Progress in Model Systems of Cystic Fibrosis Mucosal Inflammation to Understand Aberrant Neutrophil Activity

In response to repeated lung infection in cystic fibrosis (CF), white blood cells called neutrophils migrate into the lungs. Once in the lungs, neutrophils release compounds called enzymes that damage the airways over time. As a result, neutrophil activity is a good indicator of lung disease progression in CF patients. Neutrophils have an important role in normal clearing of infection; however, we need to study how to minimize their damaging effects. What factors trigger neutrophils to release damaging enzymes is still being investigated, especially in early life CF when signs of this neutrophil behaviour are first observed. This review examines the latest findings on neutrophils in early CF lungs and different ways they may contribute to lung disease. We also summarize the current and emerging tools we have in the laboratory to understand how lung tissue and neutrophils respond to infection.

Authors: Daniel R. Laucirica, Luke W. Garratt and Anthony Kicic