The Vasculature in Pulmonary Fibrosis.
The Vasculature in Pulmonary Fibrosis.
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DOI：
10.1007/s43152-022-00040-9
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发表时间：
2022-12
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当前特发性肺纤维化（IPF）发病机制的范例涉及敏感肺泡上皮的反复损伤，随后以成纤维细胞活化和细胞外基质沉积为标志的修复反应受损。单细胞 RNA 测序和肺发育生物学的进展表明，多种细胞类型参与了这种反应，并具有潜在的作用。值得注意的是，最近的工作更好地描述了肺内皮中存在的细胞类型，并确定了 IPF 患者的血管变化。对 IPF 患者的肺组织进行了单细胞分辨率检查，结果显示肺毛细血管细胞减少，表达支气管内皮相关标记物的血管细胞群增多。此外，临床前模型已证明衰老和血管通透性在肺纤维化的发展中起着重要作用。越来越多的证据表明，内皮在纤维化的背景下发生变化，这些变化可能有助于肺纤维化的发生和/或进展。需要进行更多研究来进一步确定这些血管变化的功能作用。
The current paradigm of idiopathic pulmonary fibrosis (IPF) pathogenesis involves recurrent injury to a sensitive alveolar epithelium followed by impaired repair responses marked by fibroblast activation and deposition of extracellular matrix. Multiple cell types are involved in this response with potential roles suggested by advances in single-cell RNA sequencing and lung developmental biology. Notably, recent work has better characterized the cell types present in the pulmonary endothelium and identified vascular changes in patients with IPF. Lung tissue from patients with IPF has been examined at single-cell resolution, revealing reductions in lung capillary cells and expansion of a population of vascular cells expressing markers associated with bronchial endothelium. In addition, pre-clinical models have demonstrated a fundamental role for aging and vascular permeability in the development of pulmonary fibrosis. Mounting evidence suggests that the endothelium undergoes changes in the context of fibrosis, and these changes may contribute to the development and/or progression of pulmonary fibrosis. Additional studies will be needed to further define the functional role of these vascular changes.