Serial changes of cystic air spaces in fibrosing alveolitis: a CT-pathological study

In patients with cryptogenic fibrosing alveolitis (CFA), subpleural large cysts are sometimes seen within honeycombing on computed tomography (CT). These cysts may be interpreted as bullae and the coexistence of emphysema with CFA might be assumed. We evaluated whether cystic spaces in honeycombing become larger in patients with CFA on serial CT scans. 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 reviewed focusing on the changes in size of the cystic spaces in honeycombing. 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 structures between cysts and bronchioles were detected in addition to alveolar septal dissolution and bronchiolectasis. Both the bronchiolar stenosis and the slit-like gaps have the potential to work as check-valves and increase the volume of cystic spaces. Therefore, in the evaluation of CFA, large cysts should be interpreted with caution to avoid misinterpretation 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 honeycomb lung.