Systemic Capillary Leak Syndrome

Overview

Mayo Clinic is one of the few facilities in the world with the expertise to diagnose and treat systemic capillary leak syndrome (Clarkson's disease). Since 1980, Mayo Clinic in Rochester has seen 25 patients with this extremely rare disease, more than any other medical center.

In partnership with the National Institutes of Health, Mayo Clinic is investigating capillary leak syndrome's causes and developing improved treatment options. Patients who have systemic capillary leak syndrome frequently experience complex medical problems involving many body systems. Mayo Clinic's integrated group practice enables a Mayo physician to quickly assemble a team of specialists to address all of the patient's medical needs.

Systemic capillary leak syndrome is very difficult to diagnose. Early symptoms are similar to those of a common cold. As the condition progresses, blood pressure drops because of fluid leakage. Patients subsequently develop light-headedness and painful swelling of the extremities and other parts of the body. Read more about systemic capillary leak syndrome diagnosis.

Systemic capillary leak syndrome causes a rapid drop in blood pressure that, if not treated in an intensive-care setting, can lead to multiple organ failure and death within hours. Fluids are infused to maintain blood pressure. Drug treatments are often injected to reduce or stop the capillary leak, and surgery may be necessary to prevent nerve and muscle damage to the limbs. Over the long term, medications are usually prescribed to reduce the frequency and/or severity of episodes. Read more about systemic capillary leak syndrome treatment.

About

Systemic capillary leak syndrome is a very rare disorder characterized by massive leakage of plasma from blood vessels into adjacent body cavities and muscles. The leakage causes a sharp drop in blood pressure, which can lead to organ failure and death.

The reason for this leakage is a sudden and unexplained change in the capillary walls which allows blood to pass through them. There are no known causes of capillary leak syndrome, and it does not appear to be inherited. Among the hypotheses for its cause is an acquired autoimmune deficiency or a chemical that damages or temporarily separates the cells lining the capillary walls. The condition occurs most often in adults ages 40 to 60. It is rare in children.

Treatment can cause the disease to go into remission for weeks or even years. However, capillary leak syndrome is highly unlikely to disappear entirely.