Idiopathic pulmonary fibrosis:Pathophysiological data

Idiopathic pulmonary fibrosis is the most common of the idiopathic interstitial pneumonias. The role of inflammation in idiopathic pulmonary fibrosis (IPF) is controversial. If inflammation were critical to the disease process, lung pathology would demonstrate an influx of inflammatory cells, and that the disease would respond to immunosuppression. The classic pathology does not display substantial inflammation, and no modulation of the immune system is effective as treatment. Recent data suggest that the pathophysiology of the disease is more a product of fibroblast dysfunction than of dysregulated inflammation. The concept of epithelial-mesenchymal cell transition has recently received much attention; this transition appears to play a greater role in the pathogenesis than inflammation. It's suggested that inflammation is indeed a critical factor in IPF and proposed five potential nontraditional mechanisms for the role of inflammation in the pathogenesis of IPF: the direct inflammatory hypothesis, the matrix hypothesis, the growth factor-receptor hypothesis, the plasticity hypothesis, and the vascular hypothesis.