Molecular & Cellular Pneumonology Laboratory, Department of Respiratory Medicine, Faculty of Medicine, University of Crete, Greece.
Molecular & Cellular Pneumonology Laboratory, Department of Respiratory Medicine, Faculty of Medicine, University of Crete, Greece.
Curr Opin Pharmacol. 2021 Apr;57:71-80. doi: 10.1016/j.coph.2020.12.007. Epub 2021 Feb 6.
Idiopathic pulmonary fibrosis (IPF) is a progressive, irreversible fibrotic chronic lung disease affecting predominantly older adults, with a history of smoking. The current model of disease natural course is that recurrent injury of the alveolar epithelium in the context of advanced aging/cellular senescence is followed by defective re-epithelialization and scar tissue formation. Currently, two drugs, nintedanib and pirfenidone, that modify disease progression have been approved worldwide for the treatment of IPF. However, despite treatment, patients with IPF are not cured, and eventually, disease advances in most treated patients. Enhancing biogenomic and metabolic research output, its translation into clinical precision and optimal service delivery through patient-centeredness are key elements to support effective IPF care. In this review, we summarize therapeutic options currently investigated for IPF based on the major pathogenetic pathways and molecular targets that drive pulmonary fibrosis.
特发性肺纤维化（IPF）是一种进行性、不可逆转的纤维性慢性肺部疾病，主要影响老年人，且有吸烟史。目前的疾病自然病程模型是，在衰老/细胞衰老的背景下，肺泡上皮反复损伤，随后出现上皮细胞再修复缺陷和瘢痕组织形成。目前，两种药物尼达尼布和吡非尼酮已被全球批准用于治疗 IPF，可改变疾病进展。然而，尽管进行了治疗，IPF 患者仍未治愈，大多数接受治疗的患者最终疾病仍会进展。加强生物基因组学和代谢研究成果，通过以患者为中心将其转化为临床精准医疗和最佳服务提供，是支持有效 IPF 护理的关键要素。在这篇综述中，我们根据驱动肺纤维化的主要发病途径和分子靶点，总结了目前针对 IPF 进行研究的治疗选择。
