Department of Respiratory Medicine, Royal Prince Alfred Hospital, Sydney, NSW, Australia.
Faculty of Medicine, University of Sydney, Sydney, NSW, Australia.
Respirology. 2017 Oct;22(7):1436-1458. doi: 10.1111/resp.13146. Epub 2017 Aug 27.
Idiopathic pulmonary fibrosis (IPF) is a fibrosing interstitial lung disease (ILD) of unknown aetiology with a median survival of only 2-5 years. It is characterized by progressive dyspnoea and worsening lung function, ultimately resulting in death. Until recently, there were no effective therapies for IPF; however, with the publication of two landmark clinical trials in 2014, the anti-fibrotic therapies, nintedanib and pirfenidone, have gained widespread approval. This position paper aims to highlight the current evidence for the treatment of IPF, with particular application to the Australian and New Zealand population. We also consider areas in which evidence is currently lacking, especially with regard to the broader IPF severity spectrum and treatment of co-morbid conditions. The utility of non-pharmacological therapies including pulmonary rehabilitation, oxygen as well as symptom management thought to be important in the holistic care of IPF patients are also discussed.
特发性肺纤维化（IPF）是一种病因不明的纤维性肺间质疾病（ILD），中位生存时间仅为 2-5 年。其特征为进行性呼吸困难和肺功能恶化，最终导致死亡。直到最近，IPF 仍然没有有效的治疗方法；然而，随着 2014 年两项具有里程碑意义的临床试验的发表，抗纤维化治疗药物尼达尼布和吡非尼酮已得到广泛认可。本立场文件旨在强调目前治疗 IPF 的证据，特别是针对澳大利亚和新西兰人群。我们还考虑了目前证据不足的领域，特别是在更广泛的 IPF 严重程度谱和治疗合并症方面。还讨论了包括肺康复、氧疗以及被认为对 IPF 患者整体护理很重要的症状管理在内的非药物治疗的效用。
