Interstitial Lung Disease Unit, Respiratory Department, Royal Brompton Hospital , London, UK.
Expert Opin Pharmacother. 2021 Feb;22(2):191-204. doi: 10.1080/14656566.2020.1822326. Epub 2020 Sep 29.
Idiopathic Pulmonary Fibrosis is a chronic, progressive lung disease characterized by worsening lung scarring and the radiological/histological pattern of usual interstitial pneumonia. Substantial progress has been made in the clinical management of IPF in the last decade. The two novel antifibrotics, Nintedanib and Pirfenidone have changed the landscape of IPF, by hindering disease progression; however, the drugs have significant discontinuation rates, due to adverse events and do not offer a definitive cure, as such IPF remains a deleterious disease with poor survival.
In this review, the authors focus on the current and emerging pharmacological options in the treatment of IPF. They include a summary of the current approach including treatment of comorbidities and then discuss promising drugs in the drug pipeline.
IPF remains a disease with detrimental outcomes. The plethora of emerging pharmacological treatments brings hope for the future. The current pharmacological 'one fits all' approach has been proven effective in slowing disease progression. The future lies in an oncological approach with combination of therapies. We expect to see a change in clinical trial endpoints and a more inclusive approach for the diagnosis of IPF.
AE: Acute ExacerbationA-SMA: a smooth muscle actinATX: AutotaxinCOPD: Combined Obstructive Pulmonary DiseaseCPFE: Combined Pulmonary Fibrosis and EmphysemaGER: Gastro-esophageal refluxFVC: forced vital capacityECMO: extracorporeal membrane oxygenationILD: Interstitial Lung DiseaseIPF: Idiopathic Pulmonary FibrosisNAC: N-acetylcysteineLPA: Lysophosphatidic acidPH: Pulmonary RehabilitationPR: Pulmonary rehabilitationRCTs: randomized placebo-controlled trialsUIP: usual interstitial pneumonia.
特发性肺纤维化是一种慢性、进行性肺部疾病，其特征是肺部瘢痕恶化以及通常间质性肺炎的影像学/组织学模式。在过去十年中，特发性肺纤维化的临床管理取得了实质性进展。两种新型抗纤维化药物——尼达尼布和吡非尼酮——通过阻止疾病进展改变了特发性肺纤维化的治疗格局；然而，由于不良反应，这些药物的停药率很高，而且它们并不能提供明确的治愈方法，因此特发性肺纤维化仍然是一种危害性疾病，患者的生存率较差。
在这篇综述中，作者重点介绍了特发性肺纤维化治疗中当前和新兴的药理学选择。他们总结了目前的治疗方法，包括治疗合并症，然后讨论了药物研发管道中具有前景的药物。
特发性肺纤维化仍然是一种预后不良的疾病。大量新兴的药理学治疗方法带来了未来的希望。目前针对所有患者的单一药物治疗方法已被证明可有效减缓疾病进展。未来在于联合治疗的肿瘤学方法。我们预计将看到临床试验终点的改变，以及对特发性肺纤维化的诊断采用更具包容性的方法。
AE：急性加重；A-SMA：平滑肌肌动蛋白；ATX：自分泌运动因子；COPD：慢性阻塞性肺疾病；CPFE：合并性肺纤维化和肺气肿；GER：胃食管反流；FVC：用力肺活量；ECMO：体外膜氧合；ILD：间质性肺病；IPF：特发性肺纤维化；NAC：N-乙酰半胱氨酸；LPA：溶血磷脂酸；PH：肺康复；PR：肺康复；RCTs：随机安慰剂对照试验；UIP：寻常性间质性肺炎。
