A mast cell-ILC2-Th9 pathway promotes lung inflammation in cystic fibrosis

Nat Commun. 2017 Jan 16:8:14017. doi: 10.1038/ncomms14017.

Abstract

T helper 9 (Th9) cells contribute to lung inflammation and allergy as sources of interleukin-9 (IL-9). However, the mechanisms by which IL-9/Th9 mediate immunopathology in the lung are unknown. Here we report an IL-9-driven positive feedback loop that reinforces allergic inflammation. We show that IL-9 increases IL-2 production by mast cells, which leads to expansion of CD25+ type 2 innate lymphoid cells (ILC2) and subsequent activation of Th9 cells. Blocking IL-9 or inhibiting CD117 (c-Kit) signalling counteracts the pathogenic effect of the described IL-9-mast cell-IL-2 signalling axis. Overproduction of IL-9 is observed in expectorates from cystic fibrosis (CF) patients, and a sex-specific variant of IL-9 is predictive of allergic reactions in female patients. Our results suggest that blocking IL-9 may be a therapeutic strategy to ameliorate inflammation associated with microbial colonization in the lung, and offers a plausible explanation for gender differences in clinical outcomes of patients with CF.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adolescent
  • Adult
  • Animals
  • Child
  • Child, Preschool
  • Cystic Fibrosis / genetics
  • Cystic Fibrosis / immunology*
  • Female
  • Humans
  • Immunity, Innate
  • Infant
  • Interleukin-9 / immunology
  • Lung / immunology
  • Lymphocytes / immunology*
  • Male
  • Mast Cells / immunology*
  • Mice
  • Mice, Inbred C57BL
  • Middle Aged
  • Proto-Oncogene Proteins c-kit / immunology
  • T-Lymphocytes, Helper-Inducer / immunology*
  • Young Adult

Substances

  • Interleukin-9
  • Proto-Oncogene Proteins c-kit