Kawasaki disease

Kawasaki disease - mucocutaneous-lymphatic syndrome, which develops mainly in children, affects large, medium and small (mainly coronary) arteries and is manifested by high fever, conjunctivitis, enlargement of the cervical lymph nodes, polymorphic rash on the body and extremities, cracks on the lips , inflammation of the mucous membranes ("crimson tongue"), erythema of the palms and feet, a steady increase in the number of platelets.

[1], [2], [3], [4], [5], [6], [7]

Epidemiology of Kawasaki disease

Kawasaki's disease occurs mainly in children, about equally in boys and girls. Most often children get sick in the first 2 years of life. In the world, the incidence ranges 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 US, Kawasaki's disease came in 1st place among the acquired heart pathology in children, displacing rheumatic fever.

[8], [9], [10], [11], [12], [13], [14]

Causes of Kawasaki's Disease

The cause of Kawasaki's disease is unknown. A hereditary predisposition is possible. As probable etiological agents, bacteria (propionobacteria, anaerobic streptococci), rickettsia, viruses (mainly herpes viruses) are considered. The disease often develops against the background of a previous inflammation of the respiratory tract.

In the phase of worsening of Kawasaki's disease there was a decrease in CD8 + T-lymphocytes and an increase in CD4 + T-lymphocytes, B-lymphocytes. In the blood, the concentration of inflammatory cytokines (IL-1, TNF-a, IL-10) increases. Antineutrophil cytoplasmic antibodies reacting with catechin G, myeloperoxidase, and a-granules of neutrophils are detected at different frequencies. Serum is also often found to have IgM, which has complement-activating ability.

Pathomorphological characteristic: Kawasaki disease - systemic vasculitis, affecting arteries of different caliber, more often middle (vessels of the kidneys and heart) with the development of aneurysms, inflammation of the internal organs.

[15], [16], [17], [18], [19], [20]

Symptoms of Kawasaki disease

Kawasaki disease usually begins with acute fever, which is intermittent in nature, but usually 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 is observed. It is believed that prolonged fever contributes to the development of coronary artery aneurysms and increases the risk of deaths of the disease.

The defeat of the skin and mucous membranes develops already in the first 2-4 days from the onset of the disease. Almost 90% of patients develop bilateral conjunctivitis without pronounced exsessive phenomena with a predominant lesion of the bulbar conjunctiva. Often it is combined with anterior uveitis. Inflammatory changes on the part of the eyes persist for 1-2 weeks.

Virtually all patients are diagnosed with damage to the lips and mouth. It causes redness, swelling, dryness, crackling and bleeding of the lips, an increase and reddening of the tonsils. In half of the cases, there is a presence of a crimson tongue with diffuse erythema and hypertrophy of the papillae.

One of the earliest signs of Kawasaki's disease is a polymorphous skin rash. It will occur in the first 5 days from the onset of fever in almost 70% of patients. They note the urticaria exanthema with large erythematous plaques and maculopapular elements, reminiscent of erythema multiforme. Eruptions are localized on the trunk, in the proximal parts of the upper and lower extremities, in the perineal region.

A few days after the onset of the disease, reddening and / or swelling of the skin of the palms and soles occur. They are accompanied by a sharp pain and limited mobility of the fingers and toes. After 2-3 weeks in the lesions, sloughing of the epidermis is noted.

In 50-70% of patients, one- or two-sided painful increase (more than 1.5 cm) of cervical lymph nodes is observed. Lymphadenopathy, as a rule, develops simultaneously with a fever. In some cases, it precedes an increase in body temperature.

Pathological changes in the cardiovascular system are met in almost half of the patients. In the acute phase of the disease, most often (80%) there are signs of myocarditis, such as tachycardia, rhythm of "gallop", damage to the conduction system of the heart and cardiomegaly. Often develops acute cardiovascular insufficiency, less often pericarditis.

The defeat of the heart valves (valvulitis, dysfunction of the papillary muscles) is not observed often. Some publications describe the development of aortic and / or mitral valve insufficiency. By the end of the week of the disease, 15-25% of patients develop angina or MI. In angiography, dilation and / or aneurysms of the coronary arteries, usually located in the proximal areas of the vessels, are recorded.

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

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

Characteristically unusually high excitability of children, expressed in a greater degree than with other febrile states. Some patients develop small aseptic pustules on the elbows, above the knee joints and in the buttocks. Note the transverse striation of the nails.

Often there is a lesion of other arteries: subclavian, ulnar, femoral, iliac - with the formation of an aneurysm of blood vessels in places of inflammation.

The main clinical manifestations of Kawasaki disease: fever, skin and mucous membrane damage (polymorphic skin rash, lip cracks, plantar and palmar erythema, "raspberry tongue"), coronary syndrome, lymphadenopathy.

Involvement of the heart and vessels was noted in 50% of patients and may 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 coronary artery aneurysms, the size of which determines the risk of MI in adults who have undergone Kawasaki disease. According to the recommendations of the American Heart Association, all patients who underwent Kawasaki's disease are monitored for the diagnosis of myocardial ischemia, valvular insufficiency, dyslipidemia and AH.

Diagnosis of Kawasaki disease

In the general analysis of blood: an increase in ESR and concentration of CRP, normochromic normocytic anemia, thrombocytosis, leukocytosis with a shift to the left. An increase in the level of aminotransferase, especially ALT, is noted. Accumulation in the blood of APL (mainly IgG) often serves as a marker of coronary thrombosis.

On the ECG, you can determine the extension of the interval PQ, QT, reduction of the ST wave, depression of the ST segment, inversion of the T wave. Arrhythmias are possible. EchoCG serves as a highly informative method for diagnosing Kawasaki disease of heart lesions.

An additional method of diagnosis is coronaroangiography.

[21], [22], [23], [24], [25], [26], [27], [28]

Diagnostic signs

• Fever for at least 5 days, persisting with the use of antibiotics.
• Bilateral conjunctivitis.
• Characteristic lesions of the region of the lips and oral cavity: lip cracks, dryness, reddening of the mucous membrane of the mouth, "crimson tongue".
• An increase in the cervical lymph node nerve character.
• Polymorphic skin rashes, mainly on the trunk.
• The defeat of the hands and feet: erythema of the soles and palms, their puffiness, peeling during convalescence.

Identification of 5 of 6 signs allows you to put a reliable diagnosis of "Kawasaki disease". If there are 4 signs for confirmation of the diagnosis, EchoCG, coronary angiography, with the help of which it is possible to detect aneurysms of the coronary arteries is needed.

[29], [30]

Treatment of Kawasaki disease

Treatment of Kawasaki disease includes the use of NSAIDs (acetylsalicylic acid in a daily dose of 80-100 mg / kg in 3 divided doses, then 10 mg / kg once until the full reverse development of aneurysms). Human 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. Possible mode of drug administration - once a month.

With stenosis of the coronary arteries, long-term use of ticlopidine (5 mg / kg per day) or dipyridamole (at the same dose) is indicated. A severe coronary artery disease shows aortocoronary prosthetics.

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

Forecast of Kawasaki disease

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

Adverse prognostic factors are the debut of the disease in children older than 5 years, a decrease in hemoglobin, a sharp increase in ESR, and a prolonged persistence of elevated CRP indices.

[31], [32], [33], [34]

Background

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

[35], [36], [37], [38], [39], [40], [41]