Columbia University Medical Center, New York, New York, USA.
Winthrop University Hospital, Mineola, New York, USA.
Kidney Int. 2017 Jul;92(1):37-46. doi: 10.1016/j.kint.2016.11.029. Epub 2017 Mar 17.
In various human diseases, an increase in capillary permeability to proteins leads to the loss of protein-rich fluid from the intravascular to the interstitial space. Although sepsis is the disease most commonly associated with this phenomenon, many other diseases can lead to a "sepsis-like" syndrome with manifestations of diffuse pitting edema, exudative serous cavity effusions, noncardiogenic pulmonary edema, hypotension, and, in some cases, hypovolemic shock with multiple-organ failure. The term capillary leak syndrome has been used to describe this constellation of disease manifestations associated with an increased capillary permeability to proteins. Diseases other than sepsis that can result in capillary leak syndrome include the idiopathic systemic capillary leak syndrome or Clarkson's disease, engraftment syndrome, differentiation syndrome, the ovarian hyperstimulation syndrome, hemophagocytic lymphohistiocytosis, viral hemorrhagic fevers, autoimmune diseases, snakebite envenomation, and ricin poisoning. Drugs including some interleukins, some monoclonal antibodies, and gemcitabine can also cause capillary leak syndrome. Acute kidney injury is commonly seen in all of these diseases. In addition to hypotension, cytokines are likely to be important in the pathophysiology of acute kidney injury in capillary leak syndrome. Fluid management is a critical part of the treatment of capillary leak syndrome; hypovolemia and hypotension can cause organ injury, whereas capillary leakage of administered fluid can worsen organ edema leading to progressive organ injury. The purpose of this article is to discuss the diseases other than sepsis that produce capillary leak and review their collective pathophysiology and treatment.
在各种人类疾病中，毛细血管对蛋白质的通透性增加会导致富含蛋白质的液体从血管内空间流失到间质空间。虽然败血症是最常与这种现象相关的疾病，但许多其他疾病也可能导致“类似败血症”的综合征，表现为弥漫性凹陷性水肿、渗出性浆膜腔积液、非心源性肺水肿、低血压，在某些情况下，还会出现伴有多器官衰竭的低血容量性休克。毛细血管渗漏综合征这一术语被用于描述与蛋白质通透性增加相关的这种疾病表现组合。除败血症外，还可能导致毛细血管渗漏综合征的疾病包括特发性全身性毛细血管渗漏综合征或克拉克森病、植入综合征、分化综合征、卵巢过度刺激综合征、噬血细胞性淋巴组织细胞增多症、病毒性出血热、自身免疫性疾病、蛇咬伤中毒和蓖麻毒素中毒。包括某些白细胞介素、某些单克隆抗体和吉西他滨在内的药物也可能导致毛细血管渗漏综合征。所有这些疾病都常见急性肾损伤。除了低血压外，细胞因子可能在毛细血管渗漏综合征的急性肾损伤发病机制中起着重要作用。液体管理是毛细血管渗漏综合征治疗的关键部分；低血容量和低血压会导致器官损伤，而给予的液体的毛细血管渗漏会加重器官水肿，导致进行性器官损伤。本文旨在讨论除败血症以外产生毛细血管渗漏的疾病，并回顾其共同的病理生理学和治疗方法。
