特发性系统性毛细血管渗漏综合征研究进展

Research progress of idiopathic systemic capillary leakage syndrome

特发性系统性毛细血管渗漏综合征(ISCLS)是一种罕见的疾病,表现为急性发作性毛细血管渗漏(SCL)增多,导致富蛋白液从血管内渗漏到组织间隙,从而出现进行性全身性水肿、低蛋白血症、低血压、血液浓缩等临床症状.因ISCLS常反复发作,病死率高,发病机制尚未明,且无确切治疗方案,因此充分认识ISCLS,及早进行干预极为重要.现就ISCLS的发病特点、临床特征、病理机制、诊治进展进行综述.

Idiopathic systemic capillary leak syndrome(ISCLS)is a rare disease,the clinical manifestation is increased acute episodic capillary leakage(SCL),leading to protein-rich fluid leakage from the blood vessels to the interstitial tissue space,resulting in progressive systemic edema,hypoproteinemia,hypotension,blood concentration and other clinical symptoms.Because of ISCLS frequent recurrences,high mortality,pathogenesis unclear and no definite treatment plan,it is extremely important to fully understand the disease to carry out early intervention to save the patients'lives.This article reviews the progress of ISCLS,including its disease onset characteristics,clinical features,pathological mechanism,diagnosis and treatment of ISCLS.