Understanding Interstitial Lung Diseases Associated with Connective Tissue Disease (CTD-ILD): Genetics, Cellular Pathophysiology, and Biologic Drivers.
Understanding Interstitial Lung Diseases Associated with Connective Tissue Disease (CTD-ILD): Genetics, Cellular Pathophysiology, and Biologic Drivers.
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DOI：
10.3390/ijms24032405
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发表时间：
2023-01-26
影响因子：
5.6
通讯作者：
Parimon, Tanyalak
中科院分区：
文献类型：
--
作者：
Chiang, Giuliana Cerro;Parimon, Tanyalak
结缔组织病相关间质性肺疾病（CTD-ILD）是导致肺间质异常或肺纤维化的一系列系统性自身免疫性疾病。由于疾病异质性和缺乏临床前模型，CTD-ILD 的发病机制尚不明确。一些常见的危险因素与特发性肺纤维化相互关联，特发性肺纤维化是一种广泛研究的纤维化肺病，其中包括遗传异常和环境危险因素。主要致病机制是这些危险因素促进肺泡II型细胞功能障碍，触发许多下游促纤维化途径，包括炎症级联反应，导致肺成纤维细胞增殖和活化，引起肺重塑和修复异常，导致间质病理和肺纤维化。在 CTD-ILD 中，炎症调节通路的失调是罪魁祸首。然而，需要进行验证性研究。了解这些发病机制对于开发和定制更有针对性的治疗是必要的，并为早期诊断、临床监测和疾病预测提供新发现的疾病生物标志物。本综述强调了 CTD-ILD 的核心发病机制和促进疾病生物标志物发现的生物驱动因素。
Connective tissue disease-associated interstitial lung disease (CTD-ILD) is a collection of systemic autoimmune disorders resulting in lung interstitial abnormalities or lung fibrosis. CTD-ILD pathogenesis is not well characterized because of disease heterogeneity and lack of pre-clinical models. Some common risk factors are inter-related with idiopathic pulmonary fibrosis, an extensively studied fibrotic lung disease, which includes genetic abnormalities and environmental risk factors. The primary pathogenic mechanism is that these risk factors promote alveolar type II cell dysfunction triggering many downstream profibrotic pathways, including inflammatory cascades, leading to lung fibroblast proliferation and activation, causing abnormal lung remodeling and repairs that result in interstitial pathology and lung fibrosis. In CTD-ILD, dysregulation of regulator pathways in inflammation is a primary culprit. However, confirmatory studies are required. Understanding these pathogenetic mechanisms is necessary for developing and tailoring more targeted therapy and provides newly discovered disease biomarkers for early diagnosis, clinical monitoring, and disease prognostication. This review highlights the central CTD-ILD pathogenesis and biological drivers that facilitate the discovery of disease biomarkers.
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影响因子：
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DOI：
10.1038/s41577-021-00524-z
发表时间：
2021-09
期刊：
Nature reviews. Immunology
影响因子：
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通讯作者：
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