Pathogenesis of Idiopathic Pulmonary Fibrosis

摘要

Recent years have seen a robust influx of exciting new observations regarding the mechanisms that regulate the initiation and progression of pulmonary fibrosis. New therapeutic targets include the epithelial cell, myofibroblast as well as chronic inflammation. A schematic view of the complex biological processes that have been implicated in the pathogenesis of pulmonary fibrosis is presented. Unfortunately, it remains unknown if 'standard' therapy of corticosteroids and cytotoxic medications is more effective than placebo alone in the treatment of IPF. However, new insights into pathogenesis are questioning the validity of an immunosuppressive approach and generating hypotheses for new clinical trials. IPF is a complex disorder and no unifying hypothesis has been identified at present that explains all the abnormalities. However, tremendous strides are being made in elucidating novel mechanisms for pathogenesis and these targets are being tested in the clinic.