Kawasaki disease


The Kawasaki disease is an inflammation of the vessels, which mostly affects children between two and five years. If left untreated, the coronary arteries are severely damaged in almost a third of cases, which can lead to heart attack, hemorrhage, and ultimately death. However, if the Kawasaki syndrome is detected and treated in good time, the survival rate is 99.5 percent. Read all about the symptoms and treatment of Kawasaki syndrome.

ICD codes for this disease: ICD codes are internationally valid medical diagnosis codes. They are found e.g. in medical reports or on incapacity certificates. M30ArtikelübersichtKawasaki syndrome

  • description
  • symptoms
  • Causes and risk factors
  • Examinations and diagnosis
  • treatment
  • Disease course and prognosis

Kawasaki syndrome: description

Kawasaki syndrome is an acute inflammatory disease of small and medium-sized blood vessels that can affect the entire body and all organs. Therefore, the disease can hide behind a variety of symptoms.

Paediatricians consider Kawasaki syndrome to be a rheumatic disease in a broader sense. More specifically, it belongs to the vasculitis (vascular inflammation). Another name for the Kawasaki syndrome is also the "mucocutaneous lymph node syndrome".

The Kawasaki syndrome takes its name from the Japanese Kawasaki, who first defined the disease in 1967. He described cases of children who suffered from high fever over several days, which was not to be lowered, and made a seriously ill impression.

In most cases, they are small children with Kawasaki syndrome. It is not known exactly why the inflammation occurs. It is believed that it is an overreaction of the immune system, which has been activated by pathogens at an earlier stage. In the inflammation of the Kawasaki syndrome, an inflammatory reaction occurs without pathogens attacking the vessel wall. This inflammation manifests itself in different places on the body, but the heart is especially at risk.

Dangers of Kawasaki syndrome

The inflammation of the coronary arteries can damage the vessel wall. If there is a bleeding or obstruction of the vessels, severe consequences for the patient threaten. Intensive medical monitoring is essential to detect possible complications early. Even years after the illness, a heart attack due to the damage of the coronary arteries by the Kawasaki syndrome is still possible. Adults who have suffered from Kawasaki's disease as a child can still be in mortal danger for years after the disease due to severe heart complications.

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Kawasaki syndrome: symptoms

Kawasaki syndrome can hide behind a variety of symptoms. Because the disease can in principle affect almost every organ. However, there are five main symptoms that are very typical and should always make you think of Kawasaki syndrome in combination.

  • In all cases, there is a fever over 39 ° C for more than five days. Especially in this fever is that no cause can be determined. Often, bacteria or viruses cause fever, but in Kawasaki syndrome no pathogen can be found. Even antibiotic therapy can not lower the fever.
  • The oral mucosa, tongue and lips are crimson in 90 percent of the patients. Physicians speak of these symptoms of lacquer lips and a strawberry or raspberry tongue.
  • In 80 percent of cases, there is a rash on the chest, stomach and back. This can look very different and remind of scarlet fever or measles. It also causes a rash on the palm and the sole of the foot. After two to three weeks, the skin begins to shed on fingers and toes. Skin peeling is a very late sign of Kawasaki syndrome.
  • Very often bilateral conjunctivitis (conjunctivitis) occurs. Both eyes are red and small red vessels in the eye white to recognize. Kawasaki syndrome does not cause pus formation because no bacteria are involved in the inflammation. A purulent conjunctivitis would therefore speak against the Kawasaki syndrome.
  • In about two-thirds of patients, the lymph nodes on the neck are swollen. This is often a sign that an inflammatory reaction is taking place in the body and the immune system is activated.

The affected children can be seen quickly that they are seriously ill. They are tired, not very active and in poor condition. In Kawasaki syndrome, all organs in the body can be affected, so that the disease can hide behind a variety of symptoms. Thus, in addition to the above-mentioned points, it can also cause joint pain, diarrhea, vomiting, headache, painful urination or chest pain.

By far the most dangerous complication is the inflammation of the heart vessels. If there is a deficiency of the myocardium with oxygen or a heart attack, typical symptoms such as chest pain with radiance in the arm, tightness in the chest and shortness of breath may occur. Intensive monitoring of the heart is therefore always necessary to detect serious complications in a timely manner.

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Kawasaki syndrome: causes and risk factors

The causes of Kawasaki syndrome are largely unexplained. Researchers suspect that an overreaction of the body's defense system is behind it. An inflammatory reaction in the blood vessels is triggered and the vessel wall is damaged. A genetic component should also play a role. Some experts, however, rather assume that the cells of the blood vessels themselves overreact and inflammation in this way arises.

In Germany, nine out of every 10,000 children suffer from Kawasaki syndrome annually. In Japan, the morbidity rate is more than 20 times higher. The cause is unknown. Four out of five sufferers are children between the ages of two and five. Boys are more likely to be affected by Kawasaki syndrome than girls.

Apart from a certain genetic predisposition no further risk factors are known. The potential genetic component was discovered because sibling children of Kawasaki syndrome more often develop Kawasaki syndrome themselves.

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Kawasaki syndrome: examinations and diagnosis

The diagnosis of Kawasaki syndrome is primarily made clinically. There are no specific tests for the disease. The doctor should remember Kawasaki syndrome if there are four of the five symptoms described in more detail above.

  • fever
  • skin rash
  • oral mucosa redness
  • Conjunctivitis
  • lymphadenopathy

If Kawasaki syndrome is suspected, the heart should be closely examined. In particular, an electrocardiogram (ECG) and a heart ultrasound are necessary to detect possible damage to the heart muscle early. If a child experiences a fever lasting several days and the doctor can not find any cause for the temperature increase, he should always think of Kawasaki syndrome.

Also in the blood, there are some signs that can help the doctor in the diagnosis. The so-called inflammatory levels (leucocytes, C-reactive protein, and the erythrocyte sedimentation rate) are elevated and indicate an inflammatory process. No bacteria or viruses are detected in the blood, otherwise there would be a suspicion of blood poisoning (sepsis).

In the suspected diagnosis Kawasaki syndrome, the other organs should be examined. Particularly helpful here is the ultrasound technique, because with this all organs of the abdominal cavity can be displayed well.