Narrative review: the systemic capillary leak syndrome.

[systemic capillary leak syndrome]

The systemic capillary leak syndrome (SCLS ) is a rare disease of reversible plasma extravasation and vascular collapse accompanied by hemoconcentration and hypoalbuminemia . Its cause is unknown , although it is believed to be a manifestation of transient endothelial dysfunction due to endothelial contraction , apoptosis , injury , or a combination of these . Fewer than 150 cases of SCLS have been reported , but the condition is probably underrecognized because of its nonspecific symptoms and signs and high mortality rate . Patients experience shock and massive edema , often after a nonspecific prodrome of weakness , fatigue , and myalgias , and are at risk for ischemia-induced organ failure , rhabdomyolysis and muscle compartment syndromes , and venous thromboembolism . Shock and edema reverse almost as quickly as they begin , at which time patients are at risk for death from flash pulmonary edema during rapid fluid remobilization . Diagnosis is made clinically and by exclusion of other diseases that cause similar symptoms and signs , most notably sepsis , anaphylaxis , and angioedema . Acute episodes are treated with vasopressor therapy and judicious fluid replacement , possibly with colloid solutions for their osmotic effects , to prevent the sequelae of underperfusion . Between episodes , patients may be treated with theophylline and terbutaline , which clinical experience suggests may reduce the severity and frequency of acute episodes . Prognosis is uncertain , but patients who survive an initial severe SCLS episode are estimated to have a 10 -year survival rate greater than 70 %. Much remains to be learned about SCLS , and clinicians should consider the diagnosis in patients with unexplained edema , increased hematocrit , and hypotension .

Diseases
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Diseases presenting "acute episodes" symptom

erythropoietic protoporphyria

heparin-induced thrombocytopenia

systemic capillary leak syndrome

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