Capillary leak syndrome pathophysiology

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-In-Chief: M. Hassan, M.B.B.S

Overview

The pathogenesis of capillary leak syndrome remain unclear, however, cytokines, vascular endothelial growth factor (VEGF), leukotrienes, and complement play an important role. During each episode of hyperpermeability of the vasculature, there is an increase of interleukin 2 receptor-positive peripheral blood M-cell count, as well as apoptosis of the endothelial cells.

Pathophysiology

Capillary leak syndrome also known as Clarkson's disease and spontaneous periodic edema is a life threatening condition characterized by recurrent episodes of hypovolemic shock, hemoconcentration, and hypoalbuminemia, occurring in the absence of albuminuria.

The pathogenesis of capillary leak syndrome remain unclear, however, cytokines, vascular endothelial growth factor (VEGF), leukotrienes, and complement play an important role. During each episode of hyperpermeability of the vasculature, there is an increase of interleukin 2 receptor-positive peripheral blood M-cell count. The exact cause of hyperpermeability remains unknown, and protein up to 900 kDa can extravasate from the intravascular to interstitial space. During the hyperpermeability episode, endothelial cells undergo apoptosis, which is characterized by endothelial microvascular body and bleb formation.