Distributive shock due to systemic capillary leak syndrome treated with high-dose immunosuppression

Severe cerebral involvement due to idiopathic systemic capillary leak syndrome

The idiopathic systemic capillary leak syndrome (ISCLS) is a rare disorder, characterized by recurrent attacks of hypotension, hypoalbuminemia, and hemoconcentration, which is often misdiagnosed due to overlapping features with other diseases. Even though cerebral involvement is uncommon, a broad awareness is crucial, because of its life-threatening character.

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

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.