Systemic capillary leak syndrome is a rare disorder characterized by recurrent flares of massive leakage of plasma and other blood components from blood vessels into neighboring body cavities and muscles. This leads to swelling. The symptoms result from a sudden and unexplained increase in the leakiness of small blood vessel (capillary) walls. Unless treated, massive fluid shifts result in a sharp drop in blood pressure that can lead to organ failure and death.
This condition is also called Clarkson's disease.
Systemic capillary leak syndrome may be triggered by an upper respiratory tract infection. Signs and symptoms of systemic capillary leak syndrome may include:
- Sudden swelling (edema) of the arms, legs and other parts of the body
- Rapid drop in blood pressure
Fluid may collect around the heart, lungs and soft tissues, causing a potentially life-threatening situation. You may faint due to a rapid drop in blood pressure from fluid leakage. Your doctor will likely have you undergo blood and urine tests, which will help in making a diagnosis. Such tests may reveal:
- An increased concentration of red blood cells, due to leakage of plasma
- A decreased concentration of a protein called albumin
- The presence of a distinct form of protein called monoclonal protein
No one knows exactly what causes this disorder. It doesn't appear to be inherited. One study reports that it is caused by chemicals in the body that damage or temporarily separate the cells lining the capillary walls. This separation allows the walls to become leaky.
With treatment, you may be able to control the disorder and avert attacks for long periods of time. If you have a long-term (chronic) form of the condition, you may experience persistent edema and fluid collection in body cavities without the sudden flares typical of other types of systemic capillary leak syndrome.
Nov. 26, 2014
- Druey KM, et al. Narrative review: The systemic capillary leak syndrome. Annals of Internal Medicine. 2010;153:90.
- Kapoor P, et al. Idiopathic systemic capillary leak syndrome (Clarkson's disease): The Mayo Clinic Experience. Mayo Clinic Proceedings. 2010;85:905.
- Riggin EA. Decision Support System. Mayo Clinic, Rochester, Minn. June 25, 2014.
- Gousseff M, et al. The systemic capillary leak syndrome: A case series of 28 patients from a European registry. Annals of Internal Medicine 2011;154:464.
- Xie Z, et al. Vascular endothelial hyperpermeability induces the clinical symptoms of Clarkson disease (the systemic capillary leak syndrome). Blood. 2012;119:4321.