Kawasaki disease

Kawasaki disease is a disease that involves inflammation of the blood vessels. It is typically diagnosed in young children, but older children and adults can also develop this condition. Kawasaki disease begins with a fever that lasts at least five days. Other classic symptoms may include red eyes, lips, and mouth; rash; swollen and red hands and feet; and swollen lymph nodes.^[1] Sometimes the disease affects the coronary arteries which carry oxygen-rich blood to the heart, which can lead to serious heart problems. Kawasaki disease occurs most often in people of Asian and Pacific Island descent.^[2] The cause of Kawasaki disease is unknown.^[1][2] An infection along with genetic factors may be involved.^[2][3] Treatment includes intravenous gamma globulin and high doses of aspirin in a hospital setting.^[1][2] Prognosis is generally very good, but in cases of heart complications it depends on the severity of the coronary disease.^[3] 

The cause of Kawasaki disease (KD) is unknown. The disease results when cells move into the tissues and buildup there, leading to vascular damage, but what causes the cell buildup in the first place is unknown. The body's response to a virus or infection combined with genetic factors may cause the disease. However, no specific virus or infection has been identified, and the role of genetics is not well understood.^[2][4]

Genetic factors appear to be important to this disorder, as suggested by the increased frequency of the disease in Asian and Asian-American populations and among family members of an affected child. A number of gene variants (polymorphisms) are associated with an increased risk of developing (susceptibility) KD, and some of these variants are also associated with coronary artery lesions and aneurysm formation.[12298]

Other theories suggest that the disease is caused by a response from the body's immune system.^[5]

KD is not contagious; it cannot be passed from one person to another.^[2] Other risk factors include being a male gender, being between six months and five years of age, and having a family history of KD.^[5]

Although Kawasaki disease is generally self-limiting, 10-15% of children may develop problems in their coronary arteries, even after being treated with aspirin and intravenous gamma globulin (purified antibodies--also known as IVIG).^[1] Inflammation of the heart muscle (myocardium), heart valves (endocardium) and/or sac surrounding the heart (pericardium) may occur acutely (at the onset of the disease) or many years later.^[6] The most common late complication is the persistence of coronary artery aneurysms (weakened areas of blood vessel that balloons out). Such aneurysms can lead to heart attack at a young age or later in life.^[1]

In addition, adults may present with ischemic heart disease (ischemic means that an organ, in this case the heart muscle, has not received enough blood and oxygen) as a sequela (late effects) of unrecognized Kawasaki disease in childhood.^[7] 

 

Long-term follow-up in individuals with Kawasaki disease is recommended. The goal of long-term management is to prevent a block of bloodflow to the heart, caused by a blood clot in the coronary artery (coronary thrombosis) and to treat any resulting reduction of blood flow and oxygen to the heart (myocardial ischemia). There are few studies that have been published to assist in creating guidelines for management. It has been suggested that the management should vary depending on the severity of coronary artery involvement.^[8]

In general, cardiac imaging such as computerized tomographic angiography (CTA) magnetic resonance imaging (MRI), and echocardiogram are recommended with use of CTA and MRI being utilized more frequently given evidence of more accurate imaging with age. Abnormalities of the coronary arteries may require ongoing medication, interventional catheterization, and/or cardiac surgery.^[9][8]