Preferred Label : Systemic capillary leak syndrome;
ICD-11 definition : 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
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,
the standard of preventive therapy is now periodic infusions of intravenous immunoglobulin
(IVIG), which clinical experience suggests prevents the acute episodes.;
ICD-11 synonym : SCLS - [Systemic capillary leak syndrome]; Clarkson disease;
ICD-11 acronym : SCLS;
Origin ID : 1008326816;
Automatic exact mappings (from CISMeF team)
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
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,
the standard of preventive therapy is now periodic infusions of intravenous immunoglobulin
(IVIG), which clinical experience suggests prevents the acute episodes.
