Disease pathophysiology: Systemic capillary leak syndrome is a rare disorder characterized by self-reversing episodes during which the endothelial cells which line the capillaries are thought to separate for a few days, allowing for massive leakage of plasma and other blood components from the blood vessels into neighbouring body cavities and muscles. Systemic capillary leak syndrome usually consists of two phases i.e capillary leak phase and recruitment phase. This leads to swelling. Systemic capillary leak syndrome leads tohypotension, hemoconcentration, and hypoalbuminemia. It is a life threatning syndrome often misdiagnosed with polycythemia,polycythemia vera, or sepsis.
Treatment: Mainly the intravenous administration of saline solution plus hetastarch or albumin and colloids (to increase the remaining blood flow to vital organs like the kidneys), as well as glucocorticoids (steroids like methylprednisolone, to reduce or stop the capillary leak). However, it is important to avoid overly aggressive intravenous fluid administration during this leak phase, because it may cause massive swelling of the extremities and thus serious collateral damage because of induced compartment syndromes.
he patient was diagnosed with SCLCS and treated with therbutaline and aminophylline. Research: Major research is been performed by NORD (National Organization of Rare Disorders) Statistics: SCLS is a very rare and devastating condition. According to reports of a United Kingdom registry, the median annual frequency of ISCLS was 1.23 (range: 0.13-21.18) per patient for all attacks and 0.46 (range: 0.00-14.12) per patient for severe attacks. The fatality rate during acute attacks of ISCLS is not well defined. In a review of ISCLS, 70-76% of patients who survived the initial attacks were alive a mean of 5 years after diagnosis. ISCLS is discussed below in terms of pathophysiology, complications, and treatment.