ABSTRACT
Although the pathogenesis of idiopathic pulmonary fibrosis/usual interstitial pneumonia (IPF/UIP) is mainly centered on the progressive loss of alveolar parenchyma with eventual interstitial remodeling and with pulmonary function tests which mainly reveal restrictive impairment, some pathological features suggest that more proximal airway structures, and in particular the respiratory bronchioles, can also be involved in this disease. In this chapter, we will review the morphological and molecular features that characterize conductive airway abnormalities in IPF/UIP, and will describe recent data obtained by our group also showing how bronchiolar epithelium may represent a crucial target of injury and abnormal regeneration, thus contributing significantly to the pathogenesis of this disease. Our recent data are mainly based on the demonstration of the peculiar molecular features of UIP samples analyzed in situ by immunohistochemistry, using an extended panel of antibodies. This approach can help in deciphering the
molecular complexity of the pathogenesis of IPF/UIP, by reconciling the data provided by molecular and cellular biology with those obtained by traditional histology.
