Idiopathic pulmonary fibrosis (IPF) is a fatal age-associated disease that is characterized by progressive and irreversible scarring of the lung. The pathogenesis of IPF is not completely understood and current therapies are limited to those that reduce the rate of functional decline in patients with mild-to-moderate disease. In this context, new therapeutic approaches that substantially improve the survival time and quality of life of these patients are urgently needed. Our incomplete understanding of the pathogenic mechanisms of IPF and the lack of appropriate experimental models that reproduce the key characteristics of the human disease are major challenges. As ageing is a major risk factor for IPF, age-related cell perturbations such as telomere attrition, senescence, epigenetic drift, stem cell exhaustion, loss of proteostasis and mitochondrial dysfunction are becoming targets of interest for IPF therapy. In this Review, we discuss current and emerging therapies for IPF, particularly those targeting age-related mechanisms, and discuss future therapeutic approaches.

特发性肺纤维化（IPF）是一种致命的年龄相关疾病，其特征是肺部进行性和不可逆的瘢痕形成。IPF的发病机理尚未完全了解，目前的疗法仅限于降低轻度至中度疾病患者的功能下降速度的疗法。在这种情况下，迫切需要能够显着改善这些患者的生存时间和生活质量的新治疗方法。我们对IPF的致病机制的不完全了解以及缺乏能够重现人类疾病关键特征的适当实验模型是主要挑战。由于衰老是IPF的主要危险因素，因此与年龄相关的细胞扰动（例如端粒损耗，衰老，表观遗传漂移，干细胞衰竭，蛋白质变性和线粒体功能障碍的丧失正成为IPF治疗的关注目标。在这篇综述中，我们讨论了IPF的当前和新兴疗法，特别是针对年龄相关机制的疗法，并讨论了未来的治疗方法。