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Cystic Fibrosis Lung Disease in the Aging Population

机译：囊性纤维化肺病在衰老人口中

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The demographics of the population with cystic fibrosis (CF) is continuously changing, with nowadays adults outnumbering children and a median predicted survival of over 40 years. This leads to the challenge of treating an aging CF population, while previous research has largely focused on pediatric and adolescent patients. Chronic inflammation is not only a hallmark of CF lung disease, but also of the aging process. However, very little is known about the effects of an accelerated aging pathology in CF lungs. Several chronic lung disease pathologies show signs of chronic inflammation with accelerated aging, also termed “inflammaging”; the most notable being chronic obstructive pulmonary disease (COPD) and idiopathic pulmonary fibrosis (IPF). In these disease entities, accelerated aging has been implicated in the pathogenesis via interference with tissue repair mechanisms, alterations of the immune system leading to impaired defense against pulmonary infections and induction of a chronic pro-inflammatory state. In addition, CF lungs have been shown to exhibit increased expression of senescence markers. Sustained airway inflammation also leads to the degradation and increased turnover of cystic fibrosis transmembrane regulator (CFTR). This further reduces CFTR function and may prevent the novel CFTR modulator therapies from developing their full efficacy. Therefore, novel therapies targeting aging processes in CF lungs could be promising. This review summarizes the current research on CF in an aging population focusing on accelerated aging in the context of chronic airway inflammation and therapy implications.
机译：囊性纤维化（CF）人口的人口统计学不断变化，现在成年人数量超过儿童和中位预测的生存率超过40年。这导致治疗衰老的CF群体的挑战，而以前的研究主要集中在儿科和青少年患者身上。慢性炎症不仅是CF肺病的标志，也是老化过程。然而，关于CF肺中加速老化病理学的影响很少。几种慢性肺病病理显示慢性炎症与加速衰老的迹象，也称为“焦躁”;最值得注意的是慢性阻塞性肺疾病（COPD）和特发性肺纤维化（IPF）。在这些疾病实体中，加速老化通过干扰组织修复机制而在发病机制中涉及，免疫系统的改变导致防御肺部感染和慢性促炎症的诱导障碍。此外，已显示CF肺部表现出衰老标志物的表达增加。持续的气道炎症也导致囊性纤维化跨膜调节剂（CFTR）的降解和增加。这进一步降低了CFTR功能，并且可以防止新的CFTR调制器疗法显影它们的全部功效。因此，靶向CF肺中老化过程的新疗法可能是有前途的。本综述总结了目前在慢性气道炎症和治疗意义上的加速老龄化的老化人口中的CF研究。

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