Escape Syndrome Disease: What is it, Causes, Symptoms and Treatment

Systemic capillary escape syndrome, also known as escape syndrome, is a disorder that was first described in 1960 by a physician named Bayard Clarkson. Therefore, one of the names of this disorder is Clarkson disease. It may have a very bad course due to the shock state that develops during the course of the disease. Therefore, it is important to be aware of this disorder.

What is Escape Syndrome Disease?

Escape syndrome is a very rare disease characterized by attacks in which a large portion of the plasma portion of the blood escapes out of the vessel into areas such as the spaces in the surrounding tissues or the inside of the muscle tissue. As a result of these attacks, a sudden drop in the person’s blood pressure occurs, and if the resulting shock is not treated, the condition may first result in organ failure and then death.

This disorder may have similar characteristics to severe infections such as septic shock or severe allergic reactions such as anaphylactic shock. Therefore, it is very important to distinguish between escape syndrome and these similar conditions. Attacks in escape syndrome can sometimes be triggered by an upper respiratory tract infection or physical fatigue. The frequency of attacks may vary from person to person. While in some patients, an attack of escape syndrome may manifest itself only as a single attack, in some patients it may occur as several attacks per year.

What are the Symptoms of Escape Syndrome?

Dr. As a result of Clarkson’s examination of individuals with this disorder; He found that sudden onset of fever, a sudden drop in blood pressure, and a rapid development of edema, especially evident in the face, legs, and arms. Following these developments, people experience excessive urination, which usually begins within a few days, and as a result of frequent urination, the edema in the person’s arms and legs may be relieved. In some patients, edema in the lungs can lead to heart failure and lead to a fatal course.

The symptoms that may occur with escape syndrome may be similar to many other diseases:

• Unrest
• Weakness
• Stomach ache
• Nausea
• Muscle pains
• excessive thirst
• A sudden increase in body weight

Complaints such as nasal congestion and cough, which are triggered by upper respiratory tract infection, are among the symptoms that may warn about an escape syndrome attack.

As long as the blood in the vein continues to escape into the surrounding tissues, blood volume and pressure continue to decrease. As a result, there may be a need for oxygen and nutrients in the tissues of organs such as the kidneys, brain and liver. As a result of inadequate blood supply to these organs, multiple organ failure associated with hypoperfusion (insufficient blood supply) or a tendency to clot due to blood concentration due to fluid loss in the blood circulation and edema affecting the whole body may occur.

What Causes Escape Syndrome?

Although the exact cause of escape syndrome is not known exactly, the underlying cause may be an inflammatory condition. Various studies in Europe have found that individuals with this disease develop flu-like symptoms in the time immediately preceding their attacks. One of the main triggers of the disease may be upper respiratory tract disorders caused by agents such as influenza, RSV or West Nile virus. Apart from this infectious disease, factors such as physical exhaustion or long-term travel in extremely hot environments may also act as triggers.

As a result of the development of escape syndrome, these patients experience a rapid shock and edema that affects the whole body. This attack, called the “escape phase” because approximately 70% of the plasma volume of blood escapes the vessel, can continue for several days.

The severity and frequency of escape syndrome attacks may vary from person to person and over time. In general, situations such as a decrease of 60 mmHg in blood pressure and loss of consciousness, which will cause the person to be taken to intensive care, indicate that the disease has a severe course. In some people, the drop in blood pressure is not so pronounced and people show moderate symptoms such as mild weakness, dizziness and excessive thirst.

How to Diagnose Escape Syndrome?

The basic diagnostic criteria used to diagnose escape syndrome are called the “3H” finding. Hypotension (low blood pressure), hemoconcentration (blood becomes more dense) and hypoalbuminemia (decrease in the protein called albumin in the bloodstream) create this “3H” finding. Another important stage of diagnosis is that these conditions cannot be explained by another disorder. It is typical for people to experience bilateral edema in their face, trunk, arms and legs after fluid restriction. In the examinations performed after the blood darkens, it is determined that the person’s hemoglobin level has reached a very high level.

In people suspected of having this disease, routine blood and urine tests are requested at the first stage of the diagnostic approach to exclude other possible conditions. Although various subsequent immunological studies may result in findings indicating this disease, they are not specific for escape syndrome. Therefore, the diagnosis of escape syndrome is made within the framework of the person’s general clinical condition.

Further examinations in some patients may be useful in clarifying the underlying cause of findings such as hypotension and edema. While urine tests may be beneficial in cases of edema and protein deficiency that occur due to deterioration of kidney functions, examinations such as lung x-ray, electrocardiography (ECG) and echocardiography (ECHO) can be used to detect heart failure that may occur in some people.

How is Escape Syndrome Treated?

Systemic capillary leak syndrome is a rare disease characterized by sudden onset and severe attacks. Attacks begin when the blood volume in the veins escapes and a sudden hypotension (low blood pressure) occurs in patients. Attacks, which usually last for a few days, may require urgent intervention as they can cause very serious consequences.

The underlying cause of escape syndrome has not yet been elucidated, so there is currently no targeted treatment for this disorder. Treatment in these patients basically aims to control the conditions that may occur and to prevent severe conditions that may occur during the course of the disease.

In people suspected of having this disease, treatment is carried out under intensive care conditions. An attempt is made to prevent organ failure by giving blood intravenously to individuals at a level that provides sufficient intravenous blood volume. Patients given very high amounts of fluid are carefully monitored.

In some cases, monthly intravenous immunoglobulin administration (IVIG treatment) is an approach that can be beneficial in preventing attacks that may occur in the future. Some medications that are used as part of preventive treatments but are normally used for asthma treatment may also be beneficial. It should not be forgotten that these drugs also have annoying side effects such as hand tremors.

The general treatment of escape syndrome is actually a supportive treatment. Although regularly applied IVIG treatment seems promising in some patients, it has the disadvantages of being a very expensive treatment method. If you observe signs and symptoms in your body of this disorder, for which there is currently no targeted treatment, it is recommended that you contact the nearest health institution and get support from specialist physicians.