North West Interstitial Lung Disease Unit, Manchester University NHS Foundation Trust, Manchester, UK.
North West Interstitial Lung Disease Unit, Manchester University NHS Foundation Trust, Wythenshawe Hospital, Southmoore Road, Manchester M23 9LT, UK.
Ther Adv Respir Dis. 2018 Jan-Dec;12:1753466618800618. doi: 10.1177/1753466618800618.
Idiopathic pulmonary fibrosis (IPF) is a fibrotic interstitial lung disease associated with significant morbidity and mortality. Previously, IPF has been managed using immunosuppressive therapy; however, it has been shown that this is associated with increased mortality. In the last 5 years, two disease-modifying agents have been licensed for use in IPF, namely pirfenidone and nintedanib. Nintedanib is a tyrosine kinase inhibitor with antifibrotic properties that has also been shown to significantly reduce the progression of the disease. The scientific evidence shows that nintedanib is effective and well tolerated for the treatment of IPF in mild, moderate and severe stages of the disease. Real-world experiences also support the findings of previously conducted clinical trials and show that nintedanib is effective for the management of IPF and is associated with reducing disease progression. Gastrointestinal events, mainly diarrhoea, are the main adverse events caused by the treatment. Recent real-word studies also suggest that nintedanib stabilizes lung function till lung transplantation, with no increased surgical complications or postoperative mortality after lung transplantation. In this review, we will discuss the clinical trial evidence and real-world experience for nintedanib in the management of IPF.
特发性肺纤维化(IPF)是一种纤维化性间质性肺疾病,与较高的发病率和死亡率相关。既往,IPF 的治疗采用免疫抑制疗法,但已证实该疗法会增加死亡率。在过去 5 年中,已有两种疾病修正治疗药物获 IPF 适应证批准,即吡非尼酮和尼达尼布。尼达尼布是一种具有抗纤维化特性的酪氨酸激酶抑制剂,也已被证实可显著减缓疾病进展。科学证据表明,尼达尼布对轻度、中度和重度 IPF 均具有疗效,且耐受良好。真实世界经验也支持先前进行的临床试验结果,表明尼达尼布可有效治疗 IPF,并可减缓疾病进展。胃肠道事件,主要是腹泻,是治疗引起的主要不良事件。最近的真实世界研究还表明,尼达尼布可稳定肺功能直至肺移植,肺移植后手术并发症或术后死亡率无增加。在本次综述中,我们将讨论尼达尼布治疗 IPF 的临床试验证据和真实世界经验。