Systemic Capillary Leak Syndrome

What is Systemic Capillary Leak Syndrome?

When individuals have this health disorder, they have a leakage in their blood vessels. The proteins and fluids that seep out of the blood vessels travel to various tissues in the body. As this occurs, a person’s blood pressure plunges very low and if left untreated, this can cause organ failure and eventually become fatal.

The exact cause of this disorder is not completely understood, but it is not an inherited syndrome. Many individuals who are diagnosed with systemic capillary leak syndrome have a small amount of M protein identified in their blood. Another theory is that chemicals found in the body impair the cells that line the capillary walls.

What are the Symptoms of Systemic Capillary Leak Syndrome?

The first symptoms, which are coughing and congestion, are often inaccurately diagnosed as a respiratory illness. As the symptoms progress, individuals will become nauseated and lightheaded, and they will have a headache and stomach pain.

It is also common for the extremities, such as the legs and arms, to become swollen. Individuals can faint and go into shock if their blood pressure drops to an unsafe level. People who have chronic systemic capillary leak syndrome may have repeated bouts of fluid retention in various cavities of their body.

Systemic Capillary Leak Syndrome Causes

Systemic Capillary Leak Syndrome (also known as Clarkson’s Disease or spontaneous periodic edema) is the result of low levels of monoclonal (M) proteins spontaneously produced by the patient’s blood causing capillaries to leak fluid, resulting in edema of organs, particularly the lungs, and a rapid, critical loss of blood pressure.

Hypotension is caused by the separation of the cells lining the capillaries which allows fluid to leak from the capillaries into the interstitial space. Acute attacks can cause permanent damage to vital organs (through blood loss and oxygen deprivation) and can be fatal. SCLS is not hereditary but may be the result of an autoimmune disorder. It does not appear specifically in one racial group over another. It is also possible that it is caused by some sort of damage to the cells lining the capillaries. SCLS is more prevalent in men and older patients, but is very rare.

How is a Systemic Capillary Leak Syndrome Treated?

One of the most important steps of treatment is to maintain a healthy blood pressure level to keep the vital organs functioning properly. This is accomplished through substantial amounts of colloid and albumin, which are given through intravenous fluids.  While administering the fluids, physicians must monitor the amount of swelling in the body to prevent too much fluid from building up in the extremities or in the lungs.

To help get rid of surplus fluids, diuretics may be given to the individual. Additional medications include glucocorticoids, terbutaline and theophylline. In some cases, leukotriene inhibitors must be prescribed if the other medications are not effective.

Systemic Capillary Leak Syndrome Prevention

Because the exact cause of Systemic Capillary Leak Syndrome is not known and the condition is so rare (there are less than 100 known cases in the world at any given time), prevention of the disease is not really feasible. Prevention rests in the prevention of acute attacks, as those attacks are extremely dangerous and potentially fatal. A hormone like cytokines is the first line of treatment in preventing acute attacks. A maintenance therapy that is typically employed to prevent attacks is a combination of the medications theophylline and terbutaline. Once an acute attack begins, permanent damage and death are prevented through the use of high volumes of intravenous fluids to maintain blood pressure and administering albumin and colloids to stabilize the cells in the capillaries.

Last Reviewed:
October 11, 2016
Last Updated:
November 28, 2017