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Cystic Fibrosis Lung Immunity: The Role of the Macrophage

Emanuela M Bruscia1, Tracey L Bonfield

  • 1Section of Respiratory Medicine, Department of Pediatrics, Yale University School of Medicine, New Haven, Conn., USA.

Journal of Innate Immunity
|June 24, 2016
PubMed

Insights

Macrophages (MΦs) are crucial for lung homeostasis in cystic fibrosis (CF). This review explores their multifaceted role in CF pathophysiology, highlighting inflammation and infection resolution challenges.

Area of Science:

  • Immunology
  • Pulmonology
  • Genetics

Background:

  • Cystic Fibrosis (CF) pathophysiology involves chronic inflammation and impaired infection resolution.
  • Macrophages (MΦs) are key immune cells regulating lung homeostasis and immune responses.
  • CF lung environment and CFTR dysfunction significantly alter MΦ function.

Purpose of the Study:

  • To review the literature on macrophage contributions to lung homeostasis in CF.
  • To explore the multifaceted roles of MΦs in CF pathophysiology.
  • To discuss the impact of CFTR dysfunction and modifier genes on MΦ phenotype.

Main Methods:

  • Literature review of existing research on macrophages in CF.
  • Analysis of studies examining MΦ function in the context of CF pathophysiology.
  • Synthesis of diverse perspectives on MΦ roles in CF lung environment.

Main Results:

  • Macrophages play a central role in both innate and adaptive immunity within the CF lung.
  • CF-specific environmental cues and CFTR dysfunction lead to altered MΦ activity.
  • Epigenetic and transcriptional changes in MΦs are influenced by modifier genes.

Conclusions:

  • Macrophages are critical immune guardians in the CF lung, influencing disease progression.
  • Understanding MΦ dynamics is essential for developing targeted CF therapies.
  • Further research into MΦ modulation may offer new therapeutic strategies for CF.

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