结缔组织病相关性间质性肺病发病机制及相关因素的研究进展

Research Progress on Pathogenesis and Related Factors of Interstitial Lung Disease Associated with Connective Tissue Disease

摘要: 结缔组织疾病相关间质性肺疾病(Connective Tissue Disease-Associated Interstitial Lung Disease, CTD-ILD)是一组系统性自身免疫性疾病，导致肺间质异常或肺纤维化，但是目前CTD-ILD的发病机制尚不清楚。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，包括遗传异常和环境危险因素。主要的致病机制是这些危险因素促进肺泡II型细胞功能障碍，触发许多下游的纤维原性途径，包括炎症级联，导致肺成纤维细胞增殖和活化，引起肺重构和修复异常，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主要原因。然而，还需要验证性研究。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、临床监测和疾病预后提供了新发现的疾病生物标志物。这篇综述从CTD-ILD发病机制和促进疾病生物标志物发现的生物驱动因素进行介绍。

Abstract: Connective Tissue Disease-Associated Interstitial Lung Disease (CTD-ILD) is a group of systemic autoimmune diseases that result in pulmonary interstitial abnormalities or pulmonary fibrosis but the pathogenesis of CTD-ILD is currently unknown. Several common risk factors are associated with idiopathic pulmonary fibrosis, an extensively studied fibrotic lung disease that includes genetic abnormalities and environmental risk factors. The main pathogenic mechanism is that these risk factors promote the dysfunction of alveolar type II cells and trigger many downstream fibrogenic pathways, including inflammatory cascades, leading to the proliferation and activation of lung fibroblasts, causing abnormal lung remodeling and repair, leading to interstitial pathology and pulmonary fibrosis. In CTD-ILD, dysregulation of inflammatory regulatory pathways is the main cause. However, confirmatory studies are needed. Understanding the pathogenesis is necessary to develop and tailor more targeted treatments and provides newly discovered disease biomarkers for early diagnosis, clinical monitoring, and disease prognosis. This review provides an overview of the pathogenesis of CTD-ILD and the biological drivers that promote the discovery of disease biomarkers.