Capillary leak syndrome is a disease associated with generalized edema. The condition occurs with low frequency in the population and may cause serious complications. In some cases, capillary leak syndrome is also known as Clarkson’s syndrome. The cause of capillary leak syndrome is usually that the capillary vessels are too permeable, allowing plasma and associated proteins to enter what is called the interstitium.
What is capillary leak syndrome?
Capillary leak syndrome was first described in 1960 by the American physician Clarkson. For this reason, the condition is sometimes called Clarkson syndrome. Basically, it is a comparatively rare disease. Thus, up to the year 2002, only 57 patients have been documented to have capillary leak syndrome. It can be seen that the affected persons were on average 46 years old. The age range extended from nine to 67 years. No difference was found between the sexes; instead, capillary leak syndrome occurs with equal frequency in males and females. In some circumstances, the disease even affects patients in childhood. In English, capillary leak syndrome is most commonly referred to as capillary leakage syndrome, from which the international abbreviation CLS is derived.
Causes
To date, the exact causes of the development of capillary leakage syndrome have not been adequately explored, so certain factors and mechanisms of disease genesis remain unknown. Some speculations assume that specific types of cytokines are responsible for the capillary vessels becoming more permeable. For example, the substance interleukin-2 is under discussion as a potential factor. Other physicians assume that the planned death of cells (medical term apoptosis) or so-called leukotrienes are involved in the development of capillary leak syndrome. In particular, apoptosis of endothelial cells is in focus. In addition, there are speculations about a genetic component of capillary leak syndrome. This point of view is mainly supported by the fact that hereditary family strains have been identified in one patient. For this reason, it seems likely that the disease, or at least a predisposition to capillary leak syndrome, is inherited. In addition, the majority of affected individuals exhibit monoclonal gammopathy. In most cases, this is the IgG subtype. For this reason, some physicians suspect that this monoclonal gammopathy is involved in the development of capillary leak syndrome. However, mutant plasma proteins do not accumulate within the interstitium.
Symptoms, complaints, and signs
In the setting of capillary leak syndrome, intermittent hypovolemic shocks occur that are severe and extremely severe. Basically, these are so-called volume-deficiency shocks that are accompanied by edema of a generalized nature. In addition, individuals suffering from capillary leak syndrome suffer from hypotension of the arteries associated with thickening of the blood. The cause of this thickening is due to the loss of plasma water from the blood. In addition, hypoalbuminemia is involved in its development. Within the framework of this phenomenon, the percentage of the protein albumin in the plasma of the blood is reduced. The cause of this condition is the greatly increased permeability of the capillary blood vessel walls. As a result, blood plasma passes into the interstitial space. Basically, each interval of capillary leak syndrome is divided into two stages. In the initial phase, affected patients suffer, on the one hand, from general symptoms such as nausea and pain in the abdominal region. On the other hand, hypotension of an arterial nature develops, as well as generalized edema. These symptoms last for a period of a few days. A feared complication during this period is a collapse of the cardiovascular system. In addition, there is a risk of kidney failure or damage to the tubule because the blood volume is too low. In some cases, there is also a condition called rhabdomyolysis, in which the striated fibers within the muscles disintegrate. During the subsequent phase, the fluid substance that has leaked from the capillaries moves.As a result, polyuria develops, with patients excreting excessive amounts of urine. In addition, the formation of edema in the lungs or so-called water lung is possible.
Diagnosis and course of the disease
The diagnosis of capillary leak syndrome focuses on the presenting clinical symptoms. It is particularly important to perform a thorough history together with the respective patient. In the subsequent phase of the examination, the treating physician analyzes the individual symptoms. For example, blood and urine tests are used. The blood values often provide important indications of the presence of capillary leak syndrome.
Complications
Capillary leak syndrome can cause significant patient dysfunction and complications. For this reason, treatment of the affected individual is usually necessary. In most cases, patients first suffer from pain in the abdominal area and further also from nausea. It is not uncommon for the capillary leak syndrome to continue to cause problems with the cardiovascular system, so that a complete collapse can occur. Renal insufficiency can also occur, which can be life-threatening for the affected person. In this case, the patient is dependent on a donor kidney or must undergo dialysis. The quality of life is considerably reduced by the capillary leak syndrome. If necessary, life expectancy can also be reduced. Unfortunately, there is no direct treatment for capillary leak syndrome, so there are no complications. It may be possible to limit the symptoms with the help of medication. However, a positive course of the disease cannot be guaranteed. It is not uncommon for capillary leak syndrome to cause psychological symptoms, so that those affected are dependent on psychological treatment. Thereby, depression and other psychological upsets can be avoided.
When should you see a doctor?
Problems of the circulatory system must be examined and treated by a doctor in sufferers of any age. Since in severe cases they can lead to collapse as well as premature death, a doctor should be consulted at the first irregularities of the cardiovascular system. If water retention is noticed in the legs or other parts of the body, there is also cause for concern. A physician should be consulted to clarify the symptoms. Breathing sounds or disturbances in breathing activity may be indications of water in the lungs. The affected person should consult a physician to determine the cause of the impairment. If the complaints generally increase in nature and extent, a visit to the doctor is required as soon as possible. In case of dizziness, nausea, vomiting or pain in the abdominal region, a doctor should be consulted. If there is a general feeling of illness, internal weakness or malaise, further investigations should be initiated. If functional disorders develop, a doctor must be consulted immediately. Without medical care, organ failure is imminent. The kidneys are particularly at risk in capillary leak syndrome. A drop in blood pressure, a decreased heartbeat or disturbances in blood flow indicate irregularities and other diseases. If there is numbness in the limbs or cold fingers as well as toes, a doctor should be consulted.
Treatment and therapy
Because very few cases of capillary leak syndrome have been observed to date, treatment methods are relatively limited. This is because there is a lack of appropriate research studies and results on the efficacy of various therapeutic approaches. Basically, no standard treatment of capillary leak syndrome exists at the present time. For prophylaxis, the agents theophylline and terbutaline have been tested, some of which have shown good efficacy. If capillary leak syndrome has a more chronic course in individual cases, treatment with glucocorticoids is promising. These include diuretic agents, for example.
Outlook and prognosis
Self-help for capillary leak syndrome, because of the risk of new hypovolemic shock, must always be closely coordinated with the treating physicians. The first measure should be a change in the previous diet plan to a healthy and balanced diet.Since the layperson often lacks the experience for this, it is advisable to seek the support of a nutritionist who is familiar with the disease in question. A well-chosen diet strengthens the immune system, but can also reduce the onset of symptoms such as nausea or pain and thus contribute to an improvement in life. In addition, avoiding stress, regular moderate exercise and plenty of rest help to relieve the body between two shock reactions. It is advisable to keep a complaints diary in which new symptoms and other abnormalities are noted. This ensures that the attending physician is always informed about the overall condition during the close-meshed examinations. This makes it easier for him to adjust the medication to the individual needs and to prevent possible further shock reactions. Since affected children are not yet able to communicate adequately until a certain age, it is incumbent on the parents in these cases to pay particular attention to possible new symptoms or abnormalities and to make note of them.
Prevention
No effective methods of preventing capillary leak syndrome have yet been researched.
Follow-up
Because capillary leak syndrome is a serious and severe disease, it should be evaluated by a physician at the first signs and symptoms. As a rule, it cannot be cured on its own, so that those affected are always dependent on a medical examination and treatment in order to alleviate the symptoms in the long term. In most cases, direct aftercare measures are severely limited or not available to the affected person at all. As a rule, those affected by capillary leak syndrome are dependent on taking various medications. It is always important to ensure that the medication is taken regularly and in the correct dosage to prevent further complications. Likewise, a doctor should always be consulted immediately if there are any questions or if anything is unclear. A healthy lifestyle with a balanced diet can have a positive effect on the course of capillary leak syndrome, so that in the process a diet plan can be drawn up by a doctor, which should be followed. In many cases, contact with other patients of the syndrome is also useful, as this can lead to an exchange of information.
This is what you can do yourself
In capillary leak syndrome, treatment focuses on relieving symptoms. For some symptoms, patients can support drug therapy with their own measures. Initially, rest and bed rest are recommended. The interval-like shock reactions place an enormous strain on the body. For this reason, affected patients need a lot of rest, accompanied by moderate exercise, avoidance of stress and regular medical examinations. Relatives and parents of affected children should keep a close eye on the sufferer in order to be able to react quickly in the event of a recurrence of hypovolemic shock. Drug treatment must be regularly readjusted to the current state of health of the affected person. For this purpose, the patient should keep a diary in which he or she records any unusual symptoms. This makes it easier for the doctor to adjust the medication. Finally, recovery can be promoted by changing the diet. A healthy and balanced diet can reduce initial symptoms such as nausea and pain in particular. In addition, a healthy lifestyle strengthens the immune system. As a result, the intensity and frequency of shock reactions decrease in the best case. Affected individuals should also drink plenty of fluids to counteract thickening of the blood.
