Kawasaki disease is a childhood disease that leads to inflammation of blood vessels. It is also known as mucocutaneous lymph node syndrome and infantile periarteritis nodosa. The disease is named after a Japanese doctor Dr. Tomisaku Kawasaki who first reported the disease in children. The disease is mostly prevalent in Asian subcontinent, especially Japan. The disease has a greater predilection of affecting coronary arteries leading to coronary artery aneurysms. However, it can also affect veins, capillaries, arterioles and other larger arteries spread throughout the body.
Can You Die From Kawasaki Disease?
Initially, it was considered to be a benign disease, but recent data suggest that about 2% people affected by Kawasaki disease die of the illness. The death is subsequent to myocardial infarction caused by thrombotic occlusion of coronary artery aneurysms. In most children who are diagnosed and treated early, it runs a benign course. However, mortality rates increase in untreated children or who have been diagnosed later in life. (1)
Kawasaki has been recognized as the leading cause of acquired heart disease in children younger than 5 years of age in the United States, after surpassing rheumatic fever. It is also a risk factor associated with ischemic heart disease in adults.
Approximately 85-90% children affected with Kawasaki disease are younger than 5 years and 90-95% children affected are younger than 10 years. So, it is safe to say that it is a disease of children. Kawasaki disease is more common in males than in females, with a ratio of around 1.5:1 and death related to serious complications are also more common in males than in females.
Risk of developing cardiac complications is greater in untreated cases, with approximately 20-25% affected by it. The treatment reduces the risk to about 3-5%. Cardiac complications found in these children include aneurysms and diffuse coronary artery ectasia, myocardial infarction, valvulitis (mainly involving mitral valve), myocardial dysfunction or heart failure, pericarditis, pericardial effusions, systemic artery aneurysms, coronary artery aneurysm rupture.
The risk factors associated with the development of coronary artery aneurysm include, fever for greater than 8 days (it is the most important predictor), recurrence of fever after an afebrile episode of at least 2 days, male sex, children younger than 1 year of age, incomplete Kawasaki disease presentation, cardiomegaly, delayed diagnosis, Asian, Pacific Islander and Hispanic ethnicity. Children younger than 6 months and older than 9 years with incomplete Kawasaki disease presentation have poor disease outcome. This is due to the delay in diagnosis, which subsequently leads to progression of symptoms and increasing their severity leading to less likelihood of successful treatment and development of complications.
Kawasaki disease is divided into complete and incomplete forms. The clinical features associated with the disease include abrupt onset of prolonged high fever (102-104 degree F) unresponsive to treatment, diffuse maculopapular rash, changes in extremities (erythema of the palms and soles, desquamation of toes and fingers) diffuse erythema involving the mouth and tongue along with fissuring and bleeding of lips, bulbar conjunctivitis and cervical lymphadenopathy. If not diagnosed earlier, internal organs, such as heart may also be affected.
Diagnosis of the disease is made by combination of the clinical presentations, which meet the criteria for complete Kawasaki disease. Echocardiography is an important diagnostic tool for complete Kawasaki disease. However, other laboratory and imaging studies are more useful in the diagnosis of incomplete Kawasaki and differentiating it from other diseases.
The goal of treatment is to manage fever and inflammation of the body parts along with prevention it from affecting heart leading to coronary artery aneurysms and other cardiac complications. Intravenous Immunoglobulin (IVIG) and aspirin is the mainstay of the treatment protocol. Aspirin is used for its anti-pyretic effect as well as anti-inflammatory properties. Medium to high dose aspirin is given initially until the patient is afebrile, low dose aspirin is started after 2-3 days of fever resolution. IVIG is used for reduction of acute inflammation and to reduce cardiac complications from developing.