What is the definition of Systemic Capillary Leak Syndrome?
Systemic capillary leak syndrome (SCLS) causes fluid and proteins to leak out of tiny blood vessels (capillaries) into surrounding tissues. This may lead to very low blood pressure (hypotension), hypoalbuminemia, and thickened blood due to a decrease in plasma volume (hemoconcentration). Initial symptoms may include tiredness, nausea, abdominal pain, extreme thirst, and sudden increase in body weight. Complications can include general swelling, compartment syndrome, kidney failure, and stroke. SCLS occurs in episodes which 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. In many cases, the cause is not known (idiopathic SCLS). Diagnosis is based on the symptoms, clinical examination, and other laboratory tests. Treatment is focused on managing the symptoms during an episode and preventing future episodes.
What are the alternative names for Systemic Capillary Leak Syndrome?
- Clarkson disease
- Capillary leak syndrome
- Capillary leak syndrome with monoclonal gammopathy
- Periodic systemic capillary leak syndrome
What are the causes for Systemic Capillary Leak Syndrome?
The cause of systemic capillary leak syndrome is unknown. It is thought to be due to an abnormal response of the immune system to an infection or illness.
What are the symptoms for Systemic Capillary Leak Syndrome?
The following list includes the most common signs and symptoms in people with systemic capillary leak syndrome. These features may be different from person to person. Some people may have more symptoms than others, and they can range from mild to severe. This list does not include every symptom that has been described in the condition.
Symptoms of systemic capillary leak syndrome may include:
- Low blood pressure
- Loss of albumin from the blood
- Thickened blood
- General swelling
- Fluid in the lungs and around the heart
- Kidney failure
- Heart failure
Systemic capillary leak syndrome occurs in episodes. The number and severity of episodes can be different from person to person. Before an episode of SCLS occurs, people may have general weakness, tiredness, stomach pain, and weight gain. During an episode, symptoms may include very low blood pressure, general swelling, fluid in the lungs and around the heart, and kidney failure. Long-term complications may include compartment syndrome, muscle breakdown, and heart and lung failure.
What are the current treatments for Systemic Capillary Leak Syndrome?
Treatment for systemic capillary leak syndrome (SCLS) is focused on managing the symptoms during an episode and preventing long term complications. During an episode, treatment involves stabilizing the airway and breathing using medications, hydration, and oxygen therapy.
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. Those who do not improve with IVIG or who cannot tolerate the therapy may have success with theophylline and terbutaline.
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). Other conditions may need to be excluded before systemic capillary leak syndrome can be diagnosed.