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Kawasaki Disease

Definition

Kawasaki Disease is a “vasculitis” that affects small or medium-sized blood vessels of the body (“vasculo”- refers to the blood vessels, “-itis” means inflammation). The etiology of Kawasaki Disease is unknown, although research suggests that an infectious agent (ie, viruses) results in an exaggerated “auto-immune” process resulting in a cascade of inflammation that involves the blood vessels.  Almost any blood vessel in the body may be affected, although this may also involve the coronary arteries of the heart, which are very small arteries that supply the heart muscle with it’s own blood supply. The result is dilation or “ectasia” of the arteries. Significant dilation may weaken the blood vessel walls, resulting in “aneurysm” formation.

The sequence of changes that occur in the blood vessels as part of the inflammatory process is quite complex and outside the scope of this forum. In several ways, these changes mimic those that occur when plaque builds within our blood vessels over time, a process called “atherosclerosis”. Not all patients with Kawasaki Disease will have inflammation of the coronary artery. Those who do, however, are at increased risk of ischemia (inadequate blood supply to the heart muscle) and accelerated atherosclerosis, depending on the degree of involvement.

Signs/Symptoms

The average age of presentation for Kawasaki Disease is 2 years, although it may occur at any age. Patients with Kawasaki typically will have high fevers for several days; these fevers cannot be attributable to any other causes (ie, pneumonia, ear infection, appendicitis, etc.). A rash is also typical, although the type of rash may be variable. Many patients will have conjunctivitis, although there will typically have no pus or crusting. Some patients will have bright red lips or a bright, swollen tongue. Swelling of the hands/feet, fingers/toes may also occur. Peeling of the skin of the hands/feet may occur if the process has occurred for a prolonged period of time (ie, several weeks). Patients with Kawasaki Disease are typically very irritable; infants in particular are very difficult to console despite attempts at fever control.  

Diagnosis

The diagnosis of Kawasaki Disease is “clinical”; it depends on the history of the patient’s illness and findings on physical examination. Laboratory tests including blood work, urine studies, etc., may support the diagnosis, though are insufficient to make the diagnosis alone. The major criteria for making the diagnosis of Kawasaki Disease include the following: 1) high fever (temperature > 100.4) for several days (>= 4-5 days), 2) conjunctivitis, 3) rash, 4) swollen lymph nodes, 5) swollen hands/feet, fingers/toes, 6) bright red lips or tongue, dry cracked lips. Blood work or urine studies may support the diagnosis, reflecting signs of inflammation. An electrocardiogram may demonstrate subtle signs of inflammation. An echocardiogram (or cardiac ultrasound/sonogram) may be required to evaluate for any inflammation of the heart. This inflammation may involve the heart muscle (myocardium – myocarditis), heart valves (endocardium – endocarditis), or coronary arteries supplying the heart muscle with it’s own blood supply (coronary arteritis, ectasia, or aneurysm).

Treatment

There is no cure for Kawasaki Disease. The goal of treatment is to suppress or control the inflammatory process. The initial treatment of Kawasaki Disease involves inpatient, hospital care. The mainstays of treatment include 1) IntraVenous Immune Globulin (IVIG), and 2) aspirin. Both seek to suppress inflammation, and secondarily treat the child's symptoms. Once the child's fever has resolved, transition to outpatient medical therapy will begin.

1) IVIG consists of immune globulin or anti-bodies, which are protective proteins that our body produces in response to infection. They help the immune cells of our body eradicate the infectious agent. These particular anti-bodies also have an anti-inflammatory effect by regulating the production of other proteins involved in the inflammatory cascade. IVIG are anti-bodies produced by other people, and so are not synthetic chemicals. IVIG is administered as a continuous intravenous infusion. The duration of the infusion varies, though typically takes 12 to 24 hours.    

2) Aspirin treatment occurs in two phases: high-dose and low-dose maintenance. The high-dose phase is used from the initiation of therapy and typically continues for 14 days, or until the child has been afebrile for 48 to 72 hours. High-dose aspirin has potent anti-inflammatory effects. The child will then be switched to low-dose aspirin for at least 6 weeks. Low-dose aspirin will prevent the platelets in the blood from clumping together and forming clots that may impede blood flow; this "anti-coagulant" effect of aspirin is why adults with heart disease need to take aspirin on a daily basis - to prevent clots that block the arteries of the heart. If the child's Kawasaki Disease has affected his or her heart (i.e., dilation of the coronary arteries), he will need to continue to take low-dose aspirin until the coronary artery dilation has completely resolved.  

There are rare cases when more potent anti-inflammatory agents are needed; this typically occurs when the child continues to have fever and other symptoms despite IVIG and high-dose aspirin. Occasionally, the dose of IVIG may be repeated. Other times, other medications will be used, including steroids or "immune-modulators". Management of Kawasaki Disease is most often made by your pediatrician and a team of pediatric subspecialties including Infectious Disease and Cardiology.  

 

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