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.

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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
  • Shock
  • Lightheadedness
  • Weakness
  • Fatigue
  • Nausea

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.

  • Expertise and experience. Mayo Clinic is one of only a few medical centers in the world with expertise in systemic capillary leak syndrome. Each year our team of blood experts (hematologists) diagnoses and treats a significant number of people who have this rare disorder. In addition, Mayo Clinic conducts research on this condition and shares knowledge with others.
  • Multidisciplinary approach. If you have systemic capillary leak syndrome, you may experience complex medical problems involving many body systems. Mayo Clinic's multidisciplinary team collaborates to treat the whole person and provide exactly the care you need.

At Mayo Clinic, we assemble a team of specialists who take the time to listen and thoroughly understand your health issues and concerns. We tailor the care you receive to your personal health care needs. You can trust our specialists to collaborate and offer you the best possible outcomes, safety and service.

Mayo Clinic is a not-for-profit medical institution that reinvests all earnings into improving medical practice, research and education. We're constantly involved in innovation and medical research, finding solutions to improve your care and quality of life. Your doctor or someone on your medical team is likely involved in research related to your condition.

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At Mayo Clinic, specialists in hematology coordinate the care of your systemic capillary leak syndrome and include other specialists as appropriate.

For appointments or more information, call the Central Appointment Office at 800-446-2279 (toll-free) 8 a.m. to 5 p.m. Mountain Standard Time, Monday through Friday or complete an online appointment request form.

At Mayo Clinic, specialists in hematology coordinate the care of your systemic capillary leak syndrome and include other specialists as appropriate.

For appointments or more information, call the Central Appointment Office at 904-953-0853 8 a.m. to 5 p.m. Eastern time, Monday through Friday or complete an online appointment request form.

At Mayo Clinic, specialists in hematology coordinate the care of your systemic capillary leak syndrome and include other specialists as appropriate.

For appointments or more information, call the Central Appointment Office at 507-538-3270 7 a.m. to 6 p.m. Central time, Monday through Friday or complete an online appointment request form.

See information on patient services at the three Mayo Clinic locations, including transportation options and lodging.

Systemic capillary leak syndrome is difficult to diagnose. You may undergo blood and urine tests and imaging studies. Your doctors may diagnose sudden, periodic "attacks" of this condition by seeing the following signs, symptoms and laboratory abnormalities and excluding other conditions that could cause them:

  • Concentrated blood
  • Low serum albumin in the blood
  • Presence of a distinct monoclonal protein in the blood or urine
  • Generalized body swelling
  • Muscle aches
  • Lightheadedness
  • Fatigue
  • Decreased or dark urine

Systemic capillary leak syndrome frequently causes complex medical problems involving many body systems. Episodes are typically sudden, although sometimes certain warning signs or symptoms can alert you.

An attack may include a marked drop in blood pressure. If not treated promptly, this can lead to multiple organ failure and death.

Treatment may include:

  • Fluids. You receive fluids intravenously, carefully controlled to maintain your blood pressure and prevent damage to vital organs, such as your kidneys, heart and brain.
  • Medications. You may receive a prescription for steroids, water pills (diuretics), immunoglobulin or other drugs. You'll probably need to take medications long term to reduce the frequency and severity of future episodes.

Clinical trials are research studies that test new ways to detect, prevent or treat disease. Mayo Clinic conducts more than 3,000 clinical trials and research studies each year and often coordinates national clinical trials with other medical centers.

Learn more about clinical trials and whether Mayo Clinic may be conducting a clinical trial related to your condition or procedure.

Mayo Clinic researchers study new diagnostic procedures and treatments for people who have blood conditions that affect vessels and capillaries.

Nov. 26, 2014