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The Role of Mast Cells in the Pathophysiology of Pulmonary Fibrosis

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Fibrosis in Disease

Part of the book series: Molecular and Translational Medicine ((MOLEMED))

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Abstract

Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive, and fatal disease of unknown cause. It is characterized by an accumulation of fibroblasts and myofibroblasts and extensive deposition of extracellular matrix (ECM) proteins. The microenvironment of the fibrotic ECM is characterized by altered biomechanical (stiffness) as well as altered biochemical (growth factors) properties. The ECM can also affect the behavior of multiple lung cell types, including mesenchymal cells, macrophages, and mast cells. Mast cells have been seen to accumulate within the fibrotic regions of lungs with IPF. Furthermore, in both IPF and experimental pulmonary fibrosis models, correlations between the number of mast cells and fibrosis have been reported. Mast cells contain many profibrotic mediators, such as tryptase, chymase, histamine, and TGF-β1. These mediators have been seen to activate fibroblasts and myofibroblasts. More recent studies have highlighted the role of fibrotic ECM in influencing mast cell characteristic and function. However, mast cells have not been investigated as extensively as other cells, and their role in the progression of pulmonary fibrosis has not been pursued. In this chapter, we will discuss mast cells and their pathophysiological role in pulmonary fibrosis, including IPF, and propose a new possible target for the IPF treatment.

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Authors and Affiliations

  1. Firestone Institute for Respiratory Health, St. Joseph’s Healthcare and Department of Medicine, McMaster University, Hamilton, ON, Canada

    Chiko Shimbori, Chandak Upagupta, Paul Forsythe & Martin Kolb

  2. Department of Medicine, Farncombe Family Digestive Health Research Institute, McMaster University, Hamilton, ON, Canada

    Chiko Shimbori

  3. McMaster Brain-Body Institute, St. Joseph’s Healthcare Hamilton, Hamilton, ON, Canada

    Paul Forsythe

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  1. Chiko Shimbori
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  2. Chandak Upagupta
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Correspondence to Martin Kolb .

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  1. Department of Pathology and Laboratory Medicine, Krannert Institute of Cardiology, Indiana University School of Medicine, Indiana Center for Musculoskeletal Health, Indianapolis, IN, USA

    Monte S. Willis

  2. Department of Health Promotion and Development, McGowan Institute for Regenerative Medicine, University of Pittsburgh School of Nursing, Pittsburgh, PA, USA

    Cecelia C. Yates

  3. Department of Pharmacology and Department of Pathology and Lab Medicine, McAllister Heart Institute, The University of North Carolina at Chapel Hill, Chapel Hill, NC, USA

    Jonathan C. Schisler

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Shimbori, C., Upagupta, C., Forsythe, P., Kolb, M. (2019). The Role of Mast Cells in the Pathophysiology of Pulmonary Fibrosis. In: Willis, M., Yates, C., Schisler, J. (eds) Fibrosis in Disease . Molecular and Translational Medicine. Humana Press, Cham. https://doi.org/10.1007/978-3-319-98143-7_6

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