Background The incidence of Kawasaki disease has been increasing since it was first reported by Tomisaku Kawsasaki in 1967. Among complications of the condition, the formation of coronary artery aneurysms is the most important. In particular, giant aneurysms with diameters that exceed 8 mm are likely to not regress and result in serious complications, such as acute myocardial infarction.
Objectives To understand the long-term course of patients with giant aneurysms and Kawasaki disease as well as to consider the cause of aneurysm formation and its appropriate treatment.
Methods We retrospectively studied the long-term course of 12 cases of giant coronary artery aneurysms accompanied with Kawasaki disease, which were being followed at Shiga University of Medical Science Hospital, Omihachiman Community Medical Center, and Nagahama Red Cross Hospital. These are three major facilities in Shiga prefecture of Japan, whose population is 1.4 million, comprising 200,000 children.
Results Ten male and two female patients were included. The average current age was 16.8 years, the median age was 14.3 (10.7–18.9) years, and 5 cases were of adults. The average age at the time of onset was 3.5 years, the median age was 3.7 years (1.8 - 4.4), and all experienced onset between 1 and 5 years of age. The mean period from onset to treatment start was 6.7 days (median 5.0 (4.3–8.3)), but the average period until fever declined was 16.0 days and only three patients' temperature was reduced in 10 days. Aneurysm formation occurred at 14.1 days on average (median 12 (10–17)). The average size of the maximum coronary artery aneurysm at onset was 11.3 mm, and the median size was 9.5 mm (8.8 mm – 13.8 mm).The average and median follow-up periods were 13.2 years and 11.7 years (5.4–13.4), respectively. The number of patients received steroid therapy was four, and all their onset was after 2006. None received infliximab or underwent plasmapheresis.
During the course of the condition, all patients underwent multiple centripetal echocardiography. Among all cases, 7 underwent coronary angiography CT, 10 underwent myocardial scintigraphy. All 12 patients underwent cardiac catheterization and the total number of underwent cardiac catheterization for them was thirty-one. Two adult patients had a history of acute myocardial infarction and had undergone cardiac bypass surgery. Through this survey, we found that 9 cases developed giant coronary artery aneurysm between 1983 and 2007, and 3 cases between 2012 and 2015 used prednisolone.
Conclusions All patients are currently receiving anticoagulant therapy and undergoing diagnostic imaging. In our prefecture, the incidence of giant coronary artery aneurysm accompanied with Kawasaki disease has been decreasing gradually. From 2007 to 2012, in which high dose gamma globulin therapy (2g/kg) has become commonly underwent for Kawasaki disease in Japan, there were no giant aneurysm formation in our hospitals. Three patients in whom giant aneurysms developed between 2012 and 2015 were taking oral prednisolone, thereby suggesting a relationship between prednisolone and giant aneurysm formation.
Disclosure of Interest None declared