Cerebral Involvement in Systemic Capillary Leak Syndrome

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

Idiopathic systemic capillary leak syndrome in children.

Adult subjects with systemic capillary leak syndrome (SCLS) present with acute and recurrent episodes of vascular leak manifesting as severe hypotension, hypoalbuminemia, hemoconcentration, and generalized edema. We studied clinical disease characteristics, serum cytokine profiles, and treatment modalities in a cohort of children with documented SCLS. Six children with SCLS were recruited from ...