系统性红斑狼疮相关肺动脉高压发病机制研究进展

Research Progress in Pathogenesis of Systemic Lupus Erythematosus Associated Pulmonary Arterial Hypertension

肺动脉高压(PAH)是系统性红斑狼疮(SLE)严重的肺部并发症之一,其发病机制尚不明确,发病隐匿,病死率较高。SLE相关PAH(SLE-PAH)的发病机制可能与自身免疫和免疫失调引起的炎症改变、内皮细胞功能障碍相关的血管活性介质失衡、肺血管病变等因素有关。不同发病机制引起的SLE-PAH的病理变化不同,使得SLE-PAH的治疗成为一大难题。目前,需要针对SLE-PAH的发病机制进行深入研究,以寻找新的信号通路与致病因子,从而为SLE-PAH的治疗提供新治疗靶点与方向。

Pulmonary arterial hypertension(PAH)is one of the serious pulmonary complications of systemic lupus erythematosus(SLE),the pathogenesis of which is not clear,the onset is hidden,and the mortality is high.The pathogenesis of SLE associated PAH(SLE-PAH)may be related to inflammatory changes caused by autoimmune and immune disorders,imbalance of vascular active mediators related to endothelial dysfunction,pulmonary vascular disease and other factors.The pathologic changes of SLE-PAH are different in different pathogenesis,making the treatment of SLE-PAH a difficult problem.At present,in-depth studies on the pathogenesis of SLE-PAH are needed to discover new signaling pathways and pathogenic factors,so as to provide new therapeutic targets and directions for the treatment of SLE-PAH.