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. 2017 Jun;5(2):101-110.
doi: 10.1007/s40139-017-0134-x. Epub 2017 May 2.

Heterogeneity of Fibroblasts and Myofibroblasts in Pulmonary Fibrosis

David M Habiel  1 , Cory M Hogaboam  1
Affiliations

Heterogeneity of Fibroblasts and Myofibroblasts in Pulmonary Fibrosis

David M Habiel et al. Curr Pathobiol Rep. 2017 Jun.

Abstract

Purpose of review: Idiopathic Pulmonary Fibrosis (IPF) is the most common form of interstitial lung diseases of unknown eathiopathogenesis, mean survival of 3-5 years and limited therapeutics. Characterized by a loss of alveolar type II epithelial cells and aberrant activation of stromal cells, considerable effort was undertaken to characterize the origin and activation mechanisms of fibroblasts and myofibroblasts in IPF lungs. In this review, the origin and contribution of fibroblast and myofibroblasts in lung fibrosis will be summarized.

Recent findings: Lineage tracing experiments suggested that interstitial lung fibroblasts and lipofibroblasts, pericytes and mesothelial cells differentiate into myofibroblasts. However, epithelial and bone marrow derived cells may give rise to collagen expressing fibroblasts but do not differentiate into myofibroblasts.

Summary: There is great heterogeneity in fibroblasts and myofibroblasts in fibrotic lungs. Further, there is evidence for the expansion of pericyte derived myofibroblasts and loss of lipofibroblasts and lipofibroblast derived myofibroblasts in IPF.

Keywords: Fibroblast progenitors; Idiopathic Pulmonary Fibrosis; Lung Fibroblasts; Lung Fibrosis; Mesenchymal progenitors; fibroblast heterogeneity.

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Conflict of interest statement

Conflict of Interest: David Habiel and Cory Hogaboam declare that they have no conflict of interest.

Figures

Figure 1
Figure 1
Mesodermal development in the lung and heterogeneity of fibroblasts and myofibroblasts in fibrotic lungs. Depicted is a simplified summary of mesodermal development in normal lungs (left) and potential lineages leading to the generation of fibroblasts and myofibroblasts in remodeled lungs (right).
See this image and copyright information in PMC

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