In patients with cryptogenic fibrosing alveolitis (CFA), subpleural la
rge cysts are sometimes seen within honeycombing on computed tomograph
y (CT). These cysts may be interpreted as bullae and the coexistence o
f emphysema with CFA might be assumed. We evaluated whether cystic spa
ces in honeycombing become larger in patients with CFA on serial CT sc
ans. CT scans were obtained in 16 patients with CFA, who had undergone
two CT examinations (6 to 43 months, mean 25.1 months apart), were re
viewed focusing on the changes in size of the cystic spaces in honeyco
mbing. The mechanism of enlargement of cystic spaces was investigated
using microscopic serial sections of autopsied specimens. In 15 of 16
patients, the cystic spaces in honeycombing were larger on the second
examination. Histologically, stenosis of bronchioles and slit-like str
uctures between cysts and bronchioles were detected in addition to alv
eolar septal dissolution and bronchiolectasis. Both the bronchiolar st
enosis and the slit-like gaps have the potential to work as check-valv
es and increase the volume of cystic spaces. Therefore, in the evaluat
ion of CFA, large cysts should be interpreted with caution to avoid mi
sinterpretation of the cysts as bullae associated with emphysema. From
the results of this correlative study, we suggest that a check-valve
effect is an important mechanism in the formation of large cysts in ho
neycomb lung.