Table 1

Studies that have used endobronchial biopsy to investigate airway remodelling in cystic fibrosis (CF)

AuthorYearCF subjectsStructures studiedMain findings
Gilljam181987Adults (n=7)EpitheliumLoss of microvilli, otherwise no abnormalities of the epithelium
Durieu191998Children and adults, age 10–30 years (n=6)RBM, subepitheliumThinning of the basal lamina, subepithelial fibrosis, degradation of the extracellular matrix
Wojnarowski201999Children, age 6–17 years (n=16)Epithelium, subepitheliumLymphocytic infiltrate in stable disease, neutrophilic infiltrate in exacerbation; high expression of TGF-β1 and IFN-γ associated with mild disease
Hauber212004Adults (n=10)EpitheliumIncreased PAS staining in the epithelium; increased expression of IL-9, IL-9R and hCLCA1 in mucus-producing cells
Hays222005Adults (n=7)Smooth muscleHyperplasia without hypertrophy of airway smooth muscle
Hays232006Adults (n=7)Goblet cells, mucus-secreting glandsIncreased goblet cell size but normal number; hypertrophy of submucosal glands, with normal proportion of mucous and serous acini
Hilliard242007Children, age 0–17 years (n=43)RBMRBM thickening related to TGF-β1 in BAL but independent of other markers of inflammation
Regamey252008Children, age 6–17 years (n=27)Smooth muscleIncrease in airway smooth muscle (both hyperplasia and hypertrophy)
  • hCLCA, human calcium-activated chloride channel; IFN, interferon; IL, interleukin; PAS, periodic acid-Schiff; RBM, reticular basement membrane; TGF, transforming growth factor.