Ultrastructural evidence of alveolar epithelial injury in idiopathic bronchiolitis obliterans-organizing pneumonia

Abstract

The ultrastructural features of idiopathic bronchiolitis obliterans-organizing pneumonia (BOOP) were studied in 9 patients. As expected, the characteristic air space fibrosis was composed of spindled fibroblasts and myofibroblasts arranged concentrically within an electron-lucent stroma. In 6 patients there was evidence of incorporation of air space fibrosis into the interstitium. A surprising finding in all patients was the presence of extensive epithelial damage involving peribronchiolar alveolar septa. Necrosis and sloughing of alveolar lining cells resulted in denuding of epithelial basal laminae. Complex infoldings and deep invaginations of the denuded basal laminae into alveolar septa were common. These ultrastructural changes involving the interstitium are similar to those occurring in the interstitial pneumonias, and suggest that BOOP also results from acute epithelial injury. The different clinical manifestations and prognosis of these entities may relate to the peribronchiolar localization of the epithelial damage in BOOP compared with more diffuse involvement of distal lung in the interstitial pneumonias.