Kawasaki disease is one of the most common forms of vasculitis (a condition involving inflammation of blood vessels,) that affect young children. It is not contagious, but it does occasionally occur in epidemics, primarily affecting children under the age of three. Occurrence beyond late childhood is extremely rare.
The most worrisome problem associated with Kawasaki disease is that it can cause inflammation of the coronary arteries, which supply blood to the heart. Although it is a rare complication, coronary artery inflammation can be serious or even fatal. For this reason, Kawasaki disease requires prompt diagnosis and treatment by a physician.
The cause of Kawasaki disease remains unknown, although many theories have been discussed in medical literature.
There have been reports that the COVID-19 coronavirus has caused a condition with symptoms similar to Kawasaki disease in children. This rare disorder is known as pediatric multisystem inflammatory syndrome (PMIS). For more information, read Kawasaki Disease, Pediatric Multisystem Inflammatory Syndrome, and COVID-19 Explained.
Kawasaki disease usually begins with prolonged high fever lasting for at least five days. In addition to the fever, children classically develop a variety of other symptoms caused by acute inflammation, and these help doctors make the diagnosis. These symptoms include:
In children with Kawasaki disease, the diagnosis is suspected based purely on clinical signs and symptoms. The possibility of Kawasaki disease should be considered in any child with prolonged fever lasting more than five days without another likely explanation.
Blood and urine tests may be helpful to make the diagnosis by ruling out other causes. All children with suspected Kawasaki disease should have an evaluation by a cardiologist as soon as possible with an electrocardiogram (EKG and) echocardiogram performed to determine if there is coronary artery involvement.
Kawasaki disease is treated with a medication called intravenous immune globulin (IVIG). This medication is usually administered in a hospital. Kawasaki disease treatment with IVIG is usually very effective in stopping the fever and inflammation of Kawasaki disease, and in limiting cardiac involvement when given early on in the course of the disease.
It is rare for a child taking IVIG to experience no improvement for this condition. However, some children require two treatments or additional immunosuppressive medications, including corticosteroids. In addition to IVIG, Kawasaki disease is treated with aspirin, usually for several months. Children require long-term follow up with a cardiologist, particularly if the heart was affected by the disease. Most children with Kawasaki disease recover completely following prompt diagnosis and treatment.
Reviewed and updated by Sarah Faith Taber, MD