Research Service and the Pulmonary Section Medical Service, VA Ann Arbor Health System, Department of Veterans Affairs Health System, Ann Arbor, Michigan; Division of Pulmonary and Critical Care Medicine, Department of Internal Medicine, University of Michigan, Ann Arbor, Michigan.
Research Service and the Pulmonary Section Medical Service, VA Ann Arbor Health System, Department of Veterans Affairs Health System, Ann Arbor, Michigan.
Am J Pathol. 2022 Mar;192(3):410-425. doi: 10.1016/j.ajpath.2021.11.012. Epub 2021 Dec 23.
Histopathologic evidence of deployment-related constrictive bronchiolitis (DRCB) has been identified in soldiers deployed to Southwest Asia. While inhalational injury to the airway epithelium is suspected, relatively little is known about the pathogenesis underlying this disabling disorder. Club cells are local progenitors critical for repairing the airway epithelium after exposure to various airborne toxins, and a prior study using an inducible transgenic murine model reported that 10 days of sustained targeted club cell injury causes constrictive bronchiolitis. To further understand the mechanisms leading to small airway fibrosis, a murine model was employed to show that sustained club cell injury elicited acute weight loss, caused increased local production of proinflammatory cytokines, and promoted accumulation of numerous myeloid cell subsets in the lung. Transition to a chronic phase was characterized by up-regulated expression of oxidative stress-associated genes, increased activation of transforming growth factor-β, accumulation of alternatively activated macrophages, and enhanced peribronchiolar collagen deposition. Comparative histopathologic analysis demonstrated that sustained club cell injury was sufficient to induce epithelial metaplasia, airway wall thickening, peribronchiolar infiltrates, and clusters of intraluminal airway macrophages that recapitulated key abnormalities observed in DRCB. Depletion of alveolar macrophages in mice decreased activation of transforming growth factor-β and ameliorated constrictive bronchiolitis. Collectively, these findings implicate sustained club cell injury in the development of DRCB and delineate pathways that may yield biomarkers and treatment targets for this disorder.
组织病理学证据表明,部署到西南亚的士兵中存在与部署相关的缩窄性细支气管炎(DRCB)。虽然怀疑气道上皮细胞吸入性损伤,但对这种使人丧失能力的疾病的发病机制知之甚少。Club 细胞是局部祖细胞,对于暴露于各种空气传播毒素后修复气道上皮至关重要,先前使用诱导型转基因小鼠模型的研究报告称,持续靶向 Club 细胞损伤 10 天可导致缩窄性细支气管炎。为了进一步了解导致小气道纤维化的机制,采用了小鼠模型来表明持续的 Club 细胞损伤会引起急性体重减轻,导致局部促炎细胞因子产生增加,并促进大量骨髓细胞亚群在肺部积聚。向慢性阶段的转变的特征是与氧化应激相关的基因表达上调、转化生长因子-β的激活增加、交替激活的巨噬细胞的积累以及周围细支气管胶原沉积的增强。比较组织病理学分析表明,持续的 Club 细胞损伤足以诱导上皮化生、气道壁增厚、周围细支气管浸润和管腔内气道巨噬细胞簇,这些都再现了在 DRCB 中观察到的关键异常。在小鼠中耗尽肺泡巨噬细胞可减少转化生长因子-β的激活并改善缩窄性细支气管炎。总的来说,这些发现表明持续的 Club 细胞损伤与 DRCB 的发展有关,并描绘了可能为这种疾病提供生物标志物和治疗靶点的途径。