Approach
The main goal of treatment is to prevent cardiac complications, especially coronary artery aneurysms, by treating the inflammatory process as early as possible, in addition to treating the acute illness and reducing duration of inpatient stay.
Risk factors for complications (coronary aneurysms) include those with severe inflammation (e.g., CRP >100 mg/L), persistent fever despite treatment, or persistently elevated C-reactive protein (CRP).
Presentation ≤10 days from onset or presentation >10 days from onset with evidence of ongoing inflammation
Standard treatment includes administering a single infusion of intravenous immune globulin (IVIG), early in the course of the disease within 10 days of the onset.[43] Treatment should be started as soon as a patient is diagnosed with either complete or incomplete KD.[1][31] IVIG is also indicated in patients who present after 10 days with KD, even if fever has resolved. This is considered to be the most current treatment regimen and has been successful in reducing the duration of fever and the prevalence of coronary artery aneurysms in KD.[44] Delaying treatment may lead to significant coronary artery aneurysms.[37] Live immunizations, such as measles, mumps varicella, should be deferred if the patient has been treated with IVIG due to the risk of suppressing the immune response to the vaccine. For patients at high risk of exposure to measles, the immunization can be given and repeated at least 11 months after IVIG exposure.[1][31]
Aspirin should be used with IVIG therapy and is thought to have an additive anti-inflammatory effect at higher doses, and can help prevent thrombosis via its antiplatelet effect at lower doses.[37] In the US, the anti-inflammatory dose used tends to be higher than that used in Japan and Europe. The dose of aspirin used has not been found to affect the incidence of coronary artery aneurysms.[31] Variation exists among medical centers concerning when aspirin dose should be reduced to antiplatelet levels: either 48-72 hours after fever resolves, or 14 days after the onset of symptoms and when the patient has been afebrile for at least 48-72 hours.[33] The European guidelines recommend that the dose should be reduced 48 hours after fever resolves as long as clinical symptoms are improving and the CRP is falling.[31] Low-dose aspirin should be continued for 6-8 weeks after the acute episode and can then be stopped if the echocardiogram remains normal.[1][31] European guidelines note that patients with regressed aneurysms may have persistent endothelial function abnormalities and therefore ongoing low-dose aspirin should be considered depending on individual risk.[31]
One meta-analysis and systematic review reported that prescribing low-dose or no aspirin was associated with reduced risk of coronary artery aneurysms compared with high-dose aspirin in the acute phase of KD with IVIG treatment, but results need to be validated in a prospective trial.[45]
Two-thirds of patients will be afebrile and improve within 24 hours of completion of the IVIG infusion, and 90% will be afebrile by 48 hours. This therapy regimen is effective in reducing the prevalence of coronary artery abnormalities from 20% to 25% down to 2% to 4%.[44]
Some patients may have persistent or recurrent fever 48 hours after a single dose of IVIG infusion. These patients are at increased risk of developing coronary artery abnormalities and may benefit from a second IVIG infusion. The American College of Rheumatology/Vasculitis Foundation recommends that patients with acute KD and persistent fever after initial treatment with IVIG should receive a second course of IVIG rather than advancing therapy with corticosteroids.[37] However, European guidance recommends consideration of initiating corticosteroids along with the second dose of IVIG in this situation.
One network meta-analysis found that the combination therapy with the standard therapy of IVIG and aspirin might have an additional effect on shortening the duration of fever and lowering the coronary artery aneurysms incidence rate in patients with acute KD. However, the authors caution that new randomized trials would be required to support the results.[46]
Monitoring response to treatment
Resolution of fever should not be relied upon alone as a marker of treatment success as significant systemic inflammation may continue despite fever resolution. A practical approach to monitoring response to treatment has been published in 2022, an approach used by the ongoing Kawasaki Disease Coronary Artery Aneurysm Prevention trial, an open-label trial investigating if initial treatment with corticosteroids alongside IVIG and aspirin reduces incidence of coronary artery aneurysms. The therapeutic target in KD is "zero fever, zero CRP."
In the 48 hours following IVIG, patients should be afebrile and have a normal CRP (<10 mg/L) or CRP should be at least halving every 24 hours (as the half-life of CRP is 18 hours when hepatic production is ceased).[47]
Patients refractory to IVIG
Various definitions for refractory Kawasaki have been postulated. The definition used in international guidelines and clinical trials is failure of resolution of fever within 36-48 hours of initial IVIG. However, it has been proposed that this definition should be broadened to include evidence of ongoing systemic inflammation within 48 hours of initial treatment to detect children with ongoing inflammation who are at risk of developing or worsening cardiac sequelae.[47]
IVIG resistance occurs in 10% to 20% of cases.[48] Patients with IVIG resistance are at a higher risk of developing coronary artery aneurysms.[31][49]
Outside of Japan, clinical scores to predict IVIG resistance perform suboptimally.[50][51][52] Therefore, clinicians elsewhere should assess the risk of IVIG resistance based on symptoms and laboratory values. Factors that increase the risk of IVIG resistance include: age <12 months; low serum sodium, hemoglobin, or albumin; high alanine transaminase, CRP, or bilirubin; and signs of shock or macrophage activation syndrome.[31][51]
A susceptibility gene on chromosome 19 which codes inositol 1,4,5-triphosphate 3-kinase C (ITPKC) has been shown to be significantly associated with IVIG resistance in KD patients and in those with coronary artery lesions.[19][49] It is probable that there are other genetic susceptibility genes which are yet to be identified.
Corticosteroids
In Europe, corticosteroids are given as first-line to those with severe disease (high CRP >100 mg/L, persistently elevated CRP despite treatment, anemia, hypoalbuminemia, liver dysfunction, hemophagocytic lymphohistiocytosis or shock), those with evolving coronary or peripheral aneurysms at presentation with evidence of ongoing inflammation, and as rescue treatment in those with IVIG failure (with either ongoing fever, persistent inflammation or ongoing clinical signs 48 hours after IVIG).[47][31] The European guidelines also recommend that corticosteroids are given to patients with proven IVIG resistance, Kobayashi score of 5 or more (if assessed with this tool), features of shock or macrophage activation syndrome, ages <1 year, or established presence of coronary or peripheral aneurysms with ongoing inflammation.[31]
It has been shown that corticosteroids are beneficial in refractory KD and appear to reduce the risk of heart problems after Kawasaki disease without causing any important adverse effects. One 2022 Cochrane Review supports the use of corticosteroids in the acute phase of KD as it is associated with reduced coronary artery abnormalities, shorter duration of hospital stay and reduced inflammatory markers when compared to those treated without corticosteroids.[53] Literature on the use of oral and intravenous pulse corticosteroid therapy in KD has produced conflicting results due to heterogeneity in the doses, regimens, and patient populations used in the studies.[54][55][56][57][58][59][60][61] Until more data are available, the subset of patients with KD that are IVIG-resistant and/or with life-threatening complications should be treated with corticosteroid therapy.[53][62] [
]
The American College of Rheumatology/Vasculitis Foundation recommends that patients with acute KD who are at high risk of IVIG resistance or developing coronary artery aneurysms are given IVIG with adjunctive corticosteroids as initial therapy rather than IVIG monotherapy, with high-risk defined as a Z score greater than or equal to 2.5 for the left anterior descending coronary artery or right coronary artery at initial scan and ages <6 months, pending further studies to identify at risk patients in the US population.[37]
Further prospective, multicenter, randomized controlled trials are needed to determine the efficacy of pulsed intravenous or oral doses of corticosteroids in the treatment of IVIG-resistant KD, and also for unselected cases of KD.
Tumor necrosis factor (TNF)-alpha antagonists
The TNF-alpha antagonist infliximab has been used in patients refractory to IVIG and corticosteroids.[63] Although there have been reports of IVIG resistant cases being treated with infliximab second-line instead of corticosteroids, the American Heart Association guidelines recommend corticosteroids as second-line ahead of infliximab due to the stronger evidence base, with more evidence to support the use of a corticosteroid when compared with the use of infliximab.[1]
One 2021 multicenter trial compared a second IVIG dose with infliximab in IVIG resistant children. Despite shorter duration of fever in the infliximab group there was no difference between inflammatory markers or rates of coronary artery abnormalities between the groups.[64]
The American College of Rheumatology/Vasculitis Foundation recommends that patients with acute KD who are at high risk of IVIG resistance or developing coronary artery aneurysms are given IVIG with adjunctive immunomodulatory immunosuppressive agents (e.g., infliximab, anakinra, or cyclosporine) as initial therapy rather than IVIG monotherapy, if high risk, if glucocorticoids are contraindicated. However, there is currently stronger evidence for glucocorticoids in comparison with nonglucocorticoid immunomodulating treatment to be used in addition to IVIG in high risk patients.[37]
The European SHARE guidelines also recommend consideration of using TNF-alpha antagonists in patients with persistent inflammation despite IVIG, aspirin, and corticosteroids.[31]
One Cochrane review looked at TNF-alpha antagonists used after IVIG or as initial treatment in five trials including 494 children. Four of the trials used infliximab and one used etanercept. The authors found low-certainty evidence that TNF-alpha antagonists reduced treatment resistance and were associated with fewer infusion reactions compared with no treatment or additional IVIG. No differences were found in the incidence of coronary artery abnormalities or infections between groups.[65]
In one meta-analysis of eight studies of infliximab therapy used after IVIG or as initial treatment, infliximab significantly reduced treatment resistance when used as initial treatment compared to IVIG alone, and improved clinical course when used in IVIG resistant patients compared to repeat IVIG.[66]
One network meta-analysis comparing different combinations of pharmacologic interventions for Kawasaki disease found that the combination of medium dose IVIG, aspirin, and infliximab resulted in the shortest duration of fever and lowest incidence of coronary artery abnormalities in initial stage of KD. In the refractory phase, IVIG and pulsed-dose corticosteroids and high-dose IVIG and cyclosporine were the combinations associated with the most rapid resolution of fever and lowest incidence of coronary artery abnormalities.[46] Heterogeneity of underlying studies may have introduced bias in these results.
Two meta-analyses compared IVIG, methylprednisolone, and infliximab in patients with refractory KD. They included many of the same studies but came to different conclusions. One study reported that infliximab and methylprednisolone were significantly more effective in resolving fever than a second dose of IVIG.[67] The other study reported that infliximab was significantly more effective in resolving fever than both methylprednisolone and a second dose of IVIG.[68] Neither study found any differences in the rates of coronary artery lesions between treatment groups.
One systematic review and meta-analysis found that although monoclonal antibodies and anticytokine biologics were not effective in reducing the frequency of coronary artery abnormalities in KD patients, the frequency of IVIG resistance may be reduced compared with conventional monotherapy with IVIG.[69]
Other immunomodulatory drugs or plasma exchange
Patients with refractory KD in whom a second dose of IVIG, corticosteroids, and infliximab have failed may receive an alternative immunomodulatory drug. There is no consensus or evidence as to whether cyclosporine, anakinra, or cyclophosphamide should be used after other treatments fail and cases should be discussed with a specialist center.
Very rarely, plasma exchange may be considered in refractory KD patients who have failed to respond to all of the above regimens.[1] A retrospective study from Japan assessed the benefit of adding plasma exchange rescue (PER) to KD patients who were IVIG and infliximab nonresponders. The study showed that the addition of PER resulted in improvement of fever, other acute symptoms, laboratory data, and coronary outcomes. These results remain uncertain pending future randomized trials.[70]
One network meta-analysis found that the combination therapy with high-dose IVIG and corticosteroids or cyclosporine may have an additional effect on improving the rate of decline of fever and lowering the incidence rate of coronary artery abnormalities in children with refractory KD. However, the authors caution that new randomized trials would be required to support the results.[46]
There has been abundant evidence from human patients, genetic studies, and experimental mouse models that support the involvement of interleukin-1b (IL-1b) in the pathogenesis of KD. Anakinra is a competitive inhibitor to both IL-1a and IL-1b and acts by blocking the IL-1 binding to the IL-1 receptor. The Kawakinra study, a phase 2 clinical trial, aimed to determine the efficacy, safety and tolerability of blocking IL-1 signalling in patients with acute KD who are unresponsive to treatment with IVIG. While this study supported the early use of anakinra in cases refractory to IVIG, the effect on the KD symptoms and inflammation parameters took an average of 14 days to achieve. Ultimately, the study lays down the groundwork for controlled clinical trials to fully further evaluate the efficacy of anakinra as a first line treatment for KD.[71]
Presentation >10 days from onset without evidence of ongoing inflammation
Patients with KD that present after day 10 without persistent fever, and when their acute phase markers (ESR and/or CRP) are normal, are regarded as without risk of developing coronary aneurysms.
If their initial and subsequent echocardiograms are normal, they should be treated with low-dose aspirin until 6-8 weeks from the onset. If echocardiogram at 8 weeks is normal, low-dose aspirin can be discontinued.
However, if diagnosed after day 10 with evidence of elevated ESR or CRP and/or coronary abnormalities on echocardiogram, patients should be treated as detailed above for patients with evidence of ongoing inflammation.
Long-term management
All patients with current or previous aneurysms following Kawasaki disease require lifelong follow-up by a cardiology team within a specialist Kawasaki disease service. Their risk of future cardiac events is proportional to the degree of cardiac pathology remaining after the acute illness has resolved.[12] European recommendations suggest that ECG and echocardiography should be performed every 3-6 months in children with coronary artery aneurysms, depending on their severity.[31]
The American Heart Association guidelines include a stratification system based on maximum Z-score (ever measured) and current Z-score (during long-term follow up) to categorize patients by their risk level for development of myocardial ischemia.[1] Many of the concepts in the recommendations were similar, but differed in timing. For brevity, any specific differences are noted under each risk stratum separately below, but the general concepts are:
Promote healthy lifestyle and activity at every visit.
If a patient cannot tolerate or is resistant to aspirin, use an alternative antiplatelet agent such as clopidogrel. Dipyridamole is no longer used for thromboprophylaxis.[1]
Cardiovascular risk factor assessment includes blood pressure, fasting lipid profile, body mass index, waist circumference, diet, activity, and smoking.
Restrict high-contact activities in patients on anticoagulation or dual antiplatelet therapy. Participation in competitive sports or high-intensity activities should be guided by results from testing for inducible myocardial ischemia or exercise-induced arrhythmias.
Follow-up cardiology assessment should include history and physical exam, echocardiography, and electrocardiography.
Additional cardiology assessment includes stress echocardiography, stress with magnetic resonance imaging, stress nuclear medicine, or positron emission tomography to detect inducible myocardial ischemia.
Further imaging includes angiography by computed tomography, magnetic resonance imaging, or invasive angiography.
Discourage oral contraceptive use in patients who have ever had medium aneurysms, or larger (Z score ≥5), and who still have small aneurysms (Z score ≥2.5) or larger. Advise that pregnancy be supervised by a multidisciplinary team including a cardiologist, and that anticoagulants or antiplatelet therapy may require adjustment during pregnancy or delivery.
The use of statin therapy is not yet widespread. For further information, please see the Emerging treatments section.
No involvement (Z score always <2)
Give low-dose aspirin after the acute episode, but discontinue aspirin after 4-6 weeks. Discharge patients from cardiology follow-up at 4-6 weeks after disease onset, although follow-up may be continued for up to 12 months.
Assess cardiovascular risks at least once and ideally at least 1 year from the episode of acute disease.
Counsel patients about contraception and pregnancy as you would patients without KD.
Dilation only (Z scores ≥2.0 but <2.5, or a decrease in Z score during follow-up ≥1)
Give low-dose aspirin, but discontinue it 4-6 weeks after the episode of acute disease. Discharge from cardiology if luminal dimensions have returned to normal by 4-6 weeks after disease onset, follow-up may be continued for 12 months. If dilation persists at 4-6 weeks after disease onset, continue follow-up for 12 months. Dilation usually resolves within 1 year but if dilation persists, consider whether this represents a dominant branch. If it is a dominant branch, you may discharge the patient, but may also wish to follow up the patient every 2-5 years.
Assess cardiovascular risks at least once and ideally at least 1 year from the episode of acute disease.
Advise patients about contraception and pregnancy as you would advise patients without KD.
Small aneurysms (Z score ≥2.5 to <5.0)
Current or persistent small aneurysms
Treat these patients with low-dose aspirin. Follow up at 4-6 weeks after the acute disease episode, assess after 6 months and 1 year, and follow up every year thereafter.
Assess cardiovascular risks at least once and ideally at least 1 year from the episode of acute disease. Obtain a follow-up fasting lipid profile.
Assess for inducible myocardial ischemia every 2-3 years, if the patient has symptoms suggestive of ischemia or has signs suggestive of ventricular dysfunction. Consider further imaging with angiography every 3-5 years.
Advise patients about contraception and pregnancy as you would patients without KD.
Regression to normal Z score or dilation only
Consider treatment with low-dose aspirin, although it is reasonable to discontinue aspirin after coronary arteries regress to normal. Assess these patients every 1-3 years. There is no need to perform echocardiography unless there is evidence for inducible myocardial ischemia, the patient has symptoms suggestive of ischemia, or the patient has signs suggestive of ventricular dysfunction.
Assess cardiovascular risks every 2 years. Obtain a follow-up fasting lipid profile.
Assess for inducible myocardial ischemia every 3-5 years, if the patient has symptoms suggestive of ischemia or has signs suggestive of ventricular dysfunction. Consider further imaging with angiography only if there is evidence for inducible myocardial ischemia or ventricular dysfunction.
Advise patients about contraception and pregnancy as you would patients without KD.
Medium aneurysms (Z score ≥5 to <10, with an absolute luminal dimension <8 mm)
Current or persistent medium aneurysms
Treat with low-dose aspirin. Consider dual antiplatelet therapy with an additional antiplatelet agent.
Patients should be reviewed at 4-6 weeks after the acute disease episode, then assess after 3 months, 6 months, and 1 year, and follow up every 6-12 months thereafter.
Assess cardiovascular risks at least once and ideally at least 1 year from the episode of acute disease. Obtain a follow-up fasting lipid profile.
Assess for inducible myocardial ischemia every 1-3 years, or if the patient has symptoms suggestive of ischemia or signs suggestive of ventricular dysfunction. Consider further imaging with angiography every 2-5 years.
Advise that participation in competitive sports or high-intensity activities should be guided by results from testing for inducible myocardial ischemia or exercise-induced arrhythmias. Restrict activities involving a risk of bodily contact, trauma, or injury for patients taking dual antiplatelet therapy.
Discourage patients from using oral contraceptives that increase thrombosis risk, and recommend that pregnancy is supervised by a multidisciplinary team including a cardiologist. It may be necessary to alter thromboprophylaxis during pregnancy and delivery.
Regression to small aneurysms
Treat with low-dose aspirin. Consider dual antiplatelet therapy with an additional antiplatelet agent. Follow up patients every year.
Assess cardiovascular risks every year. Obtain a follow-up fasting lipid profile.
Assess for inducible myocardial ischemia every 2-3 years, if the patient has symptoms suggestive of ischemia or has signs suggestive of ventricular dysfunction. Consider further imaging with angiography every 3-5 years.
Advise that participation in competitive sports or high-intensity activities should be guided by results from testing for inducible myocardial ischemia or exercise-induced arrhythmias. Restrict activities involving a risk of bodily contact, trauma, or injury for patients taking dual antiplatelet therapy.
Discourage patients from using oral contraceptives that increase thrombosis risk, and recommend that pregnancy is supervised by a multidisciplinary team including a cardiologist. It may be necessary to alter thromboprophylaxis during pregnancy and delivery.
Regression to normal Z score or dilation only
Treat with low-dose aspirin. Do not use an additional antiplatelet agent unless there is evidence of inducible myocardial ischemia. Follow up every 1-2 years. Consider not performing routine 2D echocardiography unless there is evidence of inducible myocardial ischemia, the patient has symptoms suggestive of ischemia, or the patient has signs suggestive of ventricular dysfunction.
Assess cardiovascular risk every 2 years. Obtain a follow-up fasting lipid profile.
Assess for inducible myocardial ischemia every 2-4 years, if the patient has symptoms suggestive of ischemia or has signs suggestive of ventricular dysfunction. Further imaging with angiography may not be needed in the absence of evidence of inducible myocardial ischemia.
Advise that participation in competitive sports or high-intensity activities should be guided by results from testing for inducible myocardial ischemia or exercise-induced arrhythmias.
No restrictions regarding contraception and pregnancy are applicable to this group of patients.
Large and giant aneurysms (Z score ≥10 or absolute dimension ≥8 mm)
Current or persistent large and giant aneurysms
Treat with low-dose aspirin. Use warfarin to achieve a target international normalized ratio of 2-3, or a low molecular weight heparin (LMWH) to achieve anti-factor Xa levels of 0.5 to 1.0 units/mL. Consider dual antiplatelet therapy with aspirin and an additional antiplatelet agent together with warfarin or LMWH for thromboprophylaxis in the setting of very extensive or distal coronary artery aneurysms, or if there is a history of coronary artery thrombosis. Consider treatment with a beta-blocker. Assess patients at 1, 2, 3, 6, 9, and 12 months after the episode of acute disease in the first year and every 3-6 months thereafter.
Assess cardiovascular risks every 6-12 months. Obtain a follow-up fasting lipid profile.
Assess for inducible myocardial ischemia every 6-12 months, if the patient has symptoms suggestive of ischemia or has signs suggestive of ventricular dysfunction. Consider further imaging with angiography for diagnostic and prognostic purposes during the first year and every 1-5 years thereafter.
Advise that participation in competitive sports or high-intensity activities should be guided by results from testing for inducible myocardial ischemia or exercise-induced arrhythmias. Restrict or modify activities involving a risk of bodily contact, trauma, or injury for patients taking dual antiplatelet or anticoagulation therapy.
Discourage oral contraceptives that increase thrombosis risk, and recommend that pregnancy is supervised by a multidisciplinary team including a cardiologist. Advise that thromboprophylaxis during pregnancy and delivery may be altered.
Regression to medium aneurysms
Treat with low-dose aspirin. Discontinue anticoagulation (warfarin or LMWH) and substitute with an additional antiplatelet agent. Consider treatment with a beta-blocker. Assess the patient every 6-12 months.
Assess cardiovascular risks every 12 months. Obtain a follow-up fasting lipid profile.
Assess for inducible myocardial ischemia every 12 months or if the patient has symptoms suggestive of ischemia or signs suggestive of ventricular dysfunction. Consider further imaging with angiography every 2-5 years.
Advise that participation in competitive sports or high-intensity activities should be guided by results from testing for inducible myocardial ischemia or exercise-induced arrhythmias. Restrict or modify activities involving a risk of bodily contact, trauma, or injury for patients taking dual antiplatelet or anticoagulation therapy.
Discourage using oral contraceptives that increase thrombosis risk, and recommend that pregnancy is supervised by a multidisciplinary team including a cardiologist. Advise that thromboprophylaxis management during pregnancy and delivery may be altered.
Regression to small aneurysms
Treat with low-dose aspirin. Consider treatment with a beta-blocker. Assess the patient every 6-12 months.
Assess cardiovascular risks every 12 months. Obtain a follow-up fasting lipid profile.
Assess for inducible myocardial ischemia every 1-2 years, if the patient has symptoms suggestive of ischemia or has signs suggestive of ventricular dysfunction. Consider further imaging with angiography every 2-5 years.
Advise that participation in competitive sports or high-intensity activities should be guided by results from testing for inducible myocardial ischemia or exercise-induced arrhythmias. Restrict or modify activities involving a risk of bodily contact, trauma, or injury for patients taking dual antiplatelet or anticoagulation therapy.
Advise patients as per normal about contraception, but recommend that pregnancy is supervised by a multidisciplinary team including a cardiologist, and that thromboprophylaxis may be altered during pregnancy and delivery.
Regression to normal Z score or dilation only
Treat with low-dose aspirin. Follow up every 1-2 years. Consider not performing routine 2D echocardiography unless there is evidence of inducible myocardial ischemia, the patient has symptoms suggestive of ischemia, or the patient has signs suggestive of ventricular dysfunction.
Assess cardiovascular risks every 2 years. Obtain a follow-up fasting lipid profile.
Assess for inducible myocardial ischemia every 2-5 years, if the patient has symptoms suggestive of ischemia or signs suggestive of ventricular dysfunction. Further imaging with angiography may not be needed in the absence of evidence of inducible myocardial ischemia.
Advise that participation in competitive sports or high-intensity activities should be guided by results from testing for inducible myocardial ischemia or exercise-induced arrhythmias. Restrict or modify activities involving a risk of bodily contact, trauma, or injury for patients taking dual antiplatelet or anticoagulation therapy.
Advise patients about contraception as per normal, but recommend that pregnancy is supervised by a multidisciplinary team including a cardiologist, and that thromboprophylaxis may be altered during pregnancy and delivery.
Similarly the SHARE guidelines recommend a repeat echocardiogram:[31]
In patients in whom the initial echocardiogram was normal and the systemic inflammatory process has improved at 2 weeks after initial IVIG
In all patients with KD at 6-8 weeks
In those with ongoing active inflammation (symptoms or signs of KD and/or raised inflammatory markers) at least weekly to monitor for cardiac complications
In those with abnormalities on initial echocardiogram at least weekly to ensure stabilization
In those with coronary artery aneurysm 3-6 monthly.
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