Clinical Overview of Kawasaki Disease

Key points

  • Kawasaki Disease (KD) is a systemic inflammatory disease characterized by a fever plus 4 or more other commonly associated symptoms.
  • Standard treatment for KD includes intravenous immunoglobulin (IVIg) plus aspirin; children experiencing or at higher risk for cardiac complications may need additional treatment.
  • CDC disease surveillance aims to better understand KD symptoms and disease prevalence in the United States.
Healthcare providers huddle together and look down at a tablet

Overview

Kawasaki disease (KD), also known as Kawasaki syndrome, is a disease mostly affecting children younger than 5 years old that can cause damage to the heart and blood vessels. It affects boys more often than girls. The cause of KD is not known.

Most children with KD recover with proper treatment.

Signs and symptoms

Kawasaki disease (KD) is an illness in a patient with:

  • Fever that lasts 5 or more days OR
  • Fever until the intravenous immunoglobulin is given, if given before the fifth day of fever
  • AND with at least 4 of the following 5 clinical signs:
    • Rash
    • Swelling and redness of their hands and feet
    • Cervical lymphadenopathy (at least 15 cm in diameter)
    • Bilateral conjunctival injection
    • Oral mucosal changes

Atypical KD

Patients whose illness does not meet the above KD case definition but have fever and coronary artery abnormalities are classified as having atypical or incomplete KD.

Treatment

Standard treatment for children includes:

  • 2 g/kg intravenous immunoglobulin (IVIg) AND
  • Aspirin administered at either moderate dose (30-50 mg/kg/day) OR high dose (80-100 mg/kg/day)

Additional therapies may be recommended for patients experiencing or at higher risk for cardiac complications, including patients with persistent or recurring fever after initial treatment.

These therapies may include:

  • Second dose of IVIg
  • Corticosteroids or other anti-inflammatory medications
  • Anticoagulants for patients at risk of blood clots

Case reporting

Healthcare professionals can submit a report using the CDC standardized KD case report form:

Send the completed form to the mailing address provided on the form or fax to 404-471-8768.

What CDC is doing

Kawasaki disease was first described in 1967 in Japan by Tomisaku Kawasaki. The first cases outside of Japan were reported in 1976 in Hawaii. CDC has operated a KD surveillance system since 1976 and uses several data sources to track and better understand KD in the United States.

CDC analyzes large hospital discharge databases in the United States to learn more about the number of cases of KD and what symptoms children are having. Because most children with KD are hospitalized, the hospitalization rate is a good estimate of how often KD is being diagnosed.

CDC also has a voluntary KD reporting system for health care providers and health authorities to let CDC know when and where they are seeing cases. This system provides CDC with information like case symptoms and whether the child is experiencing serious complications.

CDC continues to conduct special studies to further describe how often KD is diagnosed and which symptoms are most common.

Resources

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Content Source:
National Center for Emerging and Zoonotic Infectious Diseases (NCEZID); About Division of High-Consequence Pathogens and Pathology