MA, 02129, USA

    Tristan Kooistra & Rachel S. Knipe

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  1. Eric Engelbrecht
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  2. Tristan Kooistra
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  3. Rachel S. Knipe
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Correspondence to Rachel S. Knipe.

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E.G.E. does not have any COI or competing interests. T.G.K. has been supported by NIH T32HL116275 without other COI or competing interests. R.S.K is supported by grants from the NIH (K08HL140175 and 1R33HL154125-01) and a Boehringer Ingelheim Discovery ILD Award. She is also supported by the Pulmonary Drug Discovery (PDD) Lab, a collaboration between Massachusetts General Hospital, Brigham and Women’s Hospital and Bayer Pharmaceutical. She has no other COI or competing interest.

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The original online version of this article was revised: Modifications have been made in Figure 1 caption. Full information regarding the corrections made can be found in the erratum/correction for this article.

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Engelbrecht, E., Kooistra, T. & Knipe, R.S. The Vasculature in Pulmonary Fibrosis. Curr. Tissue Microenviron. Rep. 3, 83–97 (2022). https://doi.org/10.1007/s43152-022-00040-9

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