Stavos Garantziotis, MD, and a team of researchers from the National Institute of Environmental Health (NIEHS) have identified a new mechanism that explains why some people with idiopathic pulmonary fibrosis (IPF) get more short of breath than others with the condition. The paper appears in the November issue of American Journal of Respiratory and Critical Care Medicine.
In normal, healthy individuals, the body forms new blood vessels that can help heal an injury. If you cut your finger, for example, the body delivers nutrients and cells to the injury site to promote wound healing. The same mechanism goes to work in IPF, but for some reason the process backfires or is disrupted and may be doing the patients more harm than good. The investigators explain that instead of building healthy new tissue to heal the scarring of IPF, patients with higher levels of the blood protein inter-alpha-trypsin inhibitor—which binds with a connective tissue molecule to make new blood vessels—develop vessels that are far away from where they should be, pushing the blood away from the lung and bypassing the area where the bodcy gets its oxygen, thus causing more shortness of breath.
Garantziotis says that the study is important for two resons: It demonstrates for the first time the important role that a blood circulating protein plays in lung functions; and it dientifies a potential new therapeutic target for IPF.