Kawasaki disease overview
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief:
Overview
Kawasaki disease, also known as lymph node syndrome, mucocutaneous node disease, infantile polyarteritis and Kawasaki syndrome, is a poorly understood self-limited vasculitis that affects many organs, including the skin and mucous membranes, lymph nodes, blood vessel walls, and the heart. It does not seem to be contagious. It was first described in 1967 by Dr. Tomisaku Kawasaki in Japan.. Kawasaki disease is predominantly a disease of young children, with 80% of patients younger than 5 years of age. Additional risk factors in the United States include Asian race and male sex. Kawasaki disease can cause vasculitic changes (inflammation of blood vessels) in the coronary arteries and subsequent coronary artery aneurysms. Common symptoms of kawasaki disease include high grade fever, red eyes, bright red and cracked lips, red mucous membranes in the mouth, strawberry tongue, white coating on the tongue or prominent red bumps (papillae) on the back of the tongue, red palms of the hands and the soles of the feet, swollen hands and feet, and rash. Intravenous Immunoglobulin (IVIG) and aspirin are indicated in kawasaki disease.
Historical perspective
Kawasaki disease was first discovered by Dr. Tomisaku Kawasaki when he saw his first case of Kawasaki disease in Japan, in 1961. Later in 1967, Kawasaki published his first report of Kawasaki disease in Japanese. Dr Kawasaki also developed "Japan Kawasaki Disease Research Center" in 1990.
Classification
Patients whose illness does not meet the diagnostic criteria of Kawasaki disease case definition, but who have fever and coronary artery abnormalities are classified as atypical or incomplete Kawasaki disease. The patients of atypical or incomplete kawasaki disease, an evidence of coronary abnormalities or CAA’s must be shown on the echocardiogram.
Pathophysiology
Causes
The exact cause of kawasaki disease has not been identified. The current etiological theories center primarily on immunological causes for the disease, much research is being performed to discover a definitive toxin or antigenic substance, possibly a superantigen, that is the specific cause of the disease. There are several hypothesis for the causes of Kawasaki disease, the Infectious agents which are thought to induce kawasaki disease are, parvovirus B19, meningococcal septicemia, adenovirus, bacterial toxin–mediated, superantigens, cytomegalovirus, epstein-Barr virus, human lymphotropic virus infection, klebsiella pneumoniae bacteremia, mycoplasma pneumoniae, mite-associated bacteria, measles, propionibacterium acnes, parainfluenza type 3 virus, rotavirus infection, rickettsia species and tick-borne diseases.
Differentiating Kawasaki disease from other diseases
Different rash-like conditions can be confused with Kawasaki disease and are thus included in its differential diagnosis. The various conditions that should be differentiated from Kawasaki disease include Impetigo ,Insect bites, Measles, Monkeypox, Rubella, Atypical measles, Coxsackievirus, Acne, Syphilis, Molluscum contagiosum, Mononucleosis, Toxic erythema, Rat-bite fever, Parvovirus B19, Cytomegalovirus, Scarlet fever, Rocky Mountain spotted fever, Stevens-Johnson syndrome, Varicella-zoster virus, Chickenpox, Meningococcemia, Rickettsial pox, Meningitis, Toxic shock syndrome, Roseola Infantum (exanthem subitum), Erythema Infectiosum (Fifth Disease), Enterovirus, Dengue Fever, Drug induced rash, Infectious Mononucleosis, Pharyngoconjunctival Fever, Coxsackie virus, Herpangina, and Primary herpetic gingivoestomatitis.
Epidemiology and Demographics
Kawasaki disease occurs worldwide, with the highest incidence in Japan, and it most often affects boys and younger children. KD may have a winter-spring seasonality, and community-wide outbreaks have been reported occasionally. In the continental United States, population-based and hospitalization studies have estimated an incidence of KS ranging from 9 to 19 per 100,000 children younger than 5 years of age. Approximately 4248 hospitalizations for Kawasaki disease, of which 3277 (77%) were for children under 5 years of age, were estimated among children younger than 18 years of age in the United States in the year 2000.
Risk factors
Common risk factors in the development of Kawasaki disease are believed to be environmental, genetic, and viral.
Screening
Natural History, Complications, and Prognosis
Diagnostic Criteria
Kawasaki disease is diagnosed clinically (by medical signs and symptoms), and there exists no specific laboratory test that can tell if someone has it. It is normally difficult to establish the diagnosis, especially early in the course of illness, and frequently children are not diagnosed until they have seen their doctor several times, or visited a number of different health care providers. Many other serious illnesses can cause similar symptoms, and must be considered in the differential diagnosis, including scarlet fever, toxic shock syndrome, and juvenile idiopathic arthritis. Classically, five days of fever plus four of five diagnostic criteria must be met in order to establish the diagnosis that are Mucositis: erythema of the palatine mucosa, fissure erythematous lips, "strawberry tongue"; Rash: Polymorphus, usually urticarial erythematous rash mainly in external extremities. The rash can spread to trunk; Extremities changes: Edema of hand and feet, erythema of palms & soles, desquamation of fingertips; Bilateral non-exudative conjuctival erythema, and Cervical lymphadenopathy for at least 15 milimeters.
History and Symptoms
awasaki disease often begins with a high and persistent fever that is not very responsive to normal doses of acetaminophen or ibuprofen. The fever may persist steadily for up to two weeks and is normally accompanied by irritability. Affected children develop red eyes, red mucous membranes in the mouth, red cracked lips, a "strawberry tongue", iritis, keratic precipitates (detectable by an ophthalmologist but usually too small to be seen by the unaided eye), and swollen lymph nodes. Skin rashes occur early in the disease, and peeling of the skin in the genital area, hands, and feet (especially around the nails and on the palms and soles) may occur in later phases. Some of these symptoms may come and go during the course of the illness. If left untreated, the symptoms will eventually relent, but coronary artery aneurysms will not improve, resulting in a significant risk of death or disability due to myocardial infarction (heart attack). If treated in a timely fashion, this risk can be mostly avoided and the course of illness cut short.
Physical Examination
Laboratory Findings
Kawasaki disease is diagnosed on clinical presentation, although the laboratory findings are non specific for the diagnosis of Kawasaki disease normocytic anemia, thrombocytosis, with platelets ≥ 450×103/μL (450 × 109/L) after first week of acute disease leucocytosis with white blood cell count ≥ 15,000/μL (15.0 × 109/L), elevated erythrocyte sedimentation rate, elevated liver enzyme levels, hypoalbuminemia with ≥ 3.0g/dL (30g/L), elevated C-reactive protein, hyponatremia and sterile pyuria can be noted on laboratory investigations.
Electrocardiogram
Electrocardiogram in Kawasaki disease may show evidence of ventricular dysfunction or, occasionally arrhythmia due to myocarditis. However in acute disease the electrocardiogram may show prolonged PR interval, nonspecific ST changes, T-wave changes and increased Q/R ratio which are consistent with myocarditis.
X Ray
The abnormal findings on the chest x-ray in Kawasaki disease are non specific which may include peribronchial cuffing, a reticulogranular pattern, pleural effusion, atelectasis and air trapping. In rare circumstances several years after resolution of the first episode, within the elderly population, calcifications of the coronary artery will lead to coronary artery aneurysms. These aneurysms may be visualized using a plan radiograph. This presentation is described as an “Aunt Minnie” sequelae of Kawasaki disease.
Echocardiography
X Ray
The abnormal findings on the chest x-ray in Kawasaki disease are non specific which may include peribronchial cuffing, a reticulogranular pattern, pleural effusion, atelectasis and air trapping. In rare circumstances several years after resolution of the first episode, within the elderly population, calcifications of the coronary artery will lead to coronary artery aneurysms. These aneurysms may be visualized using a plan radiograph. This presentation is described as an “Aunt Minnie” sequelae of Kawasaki disease.