Prompt Diagnosis, Careful Follow-up Essential When Managing Kawasaki Disease
Effective management of Kawasaki disease requires a prompt diagnosis and careful follow-up, according to an updated scientific statement from the American Heart Association (AHA).
The statement provides a revised diagnostic algorithm, along with new insights into the epidemiology, genetics, pathology, natural history and long-term outcomes of the disease.
AHA published guidelines for the diagnosis, treatment and long-term management of Kawasaki disease in 2004.
The updated statement, which appeared online on March 29 in Circulation, incorporates new evidence regarding underlying pathological processes; an algorithm to ensure capture of incomplete Kawasaki disease during the effective window of therapy; improved management of the acute illness that includes the use of additional therapies for patients refractory to intravenous immunoglobulin; greater use of Z scores for classifying coronary artery involvement; greater specification of long-term management based on both initial and current coronary artery involvement; and an acknowledgment of the care needs of a growing population of adults with previous history of Kawasaki disease and coronary artery aneurysms.
About 25 cases of Kawasaki disease are diagnosed per 100,000 children under 5 per year in North America, with the highest risk in children of Japanese ancestry.
The cause of Kawasaki disease remains unknown, but at least six genes have been implicated in the susceptibility to the condition.
Kawasaki disease vasculopathy is characterized by necrotizing arteritis, subacute/chronic vasculitis and luminal myofibroblastic proliferation primarily in the muscular arteries, but atherosclerotic features are not characteristic even in late deaths or transplants.
The diagnosis of classic Kawasaki disease remains essentially unchanged, but the expert writing group established a new algorithm for the evaluation of suspected incomplete Kawasaki disease that follows different pathways based on initial values of C-reactive protein and erythrocyte sedimentation rate.
The mainstay of initial treatment for both complete and incomplete Kawasaki disease is a single high dose of intravenous immunoglobulin together with acetylsalicylic acid, which should be initiated as soon as the diagnosis is established.
Treatment options for patients resistant to intravenous immunoglobulin (IVIG) include a second infusion of IVIG, IVIG plus prednisone, infliximab, cyclosporine, anakinra, cyclophosphamide and plasma exchange.
Long-term management aims to prevent thrombosis and myocardial ischemia while maintaining optimal cardiovascular health, which requires the development of effective and collaborative programs between pediatric and adult cardiology providers.
The guideline also provides detailed risk-stratified recommendations for effective long-term evaluation and management.
“These recommendations provide updated and best evidence-based guidance to healthcare providers who diagnose and manage Kawasaki disease, but clinical decision making should be individualized to specific patient circumstances,” the authors conclude.