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Treatment and Diagnostic Testing Table 2. Quality of Evidence and Strength of Recommendations Quality of Evidence Interpretation High-quality evidence Studies that provide high confidence in the effect estimate. New data from future studies are thought unlikely to change the effect. Moderate-quality evidence Studies that provide confidence that the true effect is likely to be close to the estimate but could be substantially different. Low-quality evidence Studies that provide limited confidence about the effect. e true effect may be substantially different from the estimate. Very low-quality evidence Studies that provide very little certainty about the effect. e true effect may be quite different from the estimate. Strength of Recommendation Interpretation Strong recommendation Supported by moderate- to high-quality evidence (e.g., multiple randomized controlled trials). Recommended course of action would apply to all or almost all patients. Only a small proportion of clinicians/patients would not want to follow the recommendation. In rare instances, a strong recommendation may be based on very low- to low-certainty evidence. For example, an intervention may be strongly recommended if it is considered benign, low-cost, without harms, and the consequence of not performing the intervention may be catastrophic. An intervention may be strongly recommended against if there is high certainty of more harm than the comparison with very low or low certainty about its benefit. Conditional recommendation Supported by lower-quality evidence or a close balance between desirable and undesirable outcomes. Recommended course of action would apply to the majority of the patients, but the alternative is a reasonable consideration. Conditional recommendations always warrant a shared decision-making approach. Good Practice Statement Good practice statements were agreed upon when evidence was sufficiently compelling that a formal vote was unnecessary. Ungraded Position Statement Evidence for a PICO question did not support a graded recommendation or did not favor one intervention over the other. Guidance for this question provided by the voting panel. Table 3. Diagnostic Testing and Treatment of Patients With KD Recommendations/Ungraded Position Statement/ Good Practice Statement Certainty of Evidence Good Practice Statement: Intravenous immunoglobulin is the standard-of-care therapy for the initial treatment of KD. High For patients with acute KD who are at high risk of IVIG resistance or developing coronary artery aneurysms, using IVIG with adjunctive GCs as initial therapy is conditionally recommended over IVIG alone. Low For patients with acute KD who are at high risk of IVIG resistance or developing coronary artery aneurysms, using IVIG with other non-GC immunomodulatory immunosuppressive agents as initial therapy is conditionally recommended over IVIG alone. Very low For patients with incomplete KD, prompt treatment at time of diagnosis with IVIG is strongly recommended over delaying treatment until day 10 or later. Low For patients with acute KD and suspected or diagnosed macrophage activation syndrome, treatment with IVIG for KD and additional agents to treat macrophage activation syndrome is strongly recommended. Very low For patients with acute KD and persistent fevers aer initial treatment with IVIG, a second course of IVIG is conditionally recommended over use of GCs. Very low Ungraded Position Statement: For patients with acute KD and persistent fevers aer repeated treatment with IVIG, either non-GC immunosuppressive therapy or GCs may be used. Low For patients with acute KD, using aspirin is strongly recommended over no aspirin. Very low For patients with acute KD with subsequent resolution of fevers, continued daily monitoring for fevers is strongly recommended over no monitoring for fevers. Very low For patients with acute KD with arthritis that persists aer IVIG treatment and without coronary artery aneurysms, using non-steroidal anti-inflammatory drugs (NSAIDs) to treat arthritis is conditionally recommended over not using NSAIDS. Very low Additional diagnostic testing recommendations For children with suspected incomplete KD and fever, obtaining an echocardiogram with coronary artery measurements without delay is strongly recommended over not obtaining an echocardiogram. Very low For children with unexplained shock physiolog y, obtaining an echocardiogram with coronary artery measurements is strongly recommended. Very low For children with unexplained macrophage activation syndrome, obtaining an echocardiogram with coronary artery measurements is strongly recommended. Very low Definitions and Medications

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