Kawasaki Disease

Kawasaki disease (KD) is an inflammation of the blood vessels (usually the coronary arteries) that happens quickly and goes away on its own.

Last Updated: February 25, 2024

KD usually affects children younger than five but it can also affect adults. Furthermore, boys are more likely to be diagnosed and die from the disease compared to women (1.5:1 ratio). Common manifestations of KD are high fever, inflammation of the mouth and throat membranes,  reddish rash, and swollen lymph nodes. 

The specific reason why Kawasaki disease occurs is unknown, but there is evidence that suggests that it is due to an infection or an improper immunological response to infection.

For many children with Kawasaki disease, the first sign of illness is a high fever that goes up and down (remittent fever) and lasts for about one to two weeks without treatment. In some cases, the fever can last up to three or four weeks. Other signs include inflammation of the whites of the eyes (bilateral conjunctivitis), inflammation of the mucous membranes of the mouth and throat, which causes dry, red, cracked lips and a strawberry-red tongue, swelling of the lymph nodes in the neck (cervical lymphadenopathy), redness and swelling of the hands and feet, and a reddish rash that usually affects the trunk and often the groin area. By the second or third week, the skin on the tips of the fingers and toes may peel (desquamate) and spread to the rest of the hands and feet.

The signs and symptoms are the basis for diagnosing Kawasaki Disease. Doctors use the list below to say that a patient has KD. 

Warm CREAM and FEBRILE Mnemonics for KD Criteria

Warm = Fever for > 5 days

  • C = Conjunctivitis without exudate
  • R = Rash
  • E = Edema or erythema of hands or feet, followed by desquamation and nail changes
  • A = Adenopathy, often unilateral, cervical node > 1.5 cm 
  • M = Mucosal erythema, fissures or crusting of lips or strawberry tongue
  • F = Fever for > 5 days
  • E = Enanthem of mucosal membranes
  • B = Bulbar conjunctivitis
  • R = Rash, erythematous, polymorphous
  • I = Internal organ involvement: coronary, abdominal, pneumonitis, hepatitis, orchitis
  • E = Extremity changes, initial edema, and erythema, desquamation, nail changes

After a KD diagnosis, only an echocardiography is required.

Treatment

The goal of treatment is to reduce inflammation in the coronary arteries to lower the risk of coronary artery aneurysm (CAA), which is most likely to happen two to four weeks after the disease starts. Physicians usually give high doses of both IVIG and aspirin (ASA).

Supportive care is also essential such as cold compression, fluids for hydration, and medication for the pain and swelling.

Because KD has unknown causes except that current studies point towards infection, it is best that children get the necessary vaccines to prevent common infections in the children. 

When a child is on aspirin therapy, it is especially important to make sure they are up to date on their flu and chickenpox shots.

Live virus vaccines, such as the measles-mumps-rubella, varicella, and nasal live virus influenza vaccines, should not be given to children for 11 months after IVIG. This is because the IVIG might stop the body from making antibodies against these viruses.

References

Owens AM, Plewa MC. Kawasaki Disease. [Updated 2022 Aug 7]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2022 Jan-. Available from: https://www.ncbi.nlm.nih.gov/books/NBK537163/ 

National Organization for Rare Diseases (2022). Kawasaki Disease. Retrieved December 8, 2022, from https://rarediseases.org/rare-diseases/kawasaki-disease/

Cleveland Clinic (2022). Kawasaki Disease. Retrieved December 8, 2022, from https://my.clevelandclinic.org/health/diseases/13457-kawasaki-disease

Last Updated: February 25, 2024