What is Systemic Capillary Leak Syndrome (SCLS)?
SCLS is a rare, life-threatening condition characterized by recurrent and unexplained episodes of edema and hypotension; so-called "leak episodes." Episodes of leak are typically preceded by non-specific symptoms like fever, abdominal pain, or malaise.
Why is SCLS so difficult to diagnose?
SCLS is very difficult to diagnose because it is so rare and has clinical features that overlap with more common conditions such as sepsis, anaphylaxis, renal failure, Addisons disease, or inborn errors of immunity. The defining laboratory findings of hypoalbuminemia, increased hematocrit and hypotension lack specificity for SCLS. Episodes of leak may be separated by months or years, increasing the time it takes to make the correct diagnosis.
What kind of research does your lab do in connection to SCLS?
My research program is focused on understanding blood vessel dysfunction in the setting of acute critical illness. We perform genetic sequencing, immune profiling, and vascular function assays on samples we obtain from critically ill children, including those with SCLS. We hope to understand why blood vessel dysfunction occurs in children with SCLS to help prevent leak episodes as well as to improve our understanding of how more common diseases alter blood vessel function.
Are there ways pediatric SCLS differs from adult SCLS?
There are two main reasons to think children with SCLS have a different type of disease than adults. First, almost all adults have abnormally high levels of M-protein in their blood, a condition called Monoclonal Gammopathy of Undetermined Significance (MGUS). Children with SCLS do not have MGUS. Second, leak episodes in children seem to be triggered by mild viral illnesses more so than in adults. Although these are two differences that suggest pediatric and adult SCLS may be different, more research is needed to establish what these differences mean.
What kinds of treatments are available for SCLS patients?
The initial treatment during a leak episode is limited to supportive care, sometimes even in the pediatric intensive care unit. No medicines have been shown to reduce the severity or duration of the leak episodes. In between episodes, monthly infusions of intravenous immunoglobulin (IVIG) are very effective at preventing leak episodes.
To learn more about SCLS, watch Dr. Pierce's learning module on SCLS above.