Department of Respiratory Diseases and Allergy, Aarhus University Hospital, Aarhus, Denmark,
Department of Respiratory Diseases, Interstitial Lung Disease and Lung Transplant Unit, University Hospitals Leuven, Leuven, Belgium.
Respiration. 2019;97(2):173-184. doi: 10.1159/000495046. Epub 2018 Dec 13.
Idiopathic pulmonary fibrosis (IPF) is a fatal lung disease with a dismal survival rate of only 3 years and no curative pharmacological therapy. The recent approval of 2 anti-fibrotic drugs (nintedanib and pirfenidone) that slow disease progression has provided some hope for patients. However, effectively managing anti-fibrotic treatment can be a challenge due to tolerability issues, the presence of pulmonary and extra-pulmonary comorbidities, and the need for concomitant medications in many patients. In general, making clear evidence-based decisions can be difficult for physicians because patients with comorbidities are often excluded from clinical trials. Since currently anti-fibrotic drugs are the only effective therapeutics capable of slowing disease progression, it is imperative that all treatment options are thoroughly evaluated and exhausted in each individual, irrespective of complicating factors, to permit the best outcome for the patient. In this review, we present data from clinical trials, post hoc analyses, post-marketing surveillance, and real-world studies that are relevant to the management of nintedanib treatment. In addition, we also provide practical recommendations developed by a multidisciplinary panel of experts for the management of nintedanib treatment in patients with IPF associated complications and those experiencing gastrointestinal side effects.
特发性肺纤维化(IPF)是一种致命的肺部疾病,生存率仅为 3 年,且没有有效的药物治疗。最近批准的 2 种抗纤维化药物(尼达尼布和吡非尼酮)可减缓疾病进展,为患者带来了一些希望。然而,由于耐受性问题、肺部和肺外合并症的存在,以及许多患者需要同时使用药物,有效管理抗纤维化治疗可能是一个挑战。一般来说,由于患有合并症的患者通常被排除在临床试验之外,因此医生很难做出明确的基于证据的决策。由于目前抗纤维化药物是唯一能够减缓疾病进展的有效治疗方法,因此必须彻底评估和用尽每种治疗方法,无论是否存在并发症,以使患者获得最佳结果。在这篇综述中,我们介绍了与尼达尼布治疗管理相关的临床试验、事后分析、上市后监测和真实世界研究的数据。此外,我们还提供了一个多学科专家小组制定的实用建议,用于管理与 IPF 相关并发症和胃肠道副作用的患者的尼达尼布治疗。