Long-term prognosis of patients with Kawasaki disease: at risk for future atherosclerosis?

Kawasaki disease causes coronary artery lesions, such as dilatation, aneurysms, stenosis, and even occlusion in young children, and is one of the most common acquired heart diseases in developed countries. More than 10,000 new cases are reported in Japan every year. In its acute phase, severe coronary arteritis induces morphological changes in coronary arteries. Treatments for Kawasaki disease aim to eliminate coronary artery inflammation as quickly as possible to reduce the chance of causing coronary lesions. Immunoglobulin therapy with aspirin has become the standard therapy of first choice and helps attenuate coronary lesions. In addition to coronary artery disturbances in the acute phase, sclerotic vascular changes were observed in post-Kawasaki disease patients who did not have coronary lesions in the acute phase. Recent studies have revealed peripheral vasculature endothelial dysfunction in post-Kawasaki disease patients with and without coronary lesions. The risk factors for the development of atherosclerosis in adults, such as C-reactive protein, oxidative stress, and inflammatory cytokines, are also increased in the remote phase of Kawasaki disease. This morphological and functional endothelial dysfunction as Kawasaki disease vascular sequelae may suggest the early development of atherosclerosis in patients with Kawasaki disease. However, no direct evidence for this early development has been found so far. Kawasaki disease was first reported slightly more than 40 years ago. The first documented post-Kawasaki disease patients are now becoming old enough to have atherosclerosis. Some case reports suggest myocardial infarction with atherosclerotic changes in young adults who are believed to have a history of Kawasaki disease. This paper reviews Kawasaki disease from the perspective of long-term prognosis.