Top Things to Know: Diagnosis, Treatment, & Long-Term Management of Kawasaki Disease
Published: March 29, 2017
- Kawasaki disease (KD) is an acute vasculitis of childhood that leads to coronary artery aneurysms in ≈25% of untreated cases. It accounts for 5% of acute coronary syndromes (ACS) in adults <40 years of age, and is the leading cause of acquired heart disease in children in developed countries.
- First described in Japan, KD has now been diagnosed worldwide, but the disease remains markedly, i.e., ~10 fold, more common in children in Japan.
- KD is an acute, self-limited febrile illness that predominantly affects children <5 years of age; the incidence in boys is about 1.5 times higher than in girls.
- The diagnosis of KD rests on the identification of principal clinical findings and the exclusion of other clinically similar entities with known causes.
- The cause remains unknown. We have no means to prevent KD. Until these are defined, an exact diagnostic test remains elusive, and acute treatment remains somewhat empirical.
- Careful early management of evolving coronary artery abnormalities is essential, and an increased frequency of assessments and escalation of thromboprophylaxis is necessary.
- Although intravenous immune globulin (IVIG) is the mainstay of initial treatment, the role for additional primary therapy in selected patients is discussed. Approximately 10% to 20% of patients do not respond to initial intravenous immune globulin (IVIG Resistance).
- The current scientific statement incorporates
- new evidence regarding underlying pathological processes
- an algorithm to ensure capture of incomplete KD during the effective window of therapy
- improved management of the acute illness that includes the use of additional therapies for IVIG refractory patients
- 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
- acknowledgement of the care needs of a growing population of adults with a previous history of KD and coronary artery aneurysms
- Effective care strategies to address psychosocial concerns and to ensure transition to uninterrupted expert adult cardiology care are essential to optimize quality of life across the lifespan.
- Although this scientific statement provides updated and best evidence-based guidance to healthcare providers who diagnose and manage Kawasaki disease, clinical decision-making should be individualized to specific patient circumstances.
Citation
McCrindle BW, Rowley AH, Newburger JW, Burns JC, Bolger AF, Gewitz M, et al; on behalf of the American Heart Association Rheumatic Fever, Endocarditis, and Kawasaki Disease Committee of the Council on Cardiovascular Disease in the Young; Council on Cardiovascular and Stroke Nursing; Council on Cardiovascular Surgery and Anesthesia; and Council on Epidemiology and Prevention. Diagnosis, treatment, and long-term management of Kawasaki disease: a scientific statement for health professionals from the American Heart Association [published online ahead of print March 29, 2017]. Circulation. doi: 10.1161/CIR.0000000000000484.