Acquired Heart Disease in Kawasaki Disease Staff | June 29, 2022 Published in April 2022, the 2021 American College of Rheumatology/Vasculitis Foundation Guideline for the Management of Kawasaki Disease underscores the importance of early diagnosis and intensified treatment for those with this serious condition. Intravenous immunoglobulin (IVIG) remains the treatment mainstay, and prompt, aggressive treatment may be able to reduce the risk of serious complications. The most common form of childhood primary vasculitis globally, Kawasaki disease is also the most common cause of acquired heart disease in developed countries. Learn more. The guideline strongly recommends that for children with suspected incomplete Kawasaki disease and fever or with unexplained shock physiology, rheumatologists should: A. Obtain a simple echocardiogram B. Obtain an echocardiogram with coronary artery measurements C. Only initiate treatment after 10 days and ruling out other diagnoses D. Always test for factors that seem to increase the risk of coronary artery aneurysms, such as C-reactive protein, before initiating treatment None Time's up