Catalog

Systemic capillary leak syndrome

No MMS code

Link to WHO Definition

Systemic capillary leak syndrome – http://id.who.int/icd/entity/1008326816

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 250 cases of SCLS have been reported, but the condition is probably underrecognised because of its nonspecific symptoms and signs and high mortality rate. Patients experience shock and massive oedema, 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 oedema reverse almost as quickly as they begin, at which time patients are at risk for death from flash pulmonary oedema 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 under perfusion. Between episodes, the standard of preventive therapy is now periodic infusions of intravenous immunoglobulin (IVIG), which clinical experience suggests prevents the acute episodes.