Adamali Huzaifa I, Maher Toby M
Interstitial Lung Disease Unit, Royal Brompton Hospital, London, UK.
Drug Des Devel Ther. 2012;6:261-72. doi: 10.2147/DDDT.S29928. Epub 2012 Sep 26.
Over the past decade, there has been a cohesive effort from patients, physicians, clinical and basic scientists, and the pharmaceutical industry to find definitive treatments for idiopathic pulmonary fibrosis (IPF). As understanding of disease behavior and pathogenesis has improved, the aims of those treating IPF have shifted from reversing the disease to slowing or preventing progression of this chronic fibrotic illness. It is to be hoped that by slowing disease progression, survival will be improved from the current dismal median of 3.5 years following diagnosis. In Europe and Asia, a milestone has recently been reached with the licensing of the first IPF-specific drug, pirfenidone. This review assesses the current treatment modalities available for IPF, including pirfenidone. It also turns an eye to the future and discusses the growing number of promising compounds currently in development that it is hoped, in time, will make their way into the clinic as treatments for IPF.
在过去十年中，患者、医生、临床和基础科学家以及制药行业共同努力，寻找特发性肺纤维化（IPF）的确定性治疗方法。随着对疾病行为和发病机制的认识不断提高，治疗IPF的目标已从逆转疾病转变为减缓或预防这种慢性纤维化疾病的进展。希望通过减缓疾病进展，能够将目前诊断后3.5年的惨淡中位生存期提高。在欧洲和亚洲，首个IPF特异性药物吡非尼酮的获批最近成为一个里程碑。本综述评估了目前可用于IPF的治疗方式，包括吡非尼酮。它还展望未来，讨论了目前正在研发的越来越多有前景的化合物，希望它们最终能够进入临床作为IPF的治疗药物。
