What is Kawasaki disease? What are its causes, symptoms and how is it treated?

Kawasaki disease can also be found under the name mucocutaneous nodular syndrome or mucocutaneous lymph node syndrome. It is a systemic vasculitis , which indicates a vascular involvement, most often affecting small and medium vessels .

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The disease was described by Tomisaka Kawasaki as the first Japanese pediatrician in 1967. The disease occurs in children under 5 years of age , and especially between the ages of 2 and 3, affecting most boys . The most common occurrence is in Japanese children.

Kawasaki syndrome is up to 6 times more common in Asian children .

It most often affects the coronary arteries , ie the vessels that supply oxygen and nutrients to the heart. Its serious complication can be an aneurysm, which is a bulge - a dilation of a blood vessel. In addition to vasculitis, there is fever or swelling of the limbs. However, several criteria are needed to determine this diagnosis.

The disease is multisystemic , which means that it affects other parts of the body than just blood vessels. It is manifested by fever, damage to the skin, mucous membranes, conjunctiva, but also abdominal pain or swollen lymph nodes . When diagnosing, it is important to rule out another disease. The origin and origin of the disease is not clear.

The disease usually manifests itself as acute, while early treatment is also important , which reduces the risk of cardiovascular complications. This increases especially if the disease is not treated, up to 20-35%. 

Kawasaki disease is rare, but it is one of the most common vasculitis that affects childhood .

Even today, the exact cause of Kawasaki syndrome is not fully understood . There are more opinions. It is assumed that the main component is an immune disorder , ie an autoimmune error or its hypersensitivity , ie hypersensitivity. Another opinion states that an infectious origin is also involved in the onset of the disease. . And of a bacterial or viral nature. An example would be the harmful effects of a toxin. It is produced by a bacterium from the Golden Staphylococcus strain.

It is suggested that EBV (which is the virus that causes infectious mononucleosis) interacts with or is associated with the herpes virus. And a possible coronavirus agent is also mentioned. The seasonal occurrence was observed in the disease, especially in spring and winter. Which supports the view of infectious origin.

Genetic predisposition is also reported for the disease , which could also indicate an inherited mechanism. Kawasaki syndrome is not infectious , so it is not transmitted from one child to another. The disease can also have a recurrence , ie a recurrence of the disease, in about 2 percent.

Although the disease is relatively rare, it is referred to as the most common vasculitis in childhood. Vasculitis is an inflammatory disease of the blood vessels. In the first 10 days, it damages the vessel wall . The coronary vessels, ie the heart, are most affected. These are the ones that lead oxygenated blood to the heart muscle.

If the disease is not treated, the risk of complications with the cardiovascular system rises to 20 percent or more. It is stated that the disease in this case can cause an aneurysm, which is a dilation of a vessel wall. And this bulging of the vessel wall is consequently a risk for the formation of a thrombus , i.e. a blood clot. There is also a risk of platelet aggregation (thrombus formation) at the site of vessel damage.

A blood clot that is large enough restricts blood flow through the heart vessel . This is the cause of a heart attack , which is actually a condition where the heart muscle is not sufficiently supplied with oxygen and dies. This happens mainly in the period within 8 weeks from the onset of the disease.

Also read : Information on first aid for myocardial infarction in the article on Health .

Another serious risk is an aneurysm rupture . These complications cause sudden death in approximately 2% of those affected. Inflammation of the heart muscle is also serious, as is damage to the heart valves. However, the disease is multisystemic , which means that it also affects other body systems .

Among the symptoms of Kawasaki disease include:

• vascular involvement, and thus mainly cardiovascular problems
  • blood vessel aneurysm until it ruptures
  • ischemia to myocardial infarction
  • myocarditis
  • pericarditis
  • heart rhythm disorders
  • valve defects, inflammation
• fever that lasts for more than 5 days
• conjunctivitis , ie conjunctivitis , but without purulence and discharge
• rashes on the skin , especially on the torso, the area under the diaper and on the limbs
• hives
• reddening of the skin
• skin scales
• swelling of the skin , especially severe swelling of the fingers, hands and feet
• peeling of the skin of the fingers , especially in the nail bed area
• in some cases fingertip gangrene, especially in very young children
• cracked and deep red lips and oral mucosa
• red and raspberry tongue
  

  

• redness of the pharynx
• swelling of the lymph nodes of the neck, enlarging mainly one node to a size of more than 1.5 cm
• headache
• inflammation of the nerves
• pain and swelling of the joints, pain restricts movement, for arthritis
• stomachache
• liver and gallbladder damage
• vomiting
• diarrhea
• urinary tract infection
• in boys, testicular inflammation
• weakness
• restlessness, nervousness

In the early stages of the disease, it is important to rule out another cause of the problem . If the diagnosis is confirmed, early treatment is important . This is to prevent cardiovascular complications.

Laboratory examination of blood , but also urine or cerebrospinal fluid is helpful . Thus, blood culture (blood), urine culture, inflammatory parameters, CRP, FW (blood sedimentation), blood count (KO, evidence of anemia, leukocyte levels, platelets).

Lumbar puncture is performed to examine the cerebrospinal fluid, ie the spinal fluid .

ECHO  will be used as the imaging method . This can prove that the blood vessels are involved, including an aneurysm. Furthermore, ECG , chest X-ray, scintigraphy, coronarography, but also MRI are important.

Of course, the diagnosis is based mainly on the history and clinical picture of the disease . The determination evaluates the so - called diagnostic criteria , which must be established for the detection of Kawasaki syndrome , and thus the presence of 5 out of 6 symptoms in.

The table shows the diagnostic criteria for the diagnosis of Kawasaki syndrome

The disease occurs until the 5th year of life, but mainly in the period of the 2nd - 3rd year. However, if it occurs before the 2nd or 5th year, its manifestations are more serious. Kawasaki syndrome begins with vascular involvement , ie vasculitis, in the first 10 days.

Kawasaki syndrome is divided into three phases :

• acute stage within two weeks
• subacute stage  2 - 4 weeks
• convalescence phase 1 to 3 months

The damage is mainly at the level of the heart vessels and their walls. This results in platelet aggregation . Blood clots, or thrombus , are formed . It then narrows to completely close the lumen of the vessel. Insufficient blood flow to the heart muscle to complete non-blood flow, ie ischemia, is the cause of heart muscle infarction. The time period of this is up to 8 weeks .

In addition to myocardial infarction  , vascular wall damage is involved in vascular dysfunction . And this results in a bulging of the vessel wall, an aneurysm. An aneurysm is a risk of rupture of the vessel wall. A complication is sudden death, which occurs in about 2% of cases.

Fever is present in the acute phase , even above 40 ° C, and persists for more than 5 days. Associated to non-purulent conjunctivitis . Swelling of the fingers and toes are also mentioned as characteristic symptoms . The swelling is solid.

The acute stage is then characterized by skin sowing , in the form of rashes or hives. The main area is the chest, abdomen and part under the diaper and genitals. The lips are dry , cracked , red .

Like tongue, it has the form of raspberry tongue (sometimes referred to as strawberry).

At this stage, the disease is also characterized by enlargement of the cervical node , up to 70% of those affected. The node can be more than 1.5 centimeters in size. Multisystem disorders also include damage to the liver and gallbladder. This results in abdominal pain, vomiting and diarrhea. They are associated with joint pain and swelling, which is a consequence of arthritis.

After two weeks, the disease passes into the subacute part. In the subacute phase, peeling of the skin occurs , especially in the area of ​​the fingers and nail beds . Neglecting treatment increases the risk of an aneurysm. The platelet value is rising. The convalescence phase is characterized by a decrease in inflammatory values .

Fortunately, healing occurs in most cases. Important here is early diagnosis of the disease and its treatment . The risk of complications increases due to non-treatment, but, in a small percentage of cases, also with treatment.