Kawasaki disease is a rare immunocomplex inflammatory lesion of arteries of various calibers, occurring mainly in children of the first years of life. Kawasaki disease is manifested by fever, polymorphic diffuse rash, conjunctivitis, lesions of the oral mucosa, skin and joints of the distal extremities, cervical adenopathy. The diagnosis is based on clinical criteria, the results of laboratory blood and urine tests, ECG data, ultrasound of the heart and coronary angiography. The basis of treatment is intravenous administration of immunoglobulin and acetylsalicylic acid, anticoagulants are used according to indications.
Meaning
Kawasaki disease got its name thanks to a Japanese pediatrician named Kawasaki who discovered it in 1961. Initially, it was assumed that the disease has a mild course. It was only in 1965 that a case of severe cardiac pathology associated with Kawasaki disease was identified.
Today, Kawasaki disease is one of the most common causes of acquired heart pathology in childhood. The most common disease occurs among representatives of the yellow race, especially the Japanese. In Japan, Kawasaki disease is diagnosed 30 times more often than in Australia or the UK and 10 times more often than in America.
Causes
Currently, rheumatology does not have unambiguous data on the causes of the development of disease. The most recognized theory is the assumption that Kawasaki disease develops against the background of hereditary predisposition under the influence of infectious agents of bacterial (streptococcus, staphylococcus, rickettsia) or viral (Epstein-Barr virus, herpes simplex, parvoviruses, retroviruses) nature. The connection of the disease with race, its spread in other countries mainly among Japanese emigrants, the development of the disease in 8-9% of the descendants of those who have been ill, testifies in favor of a hereditary predisposition to Kawasaki disease.
Kawasaki disease symptoms
As a rule, Kawasaki disease occurs in the first 5 years of life, some cases of the disease have been reported in children up to 8 years old. According to some data, the peak incidence occurs at the age of 9-11 months, according to others — 1.5-2 years. During Kawasaki disease, there are three periods: acute — 7-10 days, subacute — 14-21 days and a recovery period that can take from several months to 1-2 years.
Kawasaki disease begins with a rise in body temperature. Without treatment, the fever persists for 2 weeks. An increase in the febrile period is considered a prognostically unfavorable symptom.
Skin manifestations of disease can occur within 5 weeks from the onset of the disease. They are characterized by polymorphic diffuse elements in the form of small flat spots of red color (macular rash), blisters, scarlet fever-like or measles-like rashes. The elements of the rash are located mainly on the skin of the trunk, inguinal region and proximal extremities. Over time, erythematous areas appear, there is a painful compaction of the skin of the palms and soles, which causes restriction of movements in the fingers. Resolution of rash elements in Kawasaki disease begins about a week after their appearance. Erythematous spots persist for 2-3 weeks, after which their surface begins to peel off.
Lesions of the mucous membranes of the eyes and mouth. Most patients with Kawasaki disease have conjunctivitis of both eyes during the first 7 days, usually not accompanied by discharge. In some cases, it is accompanied by anterior uveitis. There is also dryness and redness of the oral mucosa, bleeding cracks on the lips, crimson color of the tongue and an increase in tonsils. Kawasaki disease in 50% of cases is accompanied by an increase in the cervical groups of lymph nodes, more often unilateral.
Damage to the heart and blood vessels in Kawasaki disease can be characterized by myocarditis, manifested by tachycardia, heart pain, arrhythmia and often leading to acute heart failure. In 25% of patients with Kawasaki disease, 5-7 weeks after the onset of the disease, aneurysmal dilations of the coronary vessels of the heart are detected, which can lead to the development of myocardial infarction. In rare cases, pericarditis, aortic or mitral insufficiency appears. Aneurysms may occur along the course of large arteries: ulnar, subclavian, femoral.
Articular syndrome is observed in 35% of cases of disease and usually lasts up to 1 month. Arthralgia and arthritis of the ankle and knee joints, lesions of small joints of the hands and feet are typical.
Possible defeat of the gastrointestinal tract with the occurrence of abdominal pain, vomiting, diarrhea. In some cases, meningitis and urethritis are observed.
Diagnostics
The generally accepted clinical diagnostic criteria for Kawasaki disease is the presence of at least 4 of the following signs against a background of fever lasting more than 5 days.
- Bilateral conjunctivitis.
- Polymorphic rash with diffuse spread over the skin.
- Defeat of the oral mucosa.
- Changes in the hands and feet with their redness and swelling.
- Enlargement of cervical lymph nodes.
If a coronary artery aneurysm is detected, the presence of 3 of these diagnostic signs is considered sufficient.
Laboratory diagnostics does not give specific signs of disease, however, the totality of the detected changes can be an additional confirmation of the correctness of the diagnosis. The clinical blood test determines anemia, leukocytosis with a shift of the leukocyte formula to the left, thrombocytosis, a significant acceleration of ESR. Biochemical blood analysis reveals an increase in immunoglobulins, seromucoid and transaminases, the appearance of CEC. In the analysis of urine, proteinuria and leukocyturia may be observed.
In order to diagnose cardiac pathology, an ECG, chest X-ray, ultrasound of the heart, angiography of the coronary arteries are performed. According to the indications, a lumbar puncture and a study of the cerebrospinal fluid are performed.
Differential diagnosis of Kawasaki disease is necessary with measles, scarlet fever, infectious mononucleosis, rubella, influenza, adenovirus infection, juvenile rheumatoid arthritis, mycoplasma pneumonia, Stevens-Johnson syndrome.
Kawasaki disease treatment
Immunoglobulin. To stop inflammatory changes occurring in the vessels, intravenous administration of immunoglobulin is performed. It is carried out in stationary conditions for 8-12 hours. If, after the administration of immunoglobulin, there is no decrease in temperature and a decrease in inflammatory phenomena, its repeated administration is indicated. The best results of immunoglobulin treatment were obtained during its implementation in the first 10 days of the development of Kawasaki disease.
Acetylsalicylic acid. In modern medicine, this drug is prescribed only if there are strict indications. However, in the treatment of Kawasaki disease, it is included in the list of necessary medications. The purpose of its use is to reduce the risk of blood clots and anti—inflammatory therapy. After a decrease in body temperature, the dose of acetylsalicylic acid is lowered to preventive.
Anticoagulants (warfarin, clopidogrel) are prescribed to prevent thrombosis in children with diagnosed vascular aneurysms. Corticosteroid therapy for Kawasaki disease is not carried out, as studies have shown that it increases the risk of coronary thrombosis.
Forecast
Kawasaki disease in most cases has a favorable prognosis, especially when therapy is started in a timely manner. However, there is a risk of fatal outcome of the disease (0.8-3%) due to coronary artery thrombosis and the development of myocardial infarction. A rarer cause of death is severe myocarditis with severe heart failure.
In about 20% of cases, children who have had Kawasaki disease retain changes in the walls of the coronary arteries, which in the distant future may lead to the early appearance of atherosclerosis or calcification, followed by cardiac ischemia, threatening the development of acute myocardial infarction. Risk factors that accelerate the development of changes in the coronary arteries are hypertension, hyperlipidemia, smoking. In this regard, patients with Kawasaki disease after recovery should be under the constant supervision of a cardiologist or rheumatologist, once every 3-5 years undergo a complete examination of the heart, including an ECHO-EG.