Cystic fibrosis and airway submucosal glands

Abstract

The chronic pulmonary infections and inflammation associated with cystic fibrosis (CF) are responsible for almost all the morbidity and mortality of this disease. Our understanding of the mechanisms that underlie the very early stages of CF lung disease, that result directly from mutations in the CF gene, is relatively poor. However, the demonstration that the predominant sites of expression of the CF gene in normal lungs are the submucosal glands, together with the histological observations showing that hyperplasia of these glands and mucin occlusion of the gland ducts are the earliest signs of disease in the CF lung, suggest that malfunction of the submucosal glands may be an important factor contributing to the early pathophysiology of CF lung disease. This review describes the function of submucosal glands in normal lungs, and the way in which their function may be disrupted in CF and may thus contribute to the early stages of CF lung disease.