Clinical Trial: Studies in the Pathogenesis of Systemic Capillary Leak Syndrome

Study Status: Recruiting
Recruit Status: Recruiting
Study Type: Observational



Official Title: Studies in the Pathogenesis of Systemic Capillary Leak Syndrome

Brief Summary:

Background:

  • Systemic Capillary Leak Syndrome (SCLS) is a disorder of unknown cause characterized by episodes of life-threatening drop in blood pressure and leakage of fluids into tissues. The outcome from an episode of SCLS may be mild and resolve on its own, or may be severe and result in death. Although SCLS likely involves abnormalities in the cells lining blood vessels, the specific cause(s) of this disorder are not known.
  • The treatment of choice for an acute SCLS episode is intravenous fluids and drugs such as norepinephrine (adrenaline), which are given to keep blood pressure at a level that will maintain vital organ function. This may be followed by a course of intravenous steroids and IVIG. Currently, there is no cure, but IVIG has been effective in diminishing the frequency and/or intensity of SCLS episodes when given regularly, as long-term effective preventive therapy for many patients who experience recurrent episodes of SCLS.
  • This protocol is focused on understanding what causes SCLS with the hope that research findings will lead to the design of safe and more effective treatments.

Objectives:

- To investigate mechanisms that may cause Systemic Capillary Leak Syndrome.

Eligibility:

  • Patients between 16 and older who have been diagnosed with SCLS. Patients who have been diagnosed with SCLS and are between the ages of 7 and 16 may participate off-site, by sending specimens to the NIH. Patients 16 and older who have been diagnosed with SCLS and cannot travel to the NIH may also participate off-site.