Department of Medicine, Cedars Sinai Medical Center, Los Angeles, California 90048; email:
Annu Rev Med. 2016;67:487-95. doi: 10.1146/annurev-med-120214-013614. Epub 2015 Nov 4.
Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive, and fatal disease that has long eluded therapy. Prognosis remains very poor, and currently lung transplantation offers the only hope of survival. Recently, great strides have been made in the development of pharmaceutical therapy to treat IPF. Pirfenidone, an oral antifibrotic agent, has been shown to slow progression of the disease and improve progression-free survival, offering new hope for patients suffering from IPF.
特发性肺纤维化（IPF）是一种慢性、进行性和致命的疾病，长期以来一直缺乏治疗方法。预后仍然非常差，目前肺移植是唯一的生存希望。最近，在开发治疗 IPF 的药物治疗方面取得了重大进展。吡非尼酮是一种口服抗纤维化药物，已被证明可减缓疾病进展并改善无进展生存期，为 IPF 患者带来了新的希望。
