Despite improvements in the diagnostic approach to idiopathic pulmonary fibrosis (IPF) in
recent years, the pathogenesis remains poorly understood. IPF is a clinical, physiologic,
radiographic, and pathologic entity that has been difficult to characterize as a homogenous
''disease''[1]. The pathologic description of IPF originally was delineated by Leibow [81] and
colleagues and termed ''usual interstitial pneumonia''(UIP). Of the fibrosing lung diseases
cases he reviewed, this was the most common, hence the ''usual''form. Currently, IPF …