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Abstract：
Capillary leak syndrome (CLS) represents a phenotype of increased fluid extravasation, resulting in intravascular hypovolemia, extravascular edema formation and ultimately hypoperfusion. While endothelial permeability is an evolutionary preserved physiological process needed to sustain life, excessive fluid leak-often caused by systemic inflammation-can have detrimental effects on patients\' outcomes. This article delves into the current understanding of CLS pathophysiology, diagnosis and potential treatments. Systemic inflammation leading to a compromise of endothelial cell interactions through various signaling cues (e.g., the angiopoietin-Tie2 pathway), and shedding of the glycocalyx collectively contribute to the manifestation of CLS. Capillary permeability subsequently leads to the seepage of protein-rich fluid into the interstitial space. Recent insights into the importance of the sub-glycocalyx space and preserving lymphatic flow are highlighted for an in-depth understanding. While no established diagnostic criteria exist and CLS is frequently diagnosed by clinical characteristics only, we highlight more objective serological and (non)-invasive measurements that hint towards a CLS phenotype. While currently available treatment options are limited, we further review understanding of fluid resuscitation and experimental approaches to target endothelial permeability. Despite the improved understanding of CLS pathophysiology, efforts are needed to develop uniform diagnostic criteria, associate clinical consequences to these criteria, and delineate treatment options.
摘要：
毛细血管渗漏综合征（CLS）表现为液体外渗增加的表型，导致血管内血容量不足，血管外水肿形成和最终灌注不足。虽然内皮通透性是维持生命所需的进化保留的生理过程，过多的液体渗漏-通常由全身性炎症引起-可能对患者的预后产生不利影响。本文深入探讨了目前对CLS病理生理学的理解，诊断和潜在的治疗。系统性炎症通过各种信号线索导致内皮细胞相互作用受损(例如，血管生成素-Tie2途径)，糖萼的脱落共同促进了CLS的表现。毛细管通透性随后导致富含蛋白质的流体渗入间隙。强调了对子糖萼空间和保持淋巴流动的重要性的最新见解，以深入了解。虽然没有既定的诊断标准，但CLS通常仅通过临床特征进行诊断。我们强调了更客观的血清学和（非）侵入性测量，提示CLS表型。虽然目前可用的治疗选择有限，我们进一步回顾了对液体复苏和靶向内皮通透性的实验方法的理解。尽管对CLS病理生理学的理解有所提高，需要努力制定统一的诊断标准，将临床后果与这些标准联系起来，并描述治疗方案。
