Kawasaki disease in children - photos, symptoms of virus infection, causes and treatment

Kawasaki disease - what is it? Symptoms and treatment

Kawasaki disease (Kawasaki syndrome, mucocutaneous lymphatic syndrome) belongs to a group of diseases called systemic vasculitis. Diseases in this group cause abnormal damage by cells of the immune system to blood vessels, most often medium- and small-sized arteries and veins. In the case of Kawasaki syndrome, it can cause damage to children's coronary arteries - the vessels that carry oxygen-rich blood and nutrients to the heart.

In addition to the coronary arteries, the inflammatory process can affect the pericardium, which surrounds the heart, as well as endocardial tissue (the inside of the heart), and even the heart muscle itself.

Kawasaki disease most often affects children, usually under 5 years of age. Older children and adults are rarely exposed to it. The peak incidence occurs between the ages of 1 and 2 years, and boys are more often affected.

Symptoms

• Diarrhea.
With Kawasaki syndrome, all organs can be affected, so in addition to the above points, joint pain, diarrhea, vomiting, headache, painful urination or chest pain may occur.

By far the most dangerous complication is inflammation of the blood vessels of the heart. If there is a deficiency in the supply of oxygen to the myocardium, a heart attack may develop, the typical symptoms of which are chest pain with radiation to the arm, chest tightness and shortness of breath. Therefore, intensive monitoring of cardiac condition is always necessary to detect serious complications in a timely manner.

A few days later a rash appears on the body. It can develop anywhere and does not have a specific pattern, it can be in the form of red spots or blisters. A few days later she disappears without a trace. Due to dilated capillaries, pink or burgundy spots appear on the body.

Lips become red, dry and cracked. When examining the tongue, you will notice that it has increased in size, turned red, its papillae are also enlarged, which in appearance resembles a ripe strawberry.

The lymph nodes become swollen, especially noticeable in the neck area.

Kawasaki disease. Pathophysiology

Despite the prominent changes in the lining of the blood vessels that define the disease, Kawasaki disease is best thought of as a generalized vasculitis that affects the small and medium-sized arteries. Although vascular inflammation is most pronounced in the coronary vessels, vasculitis can also occur in veins, capillaries, small arterioles, and large arteries.

In the early stages of the disease, the endothelial cells become swollen, but the inner elastic layer remains unchanged. Then, approximately 7-9 days after the onset of fever, there is an influx of neutrophils followed by a rapid expansion of CD8+ cytotoxic lymphocytes and immunoglobulin A-producing plasma cells.

Inflammatory cells secrete various cytokines (i.e., tumor necrosis factor, vascular endothelial growth factor, monocyte chemotaxis, and activation factors), interleukins (IL-1, IL-4, IL-6), and matrix metalloproteinases (primarily MMP3 and MMP9), which target endothelial cells. All these factors lead to a cascade of events leading to fragmentation of the internal elastic layer of blood vessels and damage to the vessels themselves. In severely damaged vessels, inflammation develops with necrosis of smooth muscle cells. After this, the inner and outer layers of the vessels begin to separate, which leads to an aneurysm.

Over the next few weeks or months, active inflammatory cells are replaced by fibroblasts and monocytes, resulting in the formation of fibrous connective tissue in the vessel wall. These layers will increase and thicken. The vessel walls eventually become narrowed or occluded due to stenosis or thrombosis. Ultimately, a person may have a myocardial infarction secondary to thrombosis of a coronary aneurysm or from the rupture of a large coronary aneurysm.

Pathology treatment methods

There are currently no radical therapy methods. This disease cannot be treated with steroids or antibiotics. The only effective treatment for Kawasaki disease is intravenous injections of acetylsalicylic acid and immunoglobulin simultaneously.

Thanks to immunoglobulin, pathologies occurring in the vessels along with inflammatory processes are stopped, which thereby prevents the formation of aneurysms. Acetylsalicylic acid, in turn, reduces the risk of blood clots by providing an anti-inflammatory effect. In addition, both drugs help reduce body temperature, eliminating fever and alleviating the patient's condition. Additionally, the patient may be prescribed anticoagulants in accordance with the doctor's indications in order to prevent the occurrence of thrombosis. These are, as a rule, Warfarin and Clopidogrel.

Symptoms

Clinical signs of the syndrome are varied and specific:

• Skin damage is manifested by the appearance of a rash, the elements of which are polymorphic. They look like small flat red spots, large blisters, measles-like rashes without vesicles and crusts. The rash is localized mainly on the torso, limbs, and groin. Elements of the rash tend to merge, forming erythematous areas. On the soles and palms, the skin becomes tight and painful, which leads to limited mobility of the fingers. After about a week, the rash begins to fade and gradually disappears, leaving flaky patches on the skin.
• Inflammation of the mucous membrane of the eyes occurs as conjunctivitis without discharge. Patients experience bilateral vascular injection of the sclera and conjunctiva without lacrimation or corneal ulceration. The mucous membrane in the mouth turns red and dries, cracks appear on the lips, which periodically bleed. The tongue becomes crimson, the tonsils are enlarged. Often the cervical lymph nodes are involved in the pathological process, and regional lymphadenitis develops. A single painful node appears, the diameter of which exceeds 1.5 centimeters.
• In persons with vasculitis, the functioning of the heart is disrupted, and serious changes are observed in the functioning of the entire vascular system. Patients develop myocarditis, manifested by rapid heartbeat, shortness of breath, cardialgia, and arrhythmia. The disease progresses steadily and often leads to acute heart failure. Inflammation may spread to the pericardium. Coronary artery aneurysms are the cause of myocardial infarction.
• Articular syndrome occurs in every third patient. Large joints of the legs and small joints of the hands are usually affected.
• Inflammation of the digestive organs is manifested by abdominal pain, dyspeptic symptoms, stool disorders, and enlarged liver.
• In some cases, the membranes of the brain and organs of the urinary system are affected.

Vivid symptoms of the disease persist for two months, and then recovery occurs. In the absence of timely treatment, the syndrome progresses and severe complications develop:

1. Acute coronary insufficiency,
2. Hemopericardium,
3. Endocarditis with damage to the mitral valve,
4. Inflammation of the heart valves
5. Hydrocele of the gallbladder,
6. Hepatitis,
7. Pancreatitis,
8. Myositis,
9. Aseptic inflammation of the meninges,
10. Joint damage
11. Inflammation of the middle ear,
12. Sensorineural deafness,
13. Gangrene of the limbs.

How does the infection progress?

Pathology has four forms of occurrence:

• Flu-like syndrome. Another name is Flu-Like. This pathology has the mildest form. Characteristic symptoms are fever, muscle pain, discomfort in the bones and general malaise. Signs of the disease are observed within three days. Next comes recovery. Influenza-like syndrome rarely causes complications, but only with qualified therapy;
• Intestinal infection. Children under two years of age most often suffer from this form of pathology. With such a disease, it is not necessary that other children will become infected from the child. In a group, this case may be exclusive and no one else may suffer from it. Characteristic symptoms are high fever, discomfort in the bones and general weakness of the body. In the first days of infection, other signs are observed - intestinal upset, nausea accompanied by vomiting. For this reason, the disease is often confused with poisoning. The period of exacerbation is 2-3 days for children of primary school age and a week for newborns. This disease can cause milk intolerance during the first time after recovery. This makes it necessary to give the child medical lactose before breastfeeding or to refuse it;
• Boston Pathology. The disease manifests itself in the same way as rubella, which makes it difficult to diagnose. A child, like an adult infected with the Coxsackie virus, in this case becomes covered with formations of a red hue. Symptoms: high fever, rashes on the epidermis in the form of blisters. The treatment period is 3-5 days. After this, recovery begins. With this form, relapse rarely occurs, and complications are most often not observed;
• Pleurodynia. Another name is Bronholm's disease. Symptoms: high fever, severe pain in the back, abdomen, muscles and chest. Spasms occur at regular intervals – once every 60 minutes. The duration of the attack is from 1 to 20 minutes. They cause spasms in the pleura and peritoneum, which cause pain during friction.

With any form of pathology, the patient needs urgent medical care.

Coxsackie viruses are divided into two large groups

There are two groups of pathology:

• Type A virus: Infection with the Coxsackie virus causes damage to the throat and may cause meningitis.
• Virus type B. Affects the brain, muscles and heart, causing irreversible changes in these internal organs.

Course options: mild, moderate and severe

Pathology has varying degrees of progression. The severity of the disease depends on how much the mucous membranes and internal organs are damaged. The degree is also affected by damage to the body by toxins.

The mild form of the pathology is characterized by a rapid course. Recovery occurs within a few days. In this case, the virus does not affect the patient’s internal organs and brain.

With moderate severity, therapy takes a little more than a week. The pathology has pronounced symptoms. The virus can affect internal organs, but with qualified treatment the problem can be quickly resolved.

The severe form of the disease is characterized by pronounced symptoms and a long course. This causes damage to the brain and internal organs.

Experts also identify the following variants of the pathology:

• smooth – recovery occurs quickly and the disease does not recur;
• wavy - the pathology either fades or flares up with renewed vigor;
• recurrent – ​​after recovery, the disease develops again;
• with complications – the disease is difficult to treat and causes complications.

Forms of the disease

There are two forms of pathology:

• Isolated. The disease occurs in one syndrome.
• Combined. In this case, the Coxsackie virus affects several organs and systems at once.

How is Kawasaki syndrome diagnosed?

Doctors make the diagnosis solely on the basis of the clinical picture. Thus, a diagnosis may be made if an unexplained high fever lasts five or more days along with four of the following five signs: bilateral conjunctivitis (inflammation of the membrane that covers the eyeball), swollen lymph nodes, skin rash, lesions of the mouth and tongue, and related changes in limb areas. But at the same time, the doctor must make sure that there are no signs of any other disease that may have the same symptoms.

If symptoms are detected, doctors conduct an ECG and echocardiography, the patient is prescribed testing to exclude other diseases (complete blood count, blood tests for ESR, C-reactive protein, antinuclear antibodies (ANA), rheumatoid factor (RF), albumin, liver enzymes, smear culture from the throat and blood culture, urinalysis, chest x-ray).

Development mechanism

The violation was and remains quite mysterious. Scientists have not come to a consensus about the etiology of this process or its origin. However, there is some progress in terms of research.

There is a group of theories regarding the pathogenesis of Kawasaki syndrome.

Hereditary factor

The vast majority of experts adhere to the version that the disorder has genetically determined features. This is supported by, among other things, racial, point and very selective preferences of the state. The Japanese suffer, more often than other Asians.

Why is that? Apparently, the predisposition is transmitted with certain genes, with a whole group. Which leads to an increase in morbidity in the population.

It has not yet been possible to say exactly how likely the disease is in a person with a complicated medical history. Research continues.

Infectious mechanism

This theory is based on the assumption that viruses (herpes, in particular Epstein-Barr) or bacteria such as staphylococci are to blame.

Here the opinions of experts are divided:

• Some suggest that the disorder is purely septic in nature. That is, it begins with damage to the arterial walls by the abnormal agents themselves.
• Others believe that any infectious focus can become a trigger. Trigger mechanism. And it is not necessary for a virus or bacteria to infect blood vessels.

And here comes the final perspective on Kawasaki disease.

Autoimmune mechanism

Proponents of this theory believe that septic agents do not directly provoke the syndrome. We are talking about increasing the sensitivity of the body.

If a bacterium or virus lasts long enough, the defenses fail. Weakening positions. The risk of their malfunction increases.

Ultimately, the immune system attacks its own cells and tissues. Causes inflammation. In this case, Kawasaki disease becomes even closer to vasculitis.

Most researchers adhere to some kind of synthetic theory. All three mechanisms play a role in etiology. Heredity and exposure to infection are the main culprits. They provoke disorder in the system.

The doctor explained Kawasaki syndrome in children with coronavirus

Some scientists associate the Kawasaki phenomenon with COVID-19, while others suggest that the manifestations of this syndrome are a consequence of the cancellation of routine vaccinations in children due to the pandemic. What do Russian experts think about this? What causes this Kawasaki syndrome? And how relevant is this problem for Russia?

MK’s questions are answered by the head of the department of diagnostics and rehabilitation treatment, head of the Center for Infectious Diseases of the Federal State Institution “National Medical Research Center for Children's Health” of the Ministry of Health of Russia, Doctor of Medical Sciences, Professor Maya BAKRADZE.

Photo: medq.ru

“At least five signs should alert you”

“This hyperinflammatory syndrome in children is an extremely rare phenomenon and the background against which it occurs is still unknown,” explained Maya Dzhemalovna. — In essence, it is a systemic vasculitis with predominant damage to medium and small arteries, which leads to the development of aneurysms and the formation of blood clots. Kawasaki syndrome was first identified and described in Japan in 1967. The name was given to it by the Japanese doctor Tomisaku Kawasaki; for a long time he observed and treated children who exhibited these symptoms.

— And what symptoms are most characteristic of this syndrome? How to distinguish it from other manifestations of diseases? After all, a rash on the body and high fever are characteristic of many childhood pathologies...

“There are many other, more serious signs of this disease that must be taken into account when making a diagnosis. At least five signs should alert you: high temperature (up to 40°C and above); changes in the eyes (scleritis); rash; peripheral edema (hands and feet); bright raspberry tongue; lymphadenitis (enlarged lymph nodes, especially cervical ones), often unilateral. The main difficulty in diagnosing this syndrome is that all 5 signs rarely appear: more often, a combination of two or three. But in all cases, it is necessary to urgently begin treatment in order to eliminate inflammation of the coronary arteries and the formation of blood clots by the 10th day of the disease. And these are risk factors for myocardial infarction and death.

- And this is for small children? Are there any effective medications against this mysterious Kawasaki?

— Yes, up to 90% of those admitted with this syndrome are children under 5 years old. The risk of severe consequences in most of them can be prevented if large doses of immunoglobulin are administered intravenously in time in combination with acetylsalicylic acid. But the problem is that often the symptoms are erased. And so far no one in the world has been able to establish a single cause of the disease. To this day, research is being conducted in different countries to determine the source of the disease in children. But a scientific substantiation of the reasons for the appearance of this “strange” syndrome has not yet been found.

Even American colleagues, in their studies of new cases of Kawasaki syndrome during the COVID-19 pandemic, divided patients into two groups: children who developed Kawasaki syndrome after a previous covid infection, and children who developed the syndrome independently, even without contact with the new coronavirus infection.

— Still, viruses are to blame for this? So, maybe the reason for the increase in Kawasaki syndrome in children in Europe was COVID-19? After all, this factor began to be identified more often during the pandemic and mainly in patients with coronavirus?

— There is no proven data yet. Whether they are viruses, bacteria or other pathogens is not known for certain to the global medical community. Unfortunately, no one has yet proven how this hyperinflammatory syndrome occurs.

— Or maybe the reason is that now, due to the pandemic, children have temporarily stopped receiving routine vaccinations, for example, against chickenpox. And has this caused the rise of Kawasaki syndrome?

— A temporary suspension of vaccination cannot provoke the development of such a syndrome, because this year the immunization of children in the world has not been carried out for only a month or two, in Russia - for one month. This is not enough for the child’s body to react in this way. At least a year must pass.

“COVID-19 acts as a trigger”

— Maya Dzhemalovna, are children with coronavirus currently being treated in your clinic? How is their recovery going? Are there any patients with dangerous Kawasaki syndrome among them?

“Fortunately, there are no children with this syndrome in our clinic. And with a suspected or confirmed diagnosis of COVID-19, about 400 children were admitted to us for treatment (since April 21). To date, approximately half of the young patients have been discharged. Today there are 150 such children in the clinic, 20 of them are in more serious condition. But there are medicines, the treatment regimen proposed by the Ministry of Health gives positive results.

The clinical guidelines approved in 2021 by the Russian Ministry of Health regarding “Mucocutaneous lymphonodular Kawasaki syndrome in children” state: “In Russia, Kawasaki syndrome is being diagnosed more and more often, but often at a later date, as a result of which treatment is prescribed untimely and not always adequately. According to an epidemiological study conducted in the Irkutsk region from 2005 to 2009, the average incidence rate was 2.7 per 100 thousand children aged 0–17 years and 6.6 per 100 thousand children under 5 years of age, while the authors admit that these figures may be underestimates. Approximately 90–95% of cases are children under 10 years of age; 85–90% of cases occur in patients under 5 years of age. Infants aged 9–11 months are most often affected. The peak incidence occurs in the winter-spring period.”

Also, the Russian Ministry of Health does not exclude the viral nature of Kawasaki syndrome. “The factors causing Kawasaki disease have not yet been precisely established; a possible combination of genetic predisposition and exposure to certain viruses is assumed,” the department’s clinical recommendations say.

By the way, in our country, since 2003, Kawasaki syndrome began to be studied at the Department of Childhood Diseases of Sechenov University. More than 300 patients with this diagnosis have passed through the hands of scientists. And the preliminary conclusion is this: “It is possible that coronavirus may be the same trigger for Kawasaki as streptococcus, staphylococcus and other viruses,” noted Natalya Golovanova, a professor at the department of childhood diseases at this university. She does not exclude that during the COVID-19 pandemic, “the risk of disease is indeed higher.”

...And while scientists have doubts, in Western countries, against the backdrop of the COVID-19 pandemic, this hyperinflammatory Kawasaki syndrome, dangerous for children, continues to be increasingly detected. More than a hundred such cases have already been recorded. Moreover, at the same time, many of these children are also diagnosed with coronavirus. Experts, including Russian ones, come to the conclusion that a connection between these two diseases does exist.

And the World Health Organization (WHO) is urging doctors everywhere to “pay particular attention to reports that some children infected with coronavirus are showing symptoms similar to another disease, Kawasaki syndrome (multisystem inflammatory syndrome).” According to WHO experts, “according to preliminary data, the development of Kawasaki syndrome may be associated specifically with infection with coronavirus. The COVID-19 infection itself does not cause the disease, but acts as a trigger, causing changes in the functioning of the immune system.”

...Or perhaps the new coronavirus began to mutate in this way?

Symptoms

The first sign of Kawasaki disease is a sharp increase in temperature to 38-40 °C. The fever cannot be controlled with conventional antipyretics and lasts about 5 days. If untreated, the fever persists for 14 days, and an increase in the duration of the febrile period is considered an unfavorable prognostic symptom.

After a few days, the child begins to show other signs of Kawasaki disease.

Skin and mucous membranes

Eyes

Most children develop conjunctivitis within the first 7 days. It is usually not accompanied by the eye discharge characteristic of this disease, and in some cases is accompanied by anterior uveitis.

Airways

The child develops dry mouth and bleeding cracks on the lips. The tongue becomes swollen and crimson, the pharynx turns red, and the tonsils become enlarged. In addition, swelling of the nasal mucosa occurs, manifested by nasal congestion.

Leather

One of the characteristic signs of this pathology is a rash localized on the torso, lower extremities and groin.

In the first 5 weeks after the onset of the disease, various polymorphic and diffuse lesions of the skin occur:

• rash in the form of red spots of different sizes;
• blisters;
• measles-like or scarlet-like rash.

The localization of the rash is usually typical, its elements are found on:

• torso;
• lower parts of the arms and legs;
• groin area.

After some period of time, the child develops erythematous changes and thickened areas on the soles and palms. They interfere with the normal movement of the fingers and after a while they begin to peel off.

With Kawasaki disease, the reverse development of the rash begins 7 days after its appearance, and erythematous spots persist for 2-3 weeks.

The lymph nodes

In about half of children, Kawasaki disease is accompanied by enlarged lymph nodes in the neck. As a rule, it is one-sided.

Digestive system

Abnormal bowel movements (in the form of watery diarrhea), abdominal pain and vomiting may appear in the initial period of the disease. Some children may develop intestinal obstruction or intussusception in the acute stage.

For 2 weeks, a child with Kawasaki disease may experience symptoms of a special form of cholecystitis, which is accompanied by vomiting, diarrhea, and abdominal pain (even hepatic colic). In some cases, the disease provokes the development of pancreatitis.

Nervous system

Signs of damage to the nervous system in this disease are usually observed in children under one year of age. They may present with symptoms of serous meningitis or Guillain-Barré syndrome. As a rule, they occur in the acute period against the background of fever and can manifest as isolated paralysis, hearing impairment and fainting.

With high disease activity, children may develop neuromuscular diseases - myopathies, myositis, damage to the diaphragm muscles.

Joints

In approximately 35% of cases, Kawasaki disease is accompanied by joint damage - arthralgia and arthritis. Joint syndrome often lasts no more than a month. Usually the knees, ankles and small joints of the feet and hands are affected.

Heart and blood vessels

Damages to the cardiovascular system in Kawasaki disease manifest themselves in the form of myocarditis, causing pain in the heart, increased heart rate and arrhythmias. Often pathological processes cause the development of acute heart failure.

Approximately 1.5-2 months after the onset of the disease, the patient may develop aneurysms of the coronary vessels, provoking myocardial infarction. Sometimes aneurysmal enlargements form on the walls of other arteries: subclavian, ulnar, femoral.

These manifestations of Kawasaki disease are the most dangerous to life and health. As a rule, the reverse development of aneurysms occurs within a month, but the high risk of death from thrombosis and myocardial infarction persists for 3 months.

In more rare cases, Kawasaki disease provokes the development of other cardiovascular pathologies:

• cardiomegaly;
• hemopericardium;
• valvulitis;
• mitral or aortic insufficiency;
• pericarditis.

Against the background of all the symptoms described above, children with Kawasaki disease become restless, overly irritable or lethargic.

Acute febrile stage

It begins with a sharp increase in body temperature.
After 1-3 days, the main symptoms of the disease appear. Rashes appear 1-3 days after the temperature rises. Fever without chills and sweating lasts 5-30 days (on average 8.5 days). Fever begins suddenly, reaches 38-40 C and does not respond to the use of antibiotics and antipyretics. Almost all external manifestations of Kawasaki disease occur at this stage. Red spots first appear on the palms and soles and within 2 days cover the trunk and limbs.

Palms and soles

Torso and limbs

Dermatitis in the diaper area

Conjunctiva of the eye

Lips and throat

Language

Cervical lymphadenopathy

Firm, non-tender, non-purulent lymphadenopathy is often limited to a single cervical lymph node. Enlarged cervical lymph nodes (at least one node exceeds 1.5 cm in diameter).

Other symptoms.

Diarrhea, liver dysfunction, aseptic meningitis, arthralgia, arthritis, urethritis, photophobia.

Subacute stage

Leather.

After the rash resolves, lamellar peeling begins. Desquamation on the hands and feet begins 10-14 days after the onset of fever. The skin sheds in sheets, starting from the areas around the nails and fingertips and progressing down to the palms and soles. It extends proximally from the fingertips (from the nail folds) and completely covers the palms and soles.

Other symptoms

.Irritation of the meninges (meningeal symptoms). Pneumonia. Arthralgia, arthritis of the knee, hip and elbow joints.

Recovery period

Beau's lines (transverse grooves on the nails) and telogen effluvium may appear.

Damage to the cardiovascular system

Kawasaki disease is the leading cause of acquired heart disease in children in the United States. Clinical cardiac symptoms are present in 16.3% of patients. In the early course of Kawasaki disease, more than 50% of patients develop myocarditis with tachycardia and a gallop rhythm. These arrhythmias sometimes lead to sudden death. In the subacute phase, the formation of aneurysms of medium-sized arteries, in particular the coronary arteries, is found in approximately 1/4 of patients. These lesions may be persistent, heal with stenosis, or resolve.

Aneurysms and blood clots form between 12 and 25 days after illness onset and can lead to subsequent heart failure, pericardial effusion, arrhythmias, or rupture of aneurysms. Abnormalities peak at 3 weeks of illness and often resolve thereafter. Complications heart disease are most common in male patients, in infants under 1 year of age, and in children over 5 years of age. Boys under 1 year of age with prolonged fever, elevated platelet counts, and high ESR are at greater risk of heart disease.

Course and prognosis

Most patients recover. Cardiovascular complications occur in 20%. Coronary artery aneurysms develop 2-8 weeks after the onset of the disease; they are often combined with myocarditis, myocardial infarction, pericarditis, peripheral artery stenosis, intestinal ischemia and intestinal obstruction, and stroke. The mortality rate is 1%. The main causes of rapid and long-term deaths are manifestations of cardiovascular failure. The worst prognosis is for children with so-called giant aneurysms, i.e. with aneurysms with a diameter of more than 8 mm. Almost 10% of children fail to achieve clinical improvement with treatment. Relapses are rare.

Clinical picture

Patients with Kawasaki syndrome go through 3 stages:

1. Acute febrile – from 7 to 10 days.
2. Subacute – from the 2nd to the 3rd week.
3. The recovery period ranges from a month to several years.

Symptoms of Kawasaki disease in children - photo:

First, the patient’s body temperature rises, as with a normal ARVI, and a fever begins. If left untreated, fever lasts up to 2 weeks. The longer this period lasts, the less chance of recovery.

Then problems with the skin arise: red spots, swelling of the skin, blisters, rashes, etc. The skin on the soles of the feet and palms may become thicker, which reduces the mobility of the fingers. These symptoms persist for about 2-3 weeks, then the skin peels off.

Damage also occurs to the mucous membrane of the mouth and eyes. In the first week, patients develop conjunctivitis in both eyes without discharge.

The mucous membrane suffers from dryness and bleeding, for example from the gums, the lips burst and crack, the tongue takes on a crimson tint, and the tonsils increase in size. In half of the cases there is a significant increase in the size of the cervical lymph nodes.

The following symptoms appear from the heart and coronary system:

• myocarditis;
• heart failure;
• arrhythmia;
• tachycardia;
• chest pain;
• vascular aneurysms;
• myocardial infarction;
• pericarditis;
• mitral insufficiency.

In every third case of the disease, patients suffer from damage to the joints in the ankle, knees, and hands.

Diarrhea, abdominal pain, nausea, vomiting, and sometimes meningitis or urethritis are also possible.

Kawasaki disease in children Symptoms?

Symptoms of Kawasaki disease include:

• Fever lasting at least 5 days.
• Red eyes.
• Rash on the body.
• Swollen, red lips and tongue.
• Swollen, red legs and arms.
• Enlarged lymph nodes in the neck For your part, try to seek medical help immediately if your child has at least one of the symptoms of Kawasaki disease. Early diagnosis and proper treatment most often prevent future problems related to the heart.

What kind of disease is this?

Kawasaki syndrome is a generalized inflammation of blood vessels with an autoimmune, i.e., arising due to one’s own immunity, nature. It is sometimes also called mucocutaneous lymph node syndrome or periarteritis nodosa.

The essence of the disease is damage to arteries and veins of medium and smaller diameter, which leads to the development of aneurysms, stenoses, and hemorrhages.

The disease was described in a monograph by Tomisaku Kawasaki in 1967, and the name was later given in honor of him.

He identified a group of sick children with the same symptoms:

• skin rash;
• long fever;
• bilateral conjunctivitis;
• enlarged cervical lymph nodes;
• swelling.

At first, the pathology was called “cutaneous mucocutaneous lymphatic syndrome,” but in 1970, lesions of the coronary vessels were also identified.

The peak incidence of Kawasaki syndrome occurs between 12 and 24 months of life, but manifestations have also been recorded before the age of 5 years. In Russia and the CIS countries, the pathology is very rare, most often found in Japan.

In adults, Kawasaki syndrome is recorded in isolated cases, therefore the disease relates to pediatrics and is considered exclusively for children. In adulthood, the course is similar, its severity depends on the primary infection.

It has been established that the main link in pathogenesis is the reaction of antibodies to endothelial cells and viruses. Antibodies attack blood vessels and cause inflammatory processes in the middle layer of the wall, causing the development of smooth muscle necrosis.

This process leads to the formation of aneurysms and ruptures, which can be accompanied by heart attacks and strokes. Most often the cardiac, subcutaneous, renal, axillary, and periovarian arteries are affected.

Kawasaki disease is always accompanied by fever - a temperature of about 40 degrees can remain throughout the course of the disease, so the body experiences severe exhaustion. The arteries of the eye also become severely inflamed, which leads to conjunctivitis. The peculiarity of its course is always a bilateral manifestation.

Constant bleeding due to rupture of aneurysms can provoke the collateral development of disseminated intravascular coagulation syndrome with the formation of a large number of blood clots and subsequent massive hemorrhage. The rheumatological prognosis in this case will be disappointing.

What is Kawasaki syndrome?

Kawasaki syndrome is a mucocutaneous lymphonodular syndrome that manifests as necrotizing systemic lesions of medium and small arteries. The disease is characterized by fever, changes in the mucous membranes, skin, damage to the coronary and other visceral arteries with the possible formation of aneurysms, thrombosis and ruptures of the vascular wall.

Kawasaki syndrome affects the arteries and can cause serious heart complications. According to the medical reference, coronary artery damage occurs in approximately 20% of untreated patients.

The disease was first described in the English-language medical literature in 1967 by a Japanese pediatrician named Tomisaku Kawasaki . He identified a group of children with fever, skin rash, conjunctivitis, redness of the throat and mouth, swelling of the arms and legs, and enlarged lymph nodes in the neck. And a few years later, patients experienced heart complications, such as coronary artery aneurysms (expansion of the lumen of these blood vessels).

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Symptoms of the disease

Kawasaki disease has 3 successive stages of development.

1. Initial, acute febrile. It lasts 1–1.5 weeks.
2. Subacute. Occurs 2–3 weeks after the active manifestation of the disease.
3. Recovery period. The duration of this stage is from 4 weeks to several years.

The appearance of the first signs of the disease is always accompanied by a prolonged fever. Typically, a child has a febrile fever for more than 5 days. If the cause of the fever is not identified in a timely manner and treatment of the disease is not started, a high temperature may be observed in the child for about 14 days. In addition, babies have the following symptoms:

• red spots, rashes and blisters on the skin of the arms and legs. They are most noticeable in the palms and heels;
• swelling of the limbs, accompanied by decreased mobility of the fingers. The most severe swelling is observed on days 3–5 of illness;
• severe peeling of the skin;
• conjunctivitis in both eyes. With Kawasaki disease, there is no pus or other pathological discharge from the eyes;
• enlarged cervical lymph nodes. The diameter of each of them is more than 15 mm;
• bleeding gums;
• redness of the eyes;
• the skin of the lips becomes dry and constantly cracks;
• ulcers on the inside of the cheek;
• the tongue turns a bright crimson color.

If there are 4 or more symptoms of the disease, the child is diagnosed with Kawasaki syndrome. If 3 or fewer signs of the disease appear, the doctor will diagnose a disease with an incomplete clinical picture.

The following symptoms of pathology may be observed in the functioning of internal organs and systems:

• vascular aneurysms;
• periodic pain in the chest area;
• myocardial infarction;
• pericarditis;
• heart failure;
• myocarditis;
• tachycardia;
• mitral insufficiency.

In addition to the specific signs of Kawasaki disease, a child may experience nausea and vomiting, diarrhea, and severe stomach discomfort. In some cases, the syndrome is accompanied by urethritis or meningitis.

“Kawasaki syndrome” in children after COVID-19: What you need to know

Reports began to appear in news feeds that in Kazakhstan, children who have had mild or asymptomatic coronavirus are developing a new disease - Kawasaki syndrome, although medical scientists call this disease multisystem inflammatory syndrome (MSI), temporarily associated with COVID-19 in children and adolescents. Chairman of the Board of JSC "Scientific Center of Pediatrics and Children's Surgery" Riza Boranbaeva told a zakon.kz correspondent about the symptoms, treatment and causes of this disease.

As a rule, 80% of children suffer from coronavirus in a mild or asymptomatic form. A child passes a positive PCR test for coronavirus, after some time a repeat test gives a negative result, and antibodies to coronavirus are even detected in children’s blood. But after 2-3 weeks, sometimes earlier, against a healthy background, the child may develop MWS, temporarily associated with COVID-19. It begins with an increase in body temperature, at this time various organs and systems are affected, a cough and shortness of breath may appear. Symptoms similar to real Kawasaki disease include a spotted rash, red tongue, enlarged red lips, swelling of the palms and feet. In addition, with MWS, the child may develop conjunctivitis and enlarged lymph nodes. Children may also complain of pain in the chest, in the heart area, abdominal pain (as with appendicitis), vomiting, nausea, and diarrhea.

A child with MBC temporarily associated with COVID-19 is not infectious. He should not be hospitalized in an infectious diseases hospital; he should be hospitalized in a multidisciplinary pediatric clinic. Many organs are affected; a multidisciplinary team of specialists is needed here - a cardiologist, rheumatologist and others. We already have a small number of children with suspicion who are under observation in clinics,” said Riza Boranbaeva.

The main difference between “Kawasaki-like syndrome” and real Kawasaki disease is that it occurs only after children have had COVID-19 in an asymptomatic or mild form. And if real Kawasaki disease occurs in children under 5 years of age, then MWS can appear in adolescents and older children. There are differences between the two diseases, but in general the course of MWS, temporarily associated with COVID-19, is similar to the development of Kawasaki disease. It is interesting that after influenza and acute respiratory viral infections, such AVS did not occur in either children or adolescents.

Treatment for MHS is carried out on an inpatient basis, the child is hospitalized, tests are taken, where doctors will see changes in the general blood test, coagulogram, specialists conduct ultrasound, CT, X-ray, the approach to diagnosis is multifaceted. The disease can be moderate or severe.

Medium-sized vessels are damaged, as in Kawasaki disease. A complication of MVC is damage to the heart vessels, the development of heart failure, and aneurysms of the heart vessels. If you diagnose it in a timely manner and provide assistance, you can avoid severe forms of the disease and quickly cure the child,” explained Riza Boranbaeva.

The first descriptions of MBC appeared in the UK and the USA; it is believed that cases of MBC are typical for countries where there was a high incidence of coronavirus. It is therefore recommended that such countries prepare their health care systems to manage the influx of pediatric patients who may develop this MBC.

Kazakhstan had a high peak in coronavirus cases in July, with many deaths, and it is expected that MHS can develop in children. This does not mean that now all children who have had COVID-19 will begin to suffer from MHS; it is still a rare pathology, but we must be prepared to provide assistance to children. Because MFS in children and adolescents is much more severe than COVID-19 itself, in some countries MFS has led to deaths among children, warned Riza Boranbaeva.

Treatment of MWS temporarily associated with COVID-19 in children and adolescents is quite serious.

In hospitals, high doses of intravenous immunoglobulins, aspirin, and hormones are used. That is, the approach is individual; the analyzes show at what stage the disease was detected, whether changes have begun in the body that require immediate treatment,” concluded Boranbaeva.

Currently, there are 3 cases of “Kawasaki-like syndrome” known in Kazakhstan.

Albert Akhmetov

Possible complications

The disease usually has a very good prognosis. Most sick children recover completely.

Adverse consequences of the disease occur only in children who are too weak or with severe immunodeficiencies.

The most unfavorable consequences of the disease include: the development of aneurysms of large blood vessels, myocardial infarction, and the appearance of various heart rhythm disturbances.

Typically, these adverse effects are recorded several years after the illness. To eliminate them, you need to consult a cardiologist and prescribe complex treatment, which in some cases is lifelong.

Complications do not occur often; only 20% of sick children are susceptible to them. They are mainly associated with cardiac activity, although it is extremely rare for the patient to have a heart attack or sudden death.

Some of the most common complications include:

1. Pathological dilatation of the coronary arteries.
2. Aneurysm.
3. Pericarditis.
4. Myocarditis.

Less common non-heart effects:

1. Inflammation of the tissues around the brain.
2. Inflammation of joint tissues.
3. Inflammation of the gallbladder tissue.
4. Inflammatory process in the inner part of the eyes.

Etiopathogenesis

The etiology and pathogenesis of the syndrome are currently not fully understood. There are several theories about the origin of the pathology - hereditary, autoimmune, infectious.

• The hereditary nature of the disease is supported by the fact that people who have ancestors with Kawasaki syndrome also develop it in 10% of cases. It has been proven that the pathology affects close relatives.
• Cyclicity and seasonality are signs confirming the infectious etiology of the disease. The number of cases reaches its maximum in spring and winter.
• Autoimmune theory: the body responds to exposure to toxins by producing antibodies to its own vascular endothelial cells.

Pathogenetic links of the syndrome:

1. Impact on the body of infectious biological agents,
2. Activation of T lymphocytes,
3. Production of antibodies to microbes and endothelial cells,
4. Development of immunological reactions against endothelial cells,
5. Inflammation of the muscular layer of the arteries,
6. Necrosis of smooth muscle cells,
7. Endothelial destruction
8. Splitting and stratification of elastic membranes of blood vessels,
9. Pathological dilatation of arteries and formation of aneurysm,
10. Fibrosis of the walls of blood vessels,
11. Proliferation and thickening of the intima,
12. The appearance of general symptoms of the disease,
13. Narrowing of the lumen of the vessel,
14. Thrombosis and occlusion of the affected artery.

Diagnostics: analysis for Coxsackie virus

Typically, infection is diagnosed by clinical symptoms. In rare cases, in severe cases, a test for the Coxsackie virus is prescribed. On average, it takes 2 weeks to get results, which is why such tests are not prescribed en masse. As a rule, the patient will recover faster than the test result. There are rapid tests with a 66–90% confidence level, but they are very difficult to obtain and do not work for all subtypes of the virus.

The doctor may prescribe a general blood test to assess the state of immunity and the severity of the inflammatory process. In some cases, biochemical blood tests may be additionally prescribed to check the functioning of internal organs. In children with symptoms of acute intestinal infection, rapid tests are sometimes performed to rule out other diseases. If a sore throat manifests itself, it is advisable to exclude a streptococcal infection, which will require the use of antibiotics.

If meningitis is suspected, a spinal tap is performed to rule out the bacterial nature of the disease. Ultrasound and ECG are used in the diagnosis of heart pathologies.

Causes

This syndrome was first established in Japan in the 60s of the 20th century. The name of the disease was given by the Japanese doctor Kawasaki. He observed and treated children for a long time who had the same symptoms. At this time, the name of this disease had not yet been established.

After presenting the results of his research at a scientific medical conference, this disease acquired its own name - Kawasaki disease.

According to statistics, boys suffer more from this disease. They get sick mainly 1.5-2 times more often than girls.

Population differences are also noted. In residents of Asian countries, the incidence rate is many times higher than in Europe. Scientists have not yet found a scientific basis for this fact.

There are also isolated cases of the disease at older ages. In Japan and America there is data on the detection of this disease at 25-30 years of age. In some cases, the disease is also found in infants and newborns.

It was not possible to establish a single cause of the disease. Currently, numerous worldwide studies are ongoing aimed at establishing the source of the disease in children.

Most scientists agree that the disease is caused by various viruses. The most probable include: herpes viruses, retro- and parvoviruses, adeno- and cytomegaloviruses and other causes.

A number of studies highlight that various bacterial infections can also lead to the development of Kawasaki disease. Scientists have found that after streptococcal, staphylococcal and meningococcal infections, children develop this disease several times more often.

In a number of countries, cases of Kawasaki disease appearing after the bite of various ticks have been reported.

Borrelia or rickettsia, which enter the blood during tick-borne infections, contribute to the development of inflammatory processes in the blood vessels. These parasites can cause the development of autoimmune reactions in babies, which lead to the development of hemorrhagic vasculitis.

Infection and genes

Photo by ITAR-TASS/ Artem Korotaev
Cases of PMVS are quite rare and most often occur in those geographical locations where the prevalence of COVID-19 is very high: in Bergamo, London, New York, Toronto, and now in Moscow.

It is interesting to note that in 2005, The Journal of Infectious Diseases published a study by a group of scientists at Yale University School of Medicine who found that the trigger for Kawasaki disease in 8 (72%) of 11 children they examined was the then new coronavirus HCoV- N.H.

Apparently, coronaviruses, even relatively safe ones (like HCoV-NH), can cause systemic vasculitis in a child, that is, immunopathological inflammation of blood vessels. Because this occurs in very rare cases, scientists suspect that such children have a genetic predisposition to the new disease.

Interestingly, there have been no reports of PMVS from China or other Asian countries during the pandemic, although Kawasaki syndrome often affects children of Asian descent. It is no coincidence that it was first described by Japanese pediatrician Tomisaku Kawasaki in 1967.

Most of the children affected in London are of African or Afro-Caribbean origin, although there are children of Asian and European descent.

“It is clear that there is a predisposing genetic component that makes a small number of children vulnerable to this disease,” said Dr. Tom Manitas, executive director of the Center for Genome Research in New York. He and his colleagues plan to sequence the genomes of 157 children affected by PMV and their parents, looking for common genetic features.

And while we don’t know them, information about how the disease manifests itself and in what cases the child needs urgent medical attention is relevant for absolutely all parents.

What is a hemorrhagic rash?

When the capillaries are damaged, some of the red blood cells come out of the vessel, which leads to the appearance of a rash with red spots - this is a hemorrhagic rash.

Initially, the rash is painless, but if you ignore the rash, it may become painful. When you press on the rash, its color does not change in any way, and the rash does not become smaller. This is the hallmark of hemorrhagic rashes.

Depending on the lesion that caused the rash, the types of rashes can be different - small dots, stripes or spots, the color of which can also be varied - blue, red, purple. The size of the spots may vary. In medical practice, medium-sized spots are usually called liver purpura, and small spots are called petechiae.

The most common area of ​​localization of the rash is the lower extremities. If a hemorrhagic type rash develops, you should immediately consult a doctor, as this is a direct indication for hospitalization, even in the absence of other symptoms.

Features of hemorrhagic rash in children

A hemorrhagic rash in children usually accompanies hemorrhagic vasculitis, hemophilia, meningococcemia, and thrombocytopenic purpura. But hemorrhagic vasculitis is the most common disease, the manifestation of which is a rash.

As already mentioned, the most common manifestation of the rash occurs on the front surfaces of the legs. However, it can also be localized on the torso, buttocks, and thighs. The rash is located symmetrically, does not disappear when pressed and does not change its color. The disease usually affects children under six years of age.

In addition to hemorrhagic rashes, other types of rashes also occur in children. In the article on the website you can see a photo of a rash on a child’s body with explanations.

Symptoms of the disease

There are 3 periods of generalized vasculitis:

1. Spicy. Lasts the first 7–10 days.
2. Subacute with a duration of 2–3 weeks.
3. The period of convalescence (recovery). Takes from several months to 2 years.

Kawasaki syndrome in children begins abruptly and violently. The temperature rises to 39.0–40.0 degrees, is intermittent, persists for the first 5–7 days, and without treatment – ​​up to 2 weeks. An increase in the duration of the febrile period is a bad sign for further prognosis. Against the background of febrile temperature, regional (usually cervical) lymph nodes enlarge, and symptoms of severe intoxication appear - severe weakness, increased heart rate, abdominal pain, nausea, diarrhea. The child suffers a hard illness, becoming irritable and restless. He often cries, refuses food, and sleeps poorly.

Skin manifestations develop during the first 5 weeks of the disease. Small blisters and a scarlet fever or measles-like rash appear on the body. Its elements are located on the limbs, body, and groin. Gradually, reddened areas appear on the feet and palms, the skin thickens and begins to hurt, which limits finger movements. Feet swell. The rash goes away within 7 days after appearance, and the erythema persists for up to 3 weeks, after which the skin begins to peel off.

Damage to the mucous membranes in the acute period manifests itself in the form of conjunctivitis of both eyes. In some, it is accompanied by anterior uveitis - inflammation of several elements of the choroid at the same time. The mucous membrane of the mouth turns red, becomes dry, the lips become covered with painful bleeding cracks, the tonsils enlarge, the color of the tongue changes to crimson.

Symptoms of Kawasaki syndrome: redness of the eyes, redness and thickening of the skin of the feet, crimson tongue, enlarged cervical lymph nodes

Changes in the blood vessels and heart in Kawasaki disease in children lead to the development of myocarditis with pain, tachycardia or arrhythmia, shortness of breath, often complicated by acute heart failure. Less commonly, the pericardial sac, the pericardium, becomes inflamed and mitral or aortic insufficiency forms. In 25% of patients, dilation of individual sections of the walls of the coronary vessels occurs at 5–7 weeks. The appearance of aneurysms of the subclavian, ulnar or femoral arteries is possible.

Joint damage is detected in approximately 35% of patients in whom both small and large joints become inflamed with corresponding symptoms.

Inflammation of the coronary arteries in Kawasaki syndrome in adults leads to loss of their elasticity and multiple aneurysms (dilations), as a result of which the risk of developing dystrophy or myocardial infarction, thrombosis, calcification and early atherosclerosis sharply increases. Patients are concerned about pain in the heart and joints, disturbances in heart rhythm and digestive function. In some cases, the development of meningitis, urethritis, and damage to the digestive tract is possible.

The patient has problems performing usual physical activity. Some daily activities, such as walking uphill, brisk walking or running, or lifting heavy objects, become difficult.

After treatment, pathological dilations of the walls of blood vessels in adults remain, but decrease over time and may disappear completely.

What are the possible complications of Kawasaki disease?

Kawasaki causes serious heart problems in about 25 percent of children with the condition.

Left untreated, the disease can lead to an increased risk of heart attack, which can be caused by:

1. myocarditis, or inflammation of the heart muscle.
2. dysrhythmia, or abnormal heart rhythm,
3. aneurysm, or weakening and bulging of the artery wall.

Treatment for this stage of the disease requires long-term use of aspirin. Patients may also need to take blood thinners, or undergo procedures such as coronary angioplasty, coronary artery stenting, or coronary artery bypass grafting.

Children who develop coronary artery disease due to Kawasaki disease should take care to avoid lifestyle factors that may increase the risk of a heart attack. These factors include obesity - being overweight, high cholesterol and smoking.

Kawasaki disease in children photo

Diagnostics

Since life-threatening complications can occur with Kawasaki disease, doctors came to the conclusion that diagnosis should be as simple and quick as possible.

The way out of the situation was the American classification, which allows one to suspect the disease at an early stage. This does not require any special tests or analyses.

Laboratory and instrumental diagnostic methods are of an auxiliary nature in this case. Basically, they are needed to detect dangerous complications in time.

In order to confirm the diagnosis of Kawasaki disease and identify complications, doctors prescribe:

General blood analysis. A multiple increase in ESR indicates the presence of autoimmune inflammation. Changes in the white blood cell count may indicate the presence of a viral or bacterial infection in the body.

• Biochemical research.
  An excess of C-reactive protein indicates the development of systemic immune inflammation. Throughout the acute period of the disease, this indicator significantly exceeds the norm. The level of alpha1 antitrypsin also increases.
• Echocardiography of the heart.
  It is carried out several times: at the time of the first diagnosis, 14 days after the onset of the disease and after two months. This sequence allows doctors not to miss the onset of complications of the disease.

• ECG.
  Detects hidden heart rhythm disturbances. With Kawasaki disease, various arrhythmias or tachycardia may develop. It is very easy to detect these changes using an ECG. Children who have had this disease undergo heart examinations regularly.
• X-ray of the chest organs.
  Gives a description of the anatomical structure of the heart and large vessels. Allows you to identify aneurysms that have arisen in large blood vessels. This method is also used to identify hidden and long-term consequences of the disease.

Treatment of Kawasaki disease

The main goal of drug therapy is to protect the cardiovascular system from damage.

The following drugs have a good effect on Kawasaki disease:

With the development of complications from the cardiovascular system (stenosis, aneurysms, myocardial infarction), children who have suffered the acute and subacute stages of Kawasaki disease may be prescribed angioplasty, coronary artery bypass grafting or stenting.