McElvaney Oisín F, Murphy Mark P, Reeves Emer P, McElvaney Noel G
Irish Centre for Genetic Lung Disease, Department of Medicine, Royal College of Surgeons in Ireland, Education and Research Centre, Beaumont Hospital, Dublin, Ireland.
Chronic Obstr Pulm Dis. 2020 Jul;7(3):203-213. doi: 10.15326/jcopdf.7.3.2019.0171.
For many years, the lung disease associated with alpha-1 antitrypsin (AAT) deficiency (AATD) was perceived as being secondary to an imbalance between this serine protease inhibitor and the target protease, neutrophil elastase (NE). More recently, a greater understanding of the pathways leading to lung inflammation has shed light on new potential attributes and presented AATD as an inflammatory condition in which proteases and neutrophils still play a major role, but in which pro-inflammatory cytokines, either induced by the actions of NE or by other pro-inflammatory processes normally modulated by AAT, are involved. In this review, we will look at the various cytokines centrally involved in AATD lung disease, and how a greater understanding of their contribution may help development of targeted therapies.
多年来，与α-1抗胰蛋白酶（AAT）缺乏症（AATD）相关的肺部疾病被认为是这种丝氨酸蛋白酶抑制剂与靶蛋白酶中性粒细胞弹性蛋白酶（NE）之间失衡的继发结果。最近，对导致肺部炎症的途径有了更深入的了解，揭示了新的潜在特征，并将AATD呈现为一种炎症性疾病，其中蛋白酶和中性粒细胞仍然起主要作用，但促炎细胞因子也参与其中，这些细胞因子要么由NE的作用诱导，要么由通常由AAT调节的其他促炎过程诱导。在这篇综述中，我们将探讨在AATD肺部疾病中起核心作用的各种细胞因子，以及对它们作用的更深入理解如何有助于开发靶向治疗方法。
