Abstract

Background: Chronic airway obstruction is characteristic of cystic fibrosis (CF) but there are few studies of airway smooth muscle remodelling in CF.

Methods: Airway smooth muscle content and mean airway smooth muscle cell size were measured by applying design-based stereology to bronchoscopic biopsy specimens obtained from seven subjects with CF and 15 healthy controls.

Results: The smooth muscle content increased by 63% in subjects with CF (mean (SD) 0.173 (0.08) v 0.106 (0.042) mm³ smooth muscle/mm³ submucosa, mean difference −0.067; 95% CI −0.12 to −0.013, p = 0.017) but there was no increase in mean cell size (2705 (351) v 2654 (757) μm³, mean difference −51; 95% CI −687 to 585, p = 0.87).

Conclusions: These findings indicate hyperplasia of airway smooth muscle cells without hypertrophy and suggest that accumulation of airway smooth muscle cells may contribute to airway narrowing and bronchial hyperresponsiveness in CF.