The Margaret Turner Warwick Centre for Fibrosing Lung Diseases, Imperial College London National Heart and Lung Institute, Imperial College, London, UK.
Respiratory Medicine Department, Imperial College NHS Hospitals, London, UK.
Expert Opin Pharmacother. 2023 May-Aug;24(10):1125-1142. doi: 10.1080/14656566.2023.2213436. Epub 2023 May 17.
Idiopathic Pulmonary Fibrosis (IPF) is a progressive and devastating lung disease, characterized by progressive lung scarring.
Prior to antifibrotic therapy (pirfenidone and nintedanib), there was no validated pharmaceutical therapy for IPF. Both antifibrotics can slow disease progression; however, IPF remains a detrimental disease with poor prognosis and treatment survival rates of less than 7 years from diagnosis. Despite their effect the disease remains non-reversible and progressing whilst their side effect profile is often challenging. Treatment of comorbidities is also crucial. In this review, we discuss the current pharmacological management as well as management of comorbidities and symptoms. We also reviewed clinicaltrials.gov and summarized all the mid- to late-stage clinical trials (phase II and III) registered in IPF over the last 7 years and discuss the most promising drugs in clinical development.
Future for IPF management will need to focus on current unresolved issues. First a primary pathogenetic pathway has not been clearly identified. Future management may involve a combination of the brushstroke approach with antifibrotics with targeted treatments for specific pathways in patient subsets following an 'oncological' approach. Another unmet need is the management of exacerbations, which are deadly in most cases, as well as either treating or preventing lung cancer.
特发性肺纤维化（IPF）是一种进行性和破坏性的肺部疾病，其特征是肺部进行性瘢痕形成。
在使用抗纤维化药物（吡非尼酮和尼达尼布）之前，IPF 没有经过验证的药物治疗方法。这两种抗纤维化药物都可以减缓疾病进展；然而，IPF 仍然是一种预后不良的疾病，诊断后治疗生存率不到 7 年。尽管它们有效果，但疾病仍然不可逆转且在进展，同时它们的副作用情况常常具有挑战性。治疗合并症也很重要。在这篇综述中，我们讨论了当前的药物治疗方法以及合并症和症状的管理。我们还回顾了 clinicaltrials.gov，并总结了过去 7 年中在 IPF 中注册的所有中期至晚期临床试验（II 期和 III 期），并讨论了临床开发中最有前途的药物。
未来的 IPF 管理需要关注当前尚未解决的问题。首先，尚未明确确定主要的发病途径。未来的管理可能涉及使用抗纤维化药物进行联合治疗，针对特定患者亚群的特定途径进行靶向治疗，采用“肿瘤学”方法。另一个未满足的需求是治疗或预防肺癌，以及管理大多数情况下致命的加重情况。
