Varone Francesco, Sgalla Giacomo, Iovene Bruno, Bruni Teresa, Richeldi Luca
a Polo Scienze Cardiovascolari e Toraciche, Unità Operativa Complessa di Pneumologia, Fondazione Policlinico Universitario Agostino Gemelli , Università Cattolica del Sacro Cuore , Rome , Italy.
Expert Opin Pharmacother. 2018 Feb;19(2):167-175. doi: 10.1080/14656566.2018.1425681. Epub 2018 Jan 12.
Idiopathic Pulmonary Fibrosis (IPF) is an interstitial lung disease characterized by the progressive loss of pulmonary function, ultimately leading to respiratory failure and death. Two novel compounds, nintedanib and pirfenidone, have shown efficacy in reducing the rate of decline of lung function in IPF patients. The multiple tyrosine kinase inhibitor nintedanib has extensively being studied as a potential angiogenesis inhibitor in clinical against various neoplastic disorders. Afterwards, this compound was successfully tested in IPF. Areas covered: Herein, the authors review the working mechanisms of nintedanib, its pharmacological profile, and its efficacy and safety for patients with IPF. Expert opinion: Nintedanib has shown to be safe and effective in patients with IPF, with a favorable long-term safety profile. There is a lack of comparative trials of pirfenidone and nintedanib, and the choice of treatment is left to the physicians' judgement. Future directions of nintedanib use are represented by the treatment of progressive fibrosing interstitial lung disease other than IPF, IPF with advanced functional impairment, and lung fibrosis secondary to connective tissue diseases. A promising safety profile for the combinational use of nintedanib and pirfenidone in IPF has also recently emerged.
特发性肺纤维化(IPF)是一种间质性肺疾病,其特征是肺功能逐渐丧失,最终导致呼吸衰竭和死亡。两种新型化合物,尼达尼布和吡非尼酮,已显示出可降低IPF患者肺功能下降速率的疗效。多酪氨酸激酶抑制剂尼达尼布作为一种潜在的血管生成抑制剂,已在临床上针对各种肿瘤性疾病进行了广泛研究。之后,该化合物在IPF中成功进行了测试。涵盖领域:在此,作者回顾了尼达尼布的作用机制、药理学特征及其对IPF患者的疗效和安全性。专家意见:尼达尼布已证明对IPF患者安全有效,具有良好的长期安全性。缺乏吡非尼酮和尼达尼布的对比试验,治疗选择留给医生判断。尼达尼布使用的未来方向表现为治疗除IPF之外的进行性纤维化间质性肺疾病、具有严重功能损害的IPF以及结缔组织病继发的肺纤维化。最近还出现了尼达尼布和吡非尼酮联合用于IPF的有前景的安全性。