Lung mast cell density defines a subpopulation of patients with idiopathic pulmonary fibrosis

Histopathology. 2012 Jul;61(1):98-106. doi: 10.1111/j.1365-2559.2012.04197.x. Epub 2012 Mar 6.

Abstract

Aims: The relationship of mast cells to the pathogenesis of lung fibrosis remains undefined despite recognition of their presence in the lungs of patients with pulmonary fibrosis. This study was performed to characterize the relationship of mast cells to fibrotic lung diseases.

Methods and results: Lung tissues from patients with idiopathic pulmonary fibrosis (IPF), chronic hypersensitivity pneumonitis (HP), systemic sclerosis (SSc)-related interstitial lung disease (ILD) and normal individuals were subjected to chymase immunostaining and the mast cell density quantified. Eosinophils were quantified by immunostaining for eosinophil peroxidase. Changes in lung function were correlated with mast cell density. Lung tissue obtained from IPF patients had a higher density of chymase-immunoreactive mast cells than that from patients with HP, SSc-related ILD or normal lungs. IPF lung tissue had a higher density of eosinophils than normal lung. There was no correlation between mast cell density and eosinophil density in IPF lung. IPF patients with high mast cell density had a slower rate of decline in forced vital capacity (FVC) than IPF patients with low mast cell density.

Conclusions: Mast cell density in IPF lungs is higher than in other fibrotic lung diseases and normal lungs. Increased mast cell density in IPF may predict slower disease progression.

Publication types

  • Research Support, N.I.H., Extramural

MeSH terms

  • Alveolitis, Extrinsic Allergic / complications
  • Alveolitis, Extrinsic Allergic / pathology*
  • Alveolitis, Extrinsic Allergic / physiopathology
  • Biomarkers / metabolism
  • Carboxylic Ester Hydrolases / metabolism
  • Cell Count
  • Chronic Disease
  • Chymases / metabolism
  • Disease Progression
  • Eosinophils / enzymology
  • Eosinophils / pathology
  • Female
  • Humans
  • Idiopathic Pulmonary Fibrosis / complications
  • Idiopathic Pulmonary Fibrosis / pathology*
  • Idiopathic Pulmonary Fibrosis / physiopathology
  • Immunoenzyme Techniques / methods
  • Lung / enzymology
  • Lung / pathology*
  • Lung / physiopathology
  • Male
  • Mast Cells / enzymology
  • Mast Cells / pathology*
  • Middle Aged
  • Respiratory Function Tests
  • Scleroderma, Systemic / complications
  • Scleroderma, Systemic / pathology*
  • Scleroderma, Systemic / physiopathology

Substances

  • Biomarkers
  • Carboxylic Ester Hydrolases
  • chloroacetate esterase
  • Chymases