Sandbox: Kawasaki - ACC -2017

Template:Kawasaki - ACC -2017 Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1],Associate Editor(s)-in-Chief: Arzu Kalayci, M.D. [2]

Diagnosis, Treatment, and Long-Term Management of Kawasaki Disease (KD)

Recommendations for Cardiovascular Assessment for Diagnosis and Monitoring During the Acute Illness

Class I

"1. Echocardiography should be performed when the diagnosis of KD is considered, but unavailability or technical limitations should not delay treatment.(Level of Evidence: B) "

"2. Coronary arteries should be imaged, and quantitative assessment of luminal dimensions, normalized as Z scores adjusted for body surface, should be performed.(Level of Evidence: B) "

"3. For uncomplicated patients, echocardiog- raphy should be repeated both within 1 to 2 weeks and 4 to 6 weeks after treatment.(Level of Evidence: B) "

"4. For patients with important and evolving coronary artery abnormalities (Z score >2.5) detected during the acute illness, more fre- quent echocardiography (at least twice per week) should be performed until luminal dimensions have stopped progressing to determine the risk for and presence of thrombosis.(Level of Evidence: B) "

Class IIa

"1. To detect coronary artery thrombosis, it may be reasonable to perform echocardiography for patients with expanding large or giant aneurysms twice per week while dimensions are expanding rapidly and at least once weekly in the first 45 days of illness, and then monthly until the third month after illness onset, because the failure to escalate thromboprophylaxis in time with the rapid expansion of aneurysms is a primary cause of morbidity and mortality . (Level of Evidence: C) "

Recommendations for Initial Treatment With Intravenous Immunoglobulin (IVIG) and Asetil Salisilat Acid (ASA)

Class I

"1. Patients with complete KD criteria and those who meet the algorithm criteria for incomplete KD should be treated with high-dose IVIG (2 g/kg given as a single intravenous infusion) within 10 days of illness onset but as soon as possible after diagnosis.(Level of Evidence: A) "

Class IIa

"1. It is reasonable to administer IVIG to children presenting after the 10th day of illness (ie, in whom the diagnosis was missed earlier) if they have either persistent fever without other explanation or coronary artery abnormalities together with ongoing systemic inflammation, as manifested by elevation of ESR or CRP (CRP >3.0 mg/dL). (Level of Evidence: B) "

"2. Administrationofmoderate-(30–50mg·kg−1·d−1) to high-dose (80–100 mg·kg−¹·d−¹) ASA is reasonable until the patient is afebrile, although there is no evidence that it reduces coronary artery aneurysms. (Level of Evidence: C) "

Class III

"1. IVIG generally should not be administered to patients beyond the tenth day of illness in the absence of fever, significant elevation of inflammatory markers, or coronary artery abnormalities . (Level of Evidence: C) "

"2. The ESR is accelerated by IVIG therapy and therefore should not be used to assess response to IVIG therapy. A persistently high ESR alone should not be interpreted as a sign of IVIG resistance. (Level of Evidence: C) "

Recommendations for Adjunctive Therapies for Primary Treatment

Class IIb

"1. Administration of a longer course of corticosteroids (eg, tapering over 2–3 weeks), together with IVIG 2 g/kg and ASA, may be considered for treatment of high-risk patients with acute KD, when such high risk can be identified in patients before initiation of treatment. (Level of Evidence: B) "

Class III

"1. Single-dose pulse methylprednisolone should not be administered with IVIG as routine primary therapy for patients with Kawasaki Disease. (Level of Evidence: B) "

Recommendations for Additional Therapy in the IVIG-Resistant Patient

Class IIa

"1. It is reasonable to administer a second dose of IVIG (2 g/kg) to patients with persistent or recrudescent fever at least 36 hours after the end of the first IVIG infusion. (Level of Evidence: B) "

Class IIb

"1. Administration of high-dose pulse steroids (usually methylprednisolone 20–30 mg/kg intravenously for 3 days, with or without a subsequent course and taper of oral prednisone) may be considered as an alternative to a second infusion of IVIG or for retreatment of patients with KD who have had recurrent or recrudescent fever after additional IVIG. (Level of Evidence: B) "

"2. Administration of a longer (eg, 2–3 weeks) tapering course of prednisolone or prednisone, together with IVIG 2 g/kg and ASA, may be considered in the retreatment of patients with KD who have had recurrent or recrudescent fever after initial IVIG treatment. (Level of Evidence: B) "

"3. Administration of infliximab (5 mg/kg) may be considered as an alternative to a second infusion of IVIG or corticosteroids for IVIG-resistant patients. (Level of Evidence: C) "

"4. Administration of cyclosporine may be considered in patients with refractory KD in whom a second IVIG infusion, infliximab, or a course of steroids has failed. (Level of Evidence: C) "

"5. Administration of immunomodulatory monoclonal antibody therapy (except TNF-α block- ers), cytotoxic agents, or (rarely) plasma exchange may be considered in highly refractory patients who have failed to respond to a second infusion of IVIG, an extended course of steroids, or infliximab. (Level of Evidence: C) "

Recommendations for Prevention of Thrombosis During the Acute Illness

Class I

"1. Low-dose ASA (3–5 mg·kg−¹·d−¹) should be administered to patients without evidence of coronary artery changes until 4 to 6 weeks after onset of illness.(Level of Evidence: C) "

Class IIa

"1. For patients with rapidly expanding coronary artery aneurysms or a maximum Z score of ≥10, systemic anticoagulation with LMWH or warfarin (international normalized ratio target 2.0–3.0) in addition to low dose ASA is reasonable. (Level of Evidence: B) "

Class IIb

"1. 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. (Level of Evidence: C) "

Class III

"1. Ibuprofen and other non steroidal anti-inflammatory drugs with known or potential involvement of cyclooxygenase pathway may be harmful in patients taking ASA for its antiplatelet effects. (Level of Evidence: B) "

Recommendations for Treatment of Coronary Artery Thrombosis

Class I

"1. Coronary artery thrombosis with actual or impending occlusion of the arterial lumen should be treated with thrombolytic therapy or, in patients of sufficient size, by mechanical restoration of coronary artery blood flow at cardiac catheterization.(Level of Evidence: C) "

"2. Thrombolytic agents should be administered together with low-dose ASA and low-dose heparin, with careful monitoring for bleeding.(Level of Evidence: C) "

Class IIb

"1. Treatment of coronary artery thrombosis with substantial thrombus burden and high risk of occlusion with a combination of reduced-dose thrombolytic therapy and abciximab may be considered. (Level of Evidence: C) "

Recommendations for Risk Stratification of Coronary Artery Abnormalities

Class IIa

"1. It is reasonable to use echocardiographic coronary artery luminal dimensions converted to BSA-adjusted Z scores to determine risk stratification. (Level of Evidence: B) "

"1. It is reasonable to incorporate both maximal and current coronary artery involvement in risk stratification. (Level of Evidence: C) "

"1. It is reasonable to incorporate the presence of additional features other than coronary artery luminal dimensions into decisions regarding risk stratification. (Level of Evidence: C) "