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 to hypotension, 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.
The 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: There are less than 100 patients reported in the world literature since its first description in 1960 by Clarkson. SCLS seems to occur more often in males and older adults. The disease may be more frequent than the literature suggests because the diagnosis is often missed or delayed. SCLS may be mistaken for a severe infection such as septic shock or toxic shock syndrome. Some features such as the swelling may lead one to suspect heart failure or kidney disease. C-1 esterase inhibitor deficiency syndrome may present with a type of recurring edema called angioedema and is often thought of in patients presenting with SCLS. In some cases the hemoconcentration and resulting high hematocrit and hemoglobin level have been mistaken for polycythemia.