Interstitial pulmonary fibrosis

Abstract

The study of animal models of IPF has demonstrated that there is a stereotyped response of the respiratory airspace walls to a wide variety of injuries. Inflammatory and immune effector cells play a major and complex role in the fibrosing process. They may contribute to the injury of the lung beyond the original insult. These cells secrete substances that play an important role in determining cell traffic in the lungs and in controlling the connective tissue-producing cells. Products derived from the inflammatory response may interfere with protection of normal lung matrix, although injury to lung matrix itself does not lead to fibrosis. Injury to endothelial cells and especially type I epithelial cells appears to play a major role in the fibrogenic response. Further understanding of the factors that injure these cells, the development of methods of protecting them from injury, and a clear understanding of their role in the fibrogenic process appear to be key to developing better methods of preventing and treating interstitial pulmonary fibrosis.