Primer on the Rheumatic Diseases. Kawasaki syndrome is known as a systemic vasculitis. This means that it’s an inflammatory disease that affects the blood vessels. Specifically, it affects medium sized muscular arteries, like the coronary arteries which supply blood to the heart. It is this complication of the coronary arteries that makes this disease dangerous.
It isn’t entirely clear what causes Kawasaki disease. However, it is believed to be the outcome of an underlying infection. The most prominent symptom of Kawasaki disease is the change that occurs to the skin of the lips. The disease usually begins with a high fever that lasts several weeks. A few days of the fever begins, a rash is on the trunk. The rash usually consists of irregular red patches that aren’t itchy. The skin of the hands and feet may peel and slough off. Most striking, however, is the intense red color that the skin of the lips and tongue turns. Medical students often remember the symptoms of Kawasaki disease by the mneumonic CRASH and Burn. This stands for: Conjunctival injection (redness of the white portion of the eye), Rash, Adenopathy (swelling of the glands in the neck), Strawberry tongue, Hands and Feet desquamation (loss of skin), and high fever (Burn). Any or all of these symptoms is expected to result in an immediate journey to the emergency room. There are no laboratory tests for Kawasaki disease. Diagnosis is based upon the physical examination and clinical criteria though some tests can assist the physician if the clinical criteria are somewhat equivocal. Common findings include high white count, anemia, elevated liver proteins, elevated ESR, and low albumin (a form of protein present in the blood).
Treatment consists of IVIG early during the course of the disease and aspirin to reduce inflammation. IVIG is the short run for intravens immunoglobulin. Immunoglobulin is relates to the antibodies that our body produces to fight of disease. IVIG is simply antibody that has been purified and concentrated so as to be given to a person as therapy. It is used to treat many conditions including Kawasaki disease, Multiple Sclerosis, Cancer, Guillian Barre syndrome, and many other disease. IVIG has been found to reduce the impact of aneurysm in the coronary arteries from 25% to fewer than 5% if administered during the acute stage of the disease. Aspirin is used to address the general inflammation that occurs throughout the body. It is started at a high dose of 80 to 100 mg per kg per day. This dose is continued until the fever has subsided and is then reduced gradually. Aspirin alone isn’t sufficient to avoid the coronary artery abnormalities.
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Four to six weeks after diagnosis of Kawasaki disease, a transthoracic echocardiogram (TTE) will be administered. This is simply an ultrasound, similar to the sonograms used in pregnancy. This is used to examine the coronary arteries supplying blood to the heart. The purpose is to screen for aneurysms and the reason it is performed several weeks later is because the aneurysms don’t form until then. Small and medium-sized the aneurysms can be followed with serial TTEs and will usually resolve within five years. According to the Primer on the Rheumatic Diseases, factors that are affiliated with regression of the aneurysms include small size of female sex, the aneurysm, and an age less than 1 year. If a large aneurysm is found, surgery may need to be carried out in order to repair it, before it ruptures.
An echocardiogram will be paid to a child with Kawasaki disease to observe the absence or presence of heart artery aneurysms while the child is found in the hospital and then a second echocardiogram will be administered a few weeks after hospital dismissal to be sure that no heart artery aneurysms have developed.
Kawasaki syndrome is a major disease that requires immediate attention. If your child exhibits any of the symptoms listed above, it’s important to go to the emergency room immediately. Early intervention is the key. Chances of recovery without complication are good with IVIG end the early administration of aspirin. However, without IVIG, there is a one in four chance that your child will develop aneurysms in the coronary arteries. The sooner the treatment is started, the more it is probable to have benefit. When in doubt, go to the emergency room more urgent care center and if the physician doesn’t specifically mention Kawasaki syndrome, if it has been considered. There’s no harm in asking.
Electrocardiogrammay also be requested to evaluate the impulses of the heartbeat the child’s as Kawasaki disease can cause serious complications in the heart as a coronary artery aneurysm.
Kawasaki disease is treatable but prompt medical intervention is necessary to prevent serious heart complications that are potentially fatal to the child. The success of treatment is in the early detection of the disease. The goal of treatment in Kawasaki disease is to lower the fever and the inflammation that can potentially damage the heart.
Source: Chapter 21-Vasculitides: E. Kawasaki Syndrome. In: Klippel JH. Primer on the Rheumatic Diseases 12th ed. Arthritis Foundation: Atlanta, GA; 2001. P409-413.