Expert Health Articles

Kawasaki Disease

Angelique Bermudez, RN

Blanchard Valley Hospital Emergency Room

Kawasaki disease is a self-limiting, acute febrile illness associated with generalized vasculitis. Generalized vasculitis is defined as the inflammation of the small and medium-sized blood vessels, causing thickening and obstruction of coronary arteries, which can lead to coronary artery aneurysms, myocardial infarction and sudden death. The diagnosis of Kawasaki disease is still unknown. However, researchers have found that a viral or bacterial infection could be responsible due to its acute, self-limiting and community outbreaks ability. Kawasaki disease affects all races and is the leading cause of acquired heart disease in children in western countries. The mortality rate of 75 percent occurs within the first six weeks of clinical presentation. 10 to 15 percent of Kawasaki cases lead to permanent coronary artery lesions. Coronary artery aneurysms can occur in 25 percent of untreated cases and five percent of those who were treated. The most prevalent cases of Kawasaki disease were in Japan, affecting 174 per 100,000 children per year. In the United States, there are 17 to 18 cases per 100,000 children affected per year. Kawasaki disease is mostly seen in patients under five years old. Kawasaki Disease is more common in children under five years of age with occurrences higher in males, including a two to one, male to female ratio. Although Kawasaki Disease affects all races, the highest rates are in patients of Asian and Pacific Island ancestry. Researchers found the reason why Japan has the highest prevalence of Kawasaki disease is due to an airborne triggering marker for the disease, affecting genetically susceptible individuals. However, diagnosis of the disease in western countries is still unknown. Furthermore, researchers report Kawasaki disease is more common in males due to a genetic factor rs1801274, a specific gene found in males, making them more susceptible to this disease.

The U.S. Centers for Disease Control and Prevention (CDC) provides diagnostic criteria for Kawasaki disease including fever lasting over five days with at least four of the following:

  • Swollen and tender lymph node, mostly seen in neck area
  • Rash
  • Redness and swelling of hands and feet
  • Oral mucosa changes
  • Bilateral conjunctivitis without drainage

There is no definite diagnostic test to diagnose Kawasaki disease. However, diagnostic tests such as echocardiogram, EKG or angiography may be ordered to evaluate cardiac involvement. Early diagnosis is vital in Kawasaki disease due to its cardiac complications.

Hence, prompt treatment is key to prevent complications. IV immunoglobulin is the gold standard for treatment of Kawasaki disease. In addition, other pharmacological management include high doses of aspirin to prevent clot formation, steroids for inflammation and swelling, analgesics for pain management and antipyretics for fever control. However, due to pediatric exposure of aspirin, it’s highly recommended to have the patient get an annual influenza vaccine and avoid exposure to varicella to prevent Reye’s syndrome. After treatment of Kawasaki Disease, patient’s families are encouraged to continue childhood vaccinations except for live vaccination such as MMR and Varicella. Live vaccinations should be postponed three months after IV immunoglobulin administration.

After treatment of Kawasaki Disease, patients are still at risk for a heart attach months to years after the acute onset. When a coronary artery aneurysm forms as complication of Kawasaki Disease, scar tissues will begin to form and eventually heal the blood vessel wall. However, the blood vessel wall will not be entirely normal as scar tissue will continue to form, eventually causing narrowing of the blood vessels and increasing the patient’s risk for a heart attack later in life.