Kawasaki syndrome – symptoms, diagnosis, treatment, complications

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Kawasaki syndrome is also known as Kawasaki disease. It is most common in children, but can also affect adults. It is characterized by the formation of inflammation of the walls of blood vessels, most often accompanied by a very high fever. Is Kawasaki disease dangerous? How can you tell the difference from measles or COVID-19? How is Kawasaki syndrome treated?

Kawasaki syndrome – what is it characterized by?

Kawasaki syndrome is called variously – Kawasaki disease, cutaneous and mucosal lymph node syndrome, disease Kawasaki (from Latin) or Kawasaki disease (from English). It is an acute disease of small and medium-sized vessels. It was first described by Tomisaku Kawasaki. As early as 1967, a Japanese pediatrician noticed convergent symptoms of an acute disease that mainly affected children.

Kawasaki syndrome belongs to the group of systemic vasculitides, the common element of which is necrotizing peripheral vasculitis – mainly arterioles and veins, especially coronary arteries. Acute inflammation can also affect the pericardial sac, endocardium, and also the heart.

Kawasaki syndrome – the etiology of the disease

Although the etiology of Kawasaki disease is not fully understood, it is believed that an autoimmune process is taking place in the endothelium, the main symptoms of which are:

1. Increase of T-helper lymphocytes and monocytes,
2. Increasing levels of interleukin 1,
3. Increasing number of receptors for interleukin 2,
4. The appearance of antibodies to the vascular endothelium.

Kawasaki syndrome – who does it most often?

Kawasaki syndrome is most often diagnosed in children under 5 years of age. The peak incidence occurs in the eighteenth month of life. The disease is diagnosed more often among boys than among girls. Kawasaki syndrome is primarily diagnosed in Asian countries. In Europe, the incidence of children is lower.

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Kawasaki syndrome – causes of occurrence

The development of Kawasaki disease is believed to be caused by the multiplication of viruses and bacteria, as the syndrome is often diagnosed with viral and bacterial infections. Genetic predisposition also plays a role in the diagnosis of Kawasaki syndrome. In people who have them, the immune system sends an incorrect response to fight the antigens of infectious agents, which in turn leads to the formation of infiltration of the blood vessel walls.

A direct consequence of the malfunctioning of inflammatory cells in the immune system is necrosis of the tissue walls, which may involve an entire organ or system. Although Kawasaki disease is a childhood disease, a small number of adult cases are known.

Kawasaki syndrome – the most common symptoms

The most common symptom of Kawasaki Syndrome that can be confusing is a high fever (above 39 degrees Celsius) for an extended period (usually five days). Strawberry tongue is a characteristic symptom of inflammation of the oral mucosa – the organ is red and the nipples are overgrown. Other organs may also be inflamed, including ulcers that come up.

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Kawasaki syndrome – acute, subacute and recovery phase

The symptoms of Kawasaki disease are identified in three phases: acute, subacute, and healing. The first is the acute phase lasting about 10 days, with symptoms such as:

1. High fever (over 39 degrees Celsius) lasting up to five days, however, there have been cases in which it persisted for 3-4 weeks,
2. Conjunctivitis without purulent exudate,
3. Rash most often located on the trunk, limbs and genital area,
4. The mucosa of the mouth and throat are red in color,
5. The lips are dry and the tongue is swollen
6. Photophobia,
7. Enlarged lymph nodes on one side
8. Irritability,
9. Glistening skin on palms and soles of feet observed.

In the acute phase of Kawasaki syndrome, myocarditis may also develop. The next is the subacute phase, distinguished by:

1. Noticed psoriasis involving the skin on the hands and feet,
2. Diarrhea,
3. Abdominal pain,
4. Arthralgia,
5. Vomiting.

The subacute phase lasts from day 10 to 6,5 weeks. Around the third week of Kawasaki disease, aneurysms can develop that are immediately life-threatening.

The recovery phase lasts from 6,5 to 9 weeks. Symptoms begin to slowly subside, however, as complications may occur, the child should still be under the constant care of a doctor.

Kawasaki syndrome – differentiation with other diseases

At the diagnostic level, Kawasaki syndrome should first of all be distinguished from other childhood diseases. It is most often confused with measles and scarlet fever, as a rash appears on the child’s body. In addition, other diseases should also be considered when differentiating:

1. Nodular arteritis,
2. Mercury poisoning,
3. Toxic shock syndrome,
4. Juvenile idiopathic arthritis,
5. Rheumatic fever,
6. Stevens-Johnson syndrome,
7. Infectious mononucleosis,
8. Reactive arthritis,
9. Henoch-Schonlein purpura (IgA vasculitis).

Kawasaki syndrome and COVID-19 – basic differences

The links between Kawasaki syndrome and SARS-CoV-2 disease are the subject of current medical research as an association has been noted between Kawasaki disease and a positive COVID-2 test in children. In both cases, there is immune system dysfunction caused by viruses (in Kawasaki disease it can also include bacteria). The common element is persistent high fever.

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Kawasaki syndrome – when to see a doctor?

You should visit a pediatrician immediately if, despite pharmacological treatment, a fever of over 39 degrees C persists in a child for more than five days. Usually there are comorbidities that should worry the parents. They include, among others red eyes, swollen glands, red hands and feet, and noticeable changes in the mouth (e.g. swollen tongue). A polymorphic rash should also be a signal to see your doctor as soon as possible.

Kawasaki syndrome – the correct diagnosis of the disease

Correct diagnosis of Kawasaki disease in children is based primarily on the exclusion of other diseases. All patients (100%) develop fever. The full-blown form of the disease is diagnosed in the presence of high temperature and 4-5 other symptoms – changes in the oral cavity concern 96%. patients, reddened conjunctiva – 86 percent, and polymorphic rash – 82 percent.

Kawasaki syndrome – treatments

Treatment of Kawasaki syndrome in children should begin as soon as possible, up to 10 days after the onset of fever, in order to minimize the risk of complications. The patient is given acetylsalicylic acid and methylprednisolone.

Treatment also includes the administration of intravenous infusions of human immunoglobulins. As a result, acute symptoms decrease within 24-48 hours. Typically, patients will be helped by just one dose of immunoglobulins.

It may be shorter for your child to be hospitalized if they are given corticosteroids. In the event of complications, including aneurysms, specialist treatment is required.

Is Kawasaki disease curable?

Kawasaki disease is fully treatable, but if not treated promptly, complications can lead to permanent changes. Patients who do not develop them may return to their lifestyle. Mortality in patients with Kawasaki syndrome is estimated at 1 percent.

Kawasaki syndrome – complications

The most frequently diagnosed complications after Kawasaki disease include cardiovascular complaints:

1. Coronary artery aneurysms,
2. Heart failure
3. Myocardial infarction,
4. Peripheral arterial obstruction.

For this reason, the patient should not only be under the care of a pediatrician, but also a cardiologist.