Idiopathic Pulmonary Fibrosis —Possible new treatments and recent clinical trials—

Abstract: Idiopathic pulmonary fibrosis (IPF) is a chronically progressive fatal disease. However, as there are no prospects of successful prognostic improve-ment by currently available methods, the development of effective new treatments to improve prognosis is eagerly awaited throughout the world. Although much remains to be elucidated as to the pathology of IPF, it is understood according to two processes: lung injury resulting from preceding inflammation and fibrosis caused by abnormal repair. Most conventional therapies, including steroid therapy, have focused on the inhibition of inflammation. However, the inhibition of fibrosis itself has been attracting recent attention, and clinical applications are evolving. This paper describes the current status of clinical trials of interferon therapy ongo-ing in Western countries, with an explanation of its mechanism of action. In addi-tion, the progress of a clinical trial of pirfenidone, the first randomized placebo-controlled study on the drug treatment of IPF in Japan, is described. Various treatment strategies for IPF suggested by basic research are also introduced