Systemic Capillary Leak Syndrome Presenting With Fulminant Recurrent Cardiogenic Shock

Recurrent undifferentiated shock: Idiopathic Systemic Capillary Leak Syndrome

Idiopathic Systemic Capillary Leak Syndrome is a potentially fatal disorder that is under diagnosed. It commonly presents as recurrent undifferentiated shock with hypotension, hypoalbuminemia and hemoconcentration. There are three distinct phases that define the syndrome; Prodromal, Extravasation and Recovery.

Systemic capillary leak syndrome.

A 40-year-old woman was referred to our hospital with severe hypovolemic shock and anasarca. The laboratory findings showed marked hemoconcentration and a decrease in total serum protein with the presence of monoclonal IgG-lambda. She had had a similar episode of generalized edema 2 years previously. We diagnosed the patient as having typical systemic capillary leak syndrome (SCLS) and she impr...

SyStemic capillary leak Syndrome

Results: Of the 34 patients whose records were reviewed, 25 fulfilled all diagnostic criteria for SCLS. The median age at diagnosis of SCLS was 44 years. Median follow-up of surviving patients was 4.9 years, and median time to diagnosis from symptom onset was 1.1 years (interquartile range, 0.5-4.1 years). Flulike illness or myalgia was reported by 14 patients (56%) at onset of an acute attack ...

Pulmonary Capillary Leak Syndrome

Systemic capillary leak syndrome associated with compartment syndrome.

Systemic capillary leak syndrome is characterized by recurrent hypovolemic shock attributable to increased systemic capillary leakage. A 26-year-old woman was admitted because of recurrent episodes of hypovolemic shock. Hemoconcentration, hypoalbuminemia, and monoclonal gammopathy were observed. We diagnosed systemic capillary leak syndrome. Three years later, she again had an attack of systemi...