AHA Scientific Statement on diagnosis and management of Kawasaki disease

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The American Heart Association (AHA) has published an updated scientific statement for health professionals on diagnosis, treatment, and long-term management of Kawasaki disease. The statement published online March 29, 2017 in Circulation recommends that clinical decision making in patients with Kawasaki disease should be individualized to specific patient circumstances.

Recognizing that prompt diagnosis is essential, these guidelines include an updated algorithm to aid the clinician in diagnosis of patients with suspected incomplete Kawasaki disease. The diagnosis of incomplete or atypical Kawasaki disease should be considered in any infant or child with prolonged unexplained fever, fewer than 4 of the classical clinical findings, and compatible laboratory or echocardiographic findings.
The guidelines recommend intravenous immunoglobulin (IVIG) as the primary treatment to be administered as soon as the diagnosis can be established within the first 10 days of illness. Treatment should not be delayed for lack of echocardiography. However, the guidelines recommend against IVIG administration to patients beyond the 10th day of illness in the absence of fever, raised inflammatory markers, or coronary artery abnormalities. Adjunctive therapies – corticosteroids, infliximab, etanercept – may benefit patients at high risk for development of coronary artery aneurysms.

For patients at increased risk of thrombosis, for example, with large or giant aneurysms (≥8 mm or Z score ≥10) and a recent history of coronary artery thrombosis, “triple therapy” with ASA, a second antiplatelet agent, and anticoagulation with warfarin or LMWH may be considered.

The goal of therapy is to prevent thrombosis and myocardial ischemia while maintaining optimal cardiovascular health.

Risk-stratification of patients for effective long-term evaluation and management should be done according to the relative risk of myocardial ischemia, either related to coronary artery thrombosis or stenoses/occlusions. The guidelines also recommend development of effective and collaborative programs between pediatric and adult cardiology providers for effective long-term management of the patient.
(Source: Medscape, Circulation March 2017)

Dr KK Aggarwal
National President IMA & HCFI