Dendritic cells (DCs) are potent antigen-presenting cells (APCs); they are
considered to be the most important APC in the lung. Recently, the number o
f DCs in the large airways was demonstrated to increase in patients with at
opic asthma, leading to the concept that DCs play an important role in airw
ay inflammation. However, little is known about the distribution of lung DC
s in the small airways under other pathological conditions. The aim of the
present study was to examine the distribution of DCs in the bronchiolar tis
sues in patients with diffuse panbronchiolitis (DPB), which is a chronic in
flammatory disorder of the airways histologically characterized by peribron
chiolitis. We investigated the distribution of DCs in the bronchiolar tissu
es of the lungs in 11 patients with DPB and 7 control subjects with normal
lungs using immunohistochemical methods. Marked increases in the number of
CD1a(+), CD1c(+) and CD83(-) DCs were found in both the bronchiolar epithel
ium and submucosal tissues of patients with DPB, compared with control subj
ects with normal lungs. The most striking increase occurred in the number o
f DCs expressing CD83, a marker of mature DCs in the submucosal tissues of
patients with DPB. The increases of these positive cells in patients with D
PB were more marked in the submucosal tissues than in the epithelium. The b
ronchiolar epithelial cells in patients with DPB strongly expressed GM-CSF
protein, which is an important cytokine for the differentiation and functio
n of DCs, suggesting that the increased local production of CM-CSF may be r
esponsible for the accumulation and differentiation of DCs in the bronchiol
ar tissues of patients with DPB. These results suggest that increased DCs i
n the bronchiolar tissues, together with their phenotypical maturation, may
play an important role in the mucosal immune response in patients with DPB
through their potent antigen-presenting function.