Idiopathic Pulmonary Fibrosis and Antifibrotic Treatments Focus on Experimental Studies

摘要

Context.-Idiopathic pulmonary fibrosis (IPF) is a progressive fatal disease that up to now has been associated with a poor outcome. Some advances have been made in understanding the multiple interrelated pathogenic path. ways underlying IPF. The disease is now believed to result from complex interactions among genetic, epigenetic, transcriptional, posttranscriptional, metabolic, and environmental factors. The discovery and validation of theranostic biomarkers are necessary to enable a more precise and earlier diagnosis of IPF and to improve the prediction of future disease behavior. Two drugs recently approved by the US Food and Drug Administration, pirfenidone and nintedanib, have shown the ability to reduce the progression of the disease, although survival benefits are only minimal and neither drug prevents or reverses the disease.