Association of Coronary Vasculitis Sequelae With Atherosclerosis in Adults With a History of Kawasaki Disease: Autopsy Pathological Findings

BACKGROUND: In Kawasaki disease (KD), coronary arteritis occurring in infancy can lead to long-term sequelae, such as luminal dilatation, stenosis, intimal thickening, and calcification. However, the long-term evolution of these lesions, particularly the effect of superimposed atherosclerosis in adulthood, remains poorly understood. This study aimed to investigate coronary artery lesions in adults with a history of KD and clarify the relationship between sequelae of vasculitis and atherosclerosis. METHODS: We analyzed 7 autopsy cases of sudden cardiac death in adults with a confirmed history of KD and documented coronary artery aneurysms. Clinical data, including acute-phase treatment history, body mass index, heart weight, coronary risk factors, and detailed coronary histopathology, were evaluated. RESULTS: All aneurysms were located at the coronary ostia and showed marked intimal thickening with severe calcification, leading to luminal narrowing. Thrombotic occlusion within the aneurysm was identified in 1 case. In 3 patients aged older than 30 years, atherosclerotic changes were observed in the thickened intima of persistent aneurysmal segments or dilated portions. One patient with a regressed aneurysm died of acute myocardial infarction caused by plaque rupture. Sequelae of vasculitis were widely distributed in nonaneurysmal coronary branches, and atherosclerotic changes were also noted in these regions. CONCLUSIONS: In adults with persistent coronary aneurysms due to KD, atherosclerotic lesions may develop not only in aneurysmal segments but also in nondilated arteries with scarring due to vasculitis. Individuals with prior KD and coronary aneurysms may require long-term management for thrombotic risk and the prevention of atherosclerosis, beginning in early adulthood.