Kawasaki disease

Kawasaki disease is a mucocutaneous lymphatic syndrome that develops primarily in children, affects large, medium and small (mainly coronary) arteries and is characterized by high fever, conjunctivitis, enlarged cervical lymph nodes, polymorphic rash on the body and limbs, cracked lips, inflammation of the mucous membranes ("raspberry tongue"), erythema of the palms and feet, and a steady increase in the number of platelets.

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Epidemiology of Kawasaki disease

Kawasaki disease occurs mainly in children, approximately equally in boys and girls. Children most often get sick in the first 2 years of life. In the world, the incidence varies from 3 to 10 per 100,000 children, but in Japan, the incidence of Kawasaki disease is higher (up to 150 new cases per year per 100,000 children). According to K. Taubert, in the USA, Kawasaki disease has taken first place among acquired heart pathologies in children, displacing rheumatic fever.

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Causes of Kawasaki Disease

The cause of Kawasaki disease is unknown. Hereditary predisposition is possible. Bacteria (propionobacteria, anaerobic streptococci), rickettsia, viruses (mainly herpes viruses) are considered as probable etiologic agents. The disease often develops against the background of previous inflammation of the respiratory tract.

In the acute phase of Kawasaki disease, a decrease in CD8+ T-lymphocytes and an increase in CD4+ T-lymphocytes and B-lymphocytes are noted. The concentration of proinflammatory cytokines (IL-1, TNF-a, IL-10) increases in the blood. Antineutrophil cytoplasmic antibodies reacting with cathepsin G, myeloperoxidase, and neutrophil a-granules are detected with varying frequency. IgM, which has complement-activating ability, is also often detected in the serum.

Pathological characteristics: Kawasaki disease is a systemic vasculitis that affects arteries of various sizes, most often medium-sized ones (vessels of the kidneys and heart) with the development of aneurysms and inflammation of internal organs.

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Symptoms of Kawasaki disease

Kawasaki disease usually begins acutely, with high fever, which is intermittent in nature, but, as a rule, reaches febrile (38-40°C) values. Its duration, regardless of the intake of acetylsalicylic acid and antibiotics, is at least 1-2 weeks, but rarely exceeds 1 month. Against the background of taking acetylsalicylic acid, glucocorticosteroids and especially with the introduction of intravenous immunoglobulin, a decrease in the severity of the temperature reaction and a shortening of its duration are observed. It is believed that prolonged fever contributes to the development of coronary artery aneurysms and increases the risk of fatal outcomes of the disease.

Skin and mucous membrane lesions develop within the first 2-4 days from the onset of the disease. Almost 90% of patients develop bilateral conjunctivitis without pronounced exudative phenomena with predominant lesions of the bulbar conjunctiva. It is often combined with anterior uveitis. Inflammatory changes in the eyes persist for 1-2 weeks.

Almost all patients are diagnosed with damage to the lips and oral cavity. It causes redness, swelling, dryness, cracking and bleeding of the lips, enlargement and redness of the tonsils. In half of the cases, the presence of a raspberry tongue with diffuse erythema and hypertrophy of the papillae is noted.

One of the earliest signs of Kawasaki disease is a polymorphic skin rash. It will appear in the first 5 days from the onset of fever in almost 70% of patients. Urticarial exanthema with large erythematous plaques and maculopapular elements, resembling erythema multiforme, is noted. The rash is localized on the trunk, in the proximal parts of the upper and lower extremities, in the perineum.

A few days after the onset of the disease, redness and/or swelling of the skin of the palms and soles occurs. They are accompanied by severe pain and limited mobility of the fingers and toes. After 2-3 weeks, peeling of the epidermis is noted in the affected areas.

In 50-70% of patients, unilateral or bilateral painful enlargement (more than 1.5 cm) of the cervical lymph nodes is observed. Lymphadenopathy usually develops simultaneously with fever. In some cases, it precedes an increase in body temperature.

Pathological changes in the cardiovascular system are found in almost half of the patients. In the acute phase of the disease, the most frequent (80%) signs of myocarditis are tachycardia, gallop rhythm, damage to the cardiac conduction system and cardiomegaly. Acute cardiovascular failure often develops, less often pericarditis.

Damage to the heart valves (valvulitis, papillary muscle dysfunction) is not often observed. Some publications describe the development of aortic and/or mitral valve insufficiency. By the end of the first week of the disease, 15-25% of patients develop signs of angina or MI. Angiography records dilations and/or aneurysms of the coronary arteries, usually localized in the proximal sections of the vessels.

Systemic manifestations of Kawasaki disease include joint damage, which occurs in 20-30% of patients. This damage leads to polyarthralgia or polyarthritis of the small joints of the hands and feet, as well as the knee and ankle joints. The duration of the joint syndrome does not exceed 3 weeks.

Some patients experience changes in the digestive system (vomiting, diarrhea, abdominal pain). In some cases, urethritis and aseptic meningitis are diagnosed.

Characteristic is unusually high excitability of children, expressed to a greater extent than in other febrile conditions. Some patients develop small aseptic pustules on the elbows, above the knee joints and in the buttocks. Transverse striations of the nails are noted.

Often, other arteries are affected: subclavian, ulnar, femoral, iliac - with the formation of vascular aneurysms at the sites of inflammation.

The main clinical manifestations of Kawasaki disease are fever, skin and mucous membrane lesions (polymorphic skin rash, cracked lips, plantar and palmar erythema, "raspberry tongue"), coronary syndrome, lymphadenopathy.

Involvement of the heart and blood vessels is noted in 50% of patients and can manifest as myocarditis, pericarditis, arteritis with the formation of aneurysms, arrhythmias. Kawasaki disease is the most common cause of sudden coronary death and myocardial infarction in childhood. A late cardiac complication is chronic aneurysms of the coronary arteries, the size of which determines the risk of developing MI in adult patients who have had Kawasaki disease. According to the recommendations of the American Heart Association, all patients who have had Kawasaki disease are monitored for the diagnosis of myocardial ischemia, valvular insufficiency, dyslipidemia and hypertension.

Diagnosis of Kawasaki disease

In the general blood test: increased ESR and CRP concentration, normochromic normocytic anemia, thrombocytosis, leukocytosis with a shift to the left. Increased aminotransferase levels, especially ALT, are noted. An increase in aPL (mainly IgG) in the blood often serves as a marker of coronary artery thrombosis.

ECG can reveal prolongation of the PQ and QT intervals, reduction of the ST wave, depression of the ST segment, and inversion of the T wave. Arrhythmia is possible. EchoCG is a highly informative method for diagnosing Kawasaki disease and heart disease.

An additional diagnostic method is coronary angiography.

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Diagnostic signs

• Fever lasting at least 5 days, persisting despite the use of antibiotics.
• Bilateral conjunctivitis.
• Characteristic lesions of the lips and oral cavity: cracked lips, dryness, redness of the oral mucosa, “raspberry tongue”.
• Enlarged cervical lymph nodes of a non-purulent nature.
• Polymorphic skin rashes, mainly on the trunk.
• Damage to the hands and feet: erythema of the soles and palms, their swelling, peeling during the recovery period.

Identification of 5 out of 6 signs allows to make a reliable diagnosis of "Kawasaki disease". In the presence of 4 signs, echocardiography and coronary angiography data are required to confirm the diagnosis, with the help of which it is possible to detect aneurysms of the coronary arteries.

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Treatment of Kawasaki disease

Treatment of Kawasaki disease includes the use of NSAIDs (acetylsalicylic acid at a daily dose of 80-100 mg/kg in 3 doses, then a single dose of 10 mg/kg until the aneurysms have completely reversed). Human normal immunoglobulin is also used at a dose of 2 g/kg once or 0.4 g/kg for 3-5 days, preferably in the first 10 days of the disease. A possible regimen for administering the drug is once a month.

In case of coronary artery stenosis, long-term administration of ticlopidine (5 mg/kg per day) or dipyridamole (at the same dose) is indicated. In case of severe coronary vessel lesions, aortocoronary artery replacement is indicated.

It is important that those who have recovered have a high risk of early development of atherosclerosis, so they should be under medical supervision.

Prognosis of Kawasaki disease

In most cases, the prognosis for Kawasaki disease is favorable. However, it largely depends on the severity of the lesion, primarily the coronary arteries.

Unfavorable prognostic factors include the onset of the disease in children over 5 years of age, a decrease in hemoglobin, a sharp increase in ESR, and prolonged maintenance of elevated CRP levels.

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History of the issue

This vasculitis was first discovered in Japan in 1961. A detailed description of the disease was given by T. Kawasaki in 1967.

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