Systemic capillary leak syndrome

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Kemas kini Tarikh 11/05/2020 . 3 minit bacaan
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What is systemic capillary leak syndrome?

Systemic capillary leak syndrome (SCLS) is a condition in which fluid and proteins leak out of tiny blood vessels, into surrounding tissues. This can result in dangerously low blood pressure (hypotensison), hypoalbuminemia, and a decrease in plasma volume (hemoconcentration). Initial symptoms may include fatigue, nausea, abdominal pain, extreme thirst, and sudden increase in body weight. Episodes of SCLS vary in frequency, with some people having one episode in their lifetime, and others having several per year. The severity also varies, and the condition can be fatal.

This condition is also called Clarkson’s disease.

How common is systemic capillary leak syndrome?

Systemic capillary leak syndrome is a rare disorder. Please discuss with your doctor for further information.


What are the symptoms of systemic capillary leak syndrome?

The common symptoms of systemic capillary leak syndrome are:

  • 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.

There may be some symptoms not listed above. If you have any concerns about a symptom, please consult your doctor.

When should I see my doctor?

If you have any signs or symptoms listed above or have any questions, please consult with your doctor. Everyone’s body acts differently. It is always best to discuss with your doctor what is best for your situation.


What causes systemic capillary leak syndrome?

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.

Risk factors

What increases my risk for systemic capillary leak syndrome?

Some people with SCLS report having had an infection (such as a virus) before the episode. Some cases of capillary leak are associated with various conditions of the heart, kidney, or liver.

Diagnosis & treatment

The information provided is not a substitute for any medical advice. ALWAYS consult with your doctor for more information.

How is systemic capillary leak syndrome diagnosed?

Systemic capillary leak syndrome is diagnosed based on physical examination, laboratory tests, and recurrence of symptoms. Recurring episodes are associated with monoclonal gammopathy in the majority of patients (when an abnormal immunoglobin protein is found in the blood). The diagnosis is supported by ruling out other illnesses with similar symptoms, including secondary capillary leak syndrome or abnormally low levels of protein in the blood (hypoproteinemia).

How is systemic capillary leak syndrome treated?

There is currently no cure for systemic capillary leak syndrome (SCLS). Treatment for SCLS during an episode is mainly supportive, aiming to stabilize symptoms and prevent severe complications. This may involve stabilizing the airway and breathing, taking certain medications, and/or intravenous (IV) infusion of fluids, medications, or blood products. Some have suggested that IV fluids are not recommended because it may worsen swelling (edema) or increase the risk for other complications. Once a person begins to recover, diuretics are almost always required.

Periodic infusions or the use of certain medications are used to try to prevent future episodes (prophylactic therapy). Having monthly infusions of intravenous immune globulin (IVIG) has been suggested. A study published in 2017 found that preventive treatment with IVIG was the strongest factor associated with survival in people with SCLS. Those who do not improve with IVIG or who cannot tolerate the therapy may have success with theophylline and terbutaline.

Research into how SCLS develops and factors that affect its course are underway, in hopes that a better understanding of SCLS will lead to more effective treatment options and a lower mortality rate.

Lifestyle changes & home remedies

What are some lifestyle changes or home remedies that can help me manage systemic capillary leak syndrome?

Please discuss with your doctor for further information.

If you have any questions, please consult with your doctor to better understand the best solution for you.

Hello Health Group does not provide medical advice, diagnosis or treatment.

Hello Health Group tidak memberikan nasihat perubatan, diagnosis atau rawatan.

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