The Role of Mast Cells in the Pathophysiology of Pulmonary Fibrosis

  • Chiko Shimbori
  • Chandak Upagupta
  • Paul Forsythe
  • Martin KolbEmail author
Part of the Molecular and Translational Medicine book series (MOLEMED)


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.


Mast cell Extracellular matrix (ECM) Fibrosis Fibroblasts Myofibroblasts Heterogeneity Chymase Tryptase TGF-β 


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Copyright information

© Springer Nature Switzerland AG 2019

Authors and Affiliations

  • Chiko Shimbori
    • 1
    • 2
  • Chandak Upagupta
    • 1
  • Paul Forsythe
    • 1
    • 3
  • Martin Kolb
    • 1
    Email author
  1. 1.Firestone Institute for Respiratory Health, St. Joseph’s Healthcare and Department of MedicineMcMaster UniversityHamiltonCanada
  2. 2.Department of Medicine, Farncombe Family Digestive Health Research InstituteMcMaster UniversityHamiltonCanada
  3. 3.McMaster Brain-Body InstituteSt. Joseph’s Healthcare HamiltonHamiltonCanada

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