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What is Systemic Capillary Leak Syndrome?
Systemic Capillary Leak syndrome is a rare disorder due to increased capillary permeability. This increased permeability will cause plasma and other blood components from blood vessels to leak into near by body cavities and muscles, leading to swelling. If untreated, this will lead to a significant drop in blood pressure that may result in organ failure and death.

What is the prevalence of Systemic Capillary Leak Syndrome?
SCLS has an extremely low prevalence rate; less than 1 in 1 million people will be affected by this disease. The onset of SCLS will usually occur in adults, however, SCLS can affect people of all ages.

How is Systemic Capillary Leak Syndrome diagnosed?
SCLS is diagnosed based on measurable symptoms such as hypotension, hemoconcentration, hypoalbuminemia, and the presence of a protein called Monoclonal Gammopathy of Unknown Significance (MGUS). However, SCLS is difficult to diagnose. Some diagnosis tests may include blood and urine tests to check for abnormalities such as dark urine, concentrated blood, or low serum albumin in the blood.

Is there any specific gene/pathway in Systemic Capillary Leak Syndrome that has been identified?
The gene that causes SCLS has yet to be confirmed, but whole genome sequencing has been done on the disorder. Current clinical trials suspect that SNP clusters in PON1, PSORS1C1, and CHCHD3 may be affected.

How is Systemic Capillary Leak Syndrome treated?
There is no curative treatment for Systemic Capillary Leak Syndrome; however, there are several general treatments available. The most common treatment is albumin and extracellular fluid administration, which is often supplemented with medications such as theophylline, terbutaline sulfate, and corticosteroids to alleviate symptoms and reduce the frequency of severe attacks.

Are there any clinical trials underway for Systemic Capillary Leak Syndrome?
Yes, the National Institute of Allergy and Infectious Diseases (NIAID) is currently recruiting patients to participate in a study on the pathogenesis of Systemic Capillary Leak Syndrome. More information on the clinical trial can be found here.

How can RareShare be helpful to Systemic Capillary Leak Syndrome patients and families?
RareShare Systemic Capillar Leak Syndrome Community provides support and information for patients, families, and friends. It currently has 282 discussion topics and 266 community members.

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