Systemic capillary leak syndrome

Overview

Systemic capillary leak syndrome is a rare disorder characterized by repeated flares of massive leakage of plasma from blood vessels into neighboring body cavities and muscles. This results in a sharp drop in blood pressure that, if not treated, can lead to organ failure and death.

Also called Clarkson's disease, this condition can be mistaken for severe reactions to widespread infections (septic shock) or severe allergic reactions (anaphylactic shock). Attacks may be triggered by an upper respiratory infection or intense physical exertion. The frequency of attacks can range from several a year to a single instance in a lifetime.

Symptoms

Attacks of systemic capillary leak syndrome are often preceded by one to two days of one or more nonspecific symptoms that may include:

• Irritability
• Fatigue
• Abdominal pain
• Nausea
• Muscle aches
• Increased thirst
• Sudden increase in body weight

As the fluid leaks out from the bloodstream, blood volume and blood pressure drop. This can starve tissues in the kidneys, brain and liver of the oxygen and nutrients they need for normal function.

Treatment

The cause of systemic capillary leak syndrome is unknown, and there's no known cure. Treatment during episodes aims to stabilize symptoms and prevent severe complications. The use of intravenous fluids must be monitored carefully.

Monthly infusions of intravenous immunoglobulin (IVIG) can help prevent future episodes. Preventive treatment with certain oral medications originally designed to treat asthma also can be helpful, but these drugs may produce troublesome side effects, such as tremors.