Treatment algorithm

Please note that formulations/routes and doses may differ between drug names and brands, drug formularies, or locations. Treatment recommendations are specific to patient groups: see disclaimer

ACUTE

presentation ≤10 days from onset; or presentation >10 days from onset with evidence of ongoing inflammation

1st line – intravenous immunoglobulin (IVIG)

Back
ACUTE
|
presentation ≤10 days from onset; or presentation >10 days from onset with evidence of ongoing inflammation
1st line – 

intravenous immunoglobulin (IVIG)

The main goal of treatment is to prevent coronary artery disease and to relieve symptoms by controlling the inflammatory process. This is monitored by the defervescence and the resolution of all acute symptoms.

IVIG as a single infusion is the standard treatment and comprises the mainstay of therapy, and has been successful in reducing the duration of fever and the prevalence of coronary artery aneurysms in KD.[37][43]

Treatment should be started as soon as a patient is diagnosed with either complete or incomplete KD.[1][31] The best results are achieved when treatment is instituted within 10 days or even within 7 days.

It is also indicated in patients who present after 10 days with KD, even if fever has resolved.

Some patients fail to defervesce within 36-48 hours. These patients are at increased risk of developing coronary artery abnormalities and should be given a second dose of IVIG.

Z scores define coronary artery abnormalities, but they do not inform acute phase management. However, risk stratification for long-term management takes into account both past (maximal) and current involvement.

Live immunisations, 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 immunisation can be given and repeated at least 11 months after IVIG exposure.[1][31]

Primary options

normal immunoglobulin human: 2 g/kg intravenously as a single dose, may repeat dose 36-48 hours later if patient fails to defervesce

Plus – high-dose aspirin

Back
ACUTE
|
presentation ≤10 days from onset; or presentation >10 days from onset with evidence of ongoing inflammation
Plus – 

high-dose aspirin

Treatment recommended for ALL patients in selected patient group

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 in KD.[37]

Aspirin appears to have no effect on the incidence or the development of coronary artery aneurysms, whether treatment is initiated before or after 5 days of therapy.

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]

Primary options

aspirin: 80-100 mg/kg/day orally given in 4 divided doses for 24-72 hours after fever cessation (up to 14 days), followed by 3-5 mg/kg once daily for 6-8 weeks

More

Consider – corticosteroid

Back
ACUTE
|
presentation ≤10 days from onset; or presentation >10 days from onset with evidence of ongoing inflammation
Consider – 

corticosteroid

Additional treatment recommended for SOME patients in selected patient group

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 intravenous immunoglobulin (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.[46] IVIG resistance occurs in 10% to 20% of cases.[47] Patients with IVIG resistance are at a higher risk of developing coronary artery aneurysms.[31][48]

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 ≥2.5 for the left anterior descending coronary artery or right coronary artery at initial scan and aged <6 months, pending further studies to identify at risk patients in the US population.[37]

In Europe, corticosteroids are given as first-line to those with severe disease (high CRP >100 mg/L, persistently elevated CRP despite treatment, anaemia, hypoalbuminaemia, liver dysfunction, haemophagocytic 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).[31][46] 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, aged <1 year, or established presence of coronary or peripheral aneurysms with ongoing inflammation.[31]

Corticosteroids are beneficial in refractory KD and appear to reduce the risk of heart problems after Kawasaki disease without causing any important side 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.[52]

Until more data are available, the subset of patients with KD that are IVIG-resistant and/or with life-threatening complications should be given the option of corticosteroid therapy.[52][61][71] [ Cochrane Clinical Answers logo ]

The literature on the use of oral versus intravenous corticosteroid therapy in KD has produced conflicting results due to heterogeneity in the doses, regimens, and patient populations used in the studies.[53][54][55][56][57][58][59][60]

Doses vary across studies and a specialist should be consulted on the appropriate dose.

Primary options

methylprednisolone: consult specialist for guidance on dose

OR

prednisolone: consult specialist for guidance on dose

2nd line – infliximab

Back
ACUTE
|
presentation ≤10 days from onset; or presentation >10 days from onset with evidence of ongoing inflammation
2nd line – 

infliximab

Infliximab is a chimeric monoclonal antibody to tumour necrosis factor (TNF)-alpha. Efficacy in other inflammatory conditions, including vasculitis, supports its use in KD.

It has been used in patients refractory to intravenous immunoglobulin (IVIG) and methylprednisolone.[62] These patients comprise an extremely small group of less than 1% of the patients with KD.

A number of clinicians use infliximab as a second-line option before a corticosteroid in patients with IVIG resistance. However, guidelines cite more evidence to support the use of a corticosteroid when compared with the use of infliximab.[1]

A multicentre, randomised, prospective trial of infliximab compared with a second IVIG infusion in 24 children with acute KD and IVIG resistance showed both treatments to be safe and well-tolerated. The study concluded that optimal management of patients resistant to IVIG remains to be determined.[72]

The European SHARE guidelines also recommend consideration of using a TNF-alpha inhibitor in patients with persistent inflammation despite IVIG, aspirin, and corticosteroids.[31]

Primary options

infliximab: 5 mg/kg intravenously as a single dose

Plus – high-dose aspirin

Back
ACUTE
|
presentation ≤10 days from onset; or presentation >10 days from onset with evidence of ongoing inflammation
Plus – 

high-dose aspirin

Treatment recommended for ALL patients in selected patient group

Aspirin is thought to have an additive anti-inflammatory effect at higher doses, and can help prevent thrombosis via its antiplatelet effect at lower doses in KD.[37] In the US, the anti-inflammatory dose used tends to be higher than that used in Japan and Europe.

Aspirin appears to have no effect on the incidence or the development of coronary artery aneurysms, whether treatment is initiated before or after 5 days of therapy.

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]

Primary options

aspirin: 80-100 mg/kg/day orally given in 4 divided doses for 24-72 hours after fever cessation (up to 14 days), followed by 3-5 mg/kg once daily for 6-8 weeks

More

3rd line – other immunomodulatory drug or plasma exchange

Back
ACUTE
|
presentation ≤10 days from onset; or presentation >10 days from onset with evidence of ongoing inflammation
3rd line – 

other immunomodulatory drug or plasma exchange

There is no consensus or evidence as to whether ciclosporin, anakinra, or cyclophosphamide should be used after other treatments fail and cases should be discussed with a specialist centre.

One network meta-analysis found that the combination therapy with high-dose IVIG and corticosteroids or ciclosporin 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 randomised trials would be required to support the results.[45]

Very rarely, plasma exchange may be considered.[1] One retrospective study from Japan assessed the benefit of adding plasma exchange rescue (PER) to KD patients who were IVIG and infliximab non-responders. 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 randomised trials.[73]

Primary options

ciclosporin: consult specialist for guidance on dose

Secondary options

anakinra: consult specialist for guidance on dose

OR

cyclophosphamide: consult specialist for guidance on dose

Plus – high-dose aspirin

Back
ACUTE
|
presentation ≤10 days from onset; or presentation >10 days from onset with evidence of ongoing inflammation
Plus – 

high-dose aspirin

Treatment recommended for ALL patients in selected patient group

Aspirin is thought to have an additive anti-inflammatory effect at higher doses, and can help prevent thrombosis via its antiplatelet effect at lower doses in KD.[37] In the US, the anti-inflammatory dose used tends to be higher than that used in Japan and Europe .

Aspirin appears to have no effect on the incidence or the development of coronary artery aneurysms, whether treatment is initiated before or after 5 days of therapy.

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]

Primary options

aspirin: 80-100 mg/kg/day orally given in 4 divided doses for 24-72 hours after fever cessation (up to 14 days), followed by 3-5 mg/kg once daily for 6-8 weeks

More

presentation >10 days from onset without evidence of ongoing inflammation

1st line – low-dose aspirin

Back
ACUTE
|
presentation >10 days from onset without evidence of ongoing inflammation
1st line – 

low-dose aspirin

Patients with KD who present after day 10 without persistent fever, and with normal acute-phase markers (erythrocyte sedimentation rate and/or C-reactive protein) and normal initial echocardiogram, are regarded as without risk of developing coronary aneurysms.

These patients should be treated with low-dose aspirin until 6-8 weeks. Further continuation of aspirin beyond 8 weeks depends on the long-term risk of myocardial ischaemia: low, moderate, or high risk.

Z scores define coronary artery abnormalities, but they do not inform acute phase management. However, risk stratification for long-term management takes into account both past (maximal) and current involvement.

Seek expert advice if in doubt about ongoing inflammation.

Primary options

aspirin: 3-5 mg/kg/day orally for 6-8 weeks

ONGOING

after initial episode: Z score always <2; no involvement at any time

1st line – discontinue low-dose aspirin and provide follow-up + advice

Back
ONGOING
|
after initial episode: Z score always <2; no involvement at any time
1st line – 

discontinue low-dose aspirin and provide follow-up + advice

Risk stratification for long-term management is based primarily on maximal coronary artery Z scores, and takes into account both past and current involvement.

Promote healthy lifestyle and activity at every visit.

Discontinue aspirin 4-6 weeks after the acute episode. These patients do not need antiplatelet therapy (low-dose aspirin) beyond the recommended 6-8 weeks after onset.

Carefully assess cardiovascular risk (blood pressure, fasting lipid profile, BMI, waist circumference, dietary and activity assessment, and smoking) and counsel at least once and preferably after 1 year after the acute illness.

Further follow-up is not necessary.

Angiography is not necessary.

No restriction of physical activity beyond 8 weeks is necessary.

Standard contraception and pregnancy counselling are appropriate.[1]

after initial episode: Z score ≥2.0 to <2.5; dilation only

1st line – discontinue low-dose aspirin and provide follow-up + advice

Back
ONGOING
|
after initial episode: Z score ≥2.0 to <2.5; dilation only
1st line – 

discontinue low-dose aspirin and provide follow-up + advice

Risk stratification for long-term management is based primarily on maximal coronary artery Z scores, and takes into account both past and current involvement.

Promote healthy lifestyle and activity at every visit.

Discontinue aspirin at 4-6 weeks after the acute episode.

Discharge from follow-up if luminal dimensions have returned to normal by 4-6 weeks, but continue follow-up for 12 months if dilation persists. Dilation usually resolves within 1 year, but if dilation persists, consider whether this represents a dominant branch, in which case you may wish to follow up the patient every 2-5 years.

Assess cardiovascular risks (blood pressure, fasting lipid profile, BMI, waist circumference, dietary and activity assessment, and smoking) at least once and ideally at least 1 year from the acute episode.[1]

Angiography is not necessary. No restriction of physical activity beyond 8 weeks is necessary.

Standard contraception and pregnancy counselling are appropriate.[1]

after initial episode: Z score ≥2.5 to <5.0; small aneurysm

1st line – antiplatelet agent

Back
ONGOING
|
after initial episode: Z score ≥2.5 to <5.0; small aneurysm
|
current or persistent
1st line – 

antiplatelet agent

Risk stratification for long-term management is based primarily on maximal coronary artery Z scores, and takes into account both past and current involvement.

Offer low-dose aspirin to all children with coronary artery dilation or aneurysm formation that is greater than mild (coronary artery Z score >2.5 to 5.0), at least until aneurysm regression is demonstrated.[74]

If patients cannot tolerate or are resistant to aspirin, clopidogrel is an alternative.

Primary options

aspirin: 3-5 mg/kg/day orally

Secondary options

clopidogrel: consult specialist for guidance on dose

Plus – follow-up + advice

Back
ONGOING
|
after initial episode: Z score ≥2.5 to <5.0; small aneurysm
|
current or persistent
Plus – 

follow-up + advice

Treatment recommended for ALL patients in selected patient group

Promote healthy lifestyle and activity at every visit.

Follow up at 4-6 weeks after the acute episode, after 6 months, 12 months and then annually thereafter, with ECG and echocardiogram.

Assess cardiovascular risks (blood pressure, fasting lipid profile, BMI, waist circumference, dietary and activity assessment, and smoking) at least once and ideally at least 1 year from the acute episode. Obtain a fasting lipid profile.

Assess for inducible myocardial ischaemia (stress echocardiography, stress with MRI, stress nuclear medicine, or PET) every 2-3 years, if the patient has symptoms suggestive of ischaemia or has signs suggestive of ventricular dysfunction.

Consider further imaging with angiography, if ischaemia is present, every 3-5 years.

No restriction of physical activity beyond 8 weeks is necessary.

Standard contraception and pregnancy counselling are appropriate.[1]

1st line – follow-up + advice

Back
ONGOING
|
after initial episode: Z score ≥2.5 to <5.0; small aneurysm
|
regression to normal Z score or dilation only
1st line – 

follow-up + advice

Risk stratification for long-term management is based primarily on maximal coronary artery Z scores, and takes into account both past and current involvement.

Promote healthy lifestyle and activity at every visit.

Assess every 1-3 years.

Assess cardiovascular risk (blood pressure, fasting lipid profile, BMI, waist circumference, dietary and activity assessment, and smoking) every 2 years. Obtain a follow-up fasting lipid profile.

There is no need to perform echocardiography unless there is evidence for inducible myocardial ischaemia, the patient has symptoms suggestive of ischaemia, or the patient has signs suggestive of ventricular dysfunction.

Assess for inducible myocardial ischaemia (stress echocardiography, stress with MRI, stress nuclear medicine, or PET) every 3-5 years. Consider further imaging with angiography only if there is evidence for inducible myocardial ischaemia or ventricular dysfunction.

Advise patients about contraception and pregnancy as you would patients without KD.

Consider – antiplatelet agent

Back
ONGOING
|
after initial episode: Z score ≥2.5 to <5.0; small aneurysm
|
regression to normal Z score or dilation only
Consider – 

antiplatelet agent

Additional treatment recommended for SOME patients in selected patient group

Consider treating with low-dose aspirin, although it is reasonable to discontinue aspirin after coronary arteries regress to normal.

If the patient cannot tolerate or is resistant to aspirin, clopidogrel is an alternative.

Primary options

aspirin: 3-5 mg/kg/day orally

Secondary options

clopidogrel: consult specialist for guidance on dose

after initial episode: Z score ≥5 to <10 (with absolute luminal dimension <8 mm); medium aneurysm

1st line – antiplatelet agent

Back
ONGOING
|
after initial episode: Z score ≥5 to <10 (with absolute luminal dimension <8 mm); medium aneurysm
|
current or persistent
1st line – 

antiplatelet agent

Risk stratification for long-term management is based primarily on maximal coronary artery Z scores, and takes into account both past and current involvement.

Treat with low-dose aspirin. Treatment should continue at least until aneurysm regression is demonstrated.

If the patient cannot tolerate or is resistant to aspirin, clopidogrel is an alternative.

Primary options

aspirin: 3-5 mg/kg/day orally

Secondary options

clopidogrel: consult specialist for guidance on dose

Plus – follow-up + advice

Back
ONGOING
|
after initial episode: Z score ≥5 to <10 (with absolute luminal dimension <8 mm); medium aneurysm
|
current or persistent
Plus – 

follow-up + advice

Treatment recommended for ALL patients in selected patient group

Promote healthy lifestyle and activity at every visit.

See patients at 4-6 weeks after the acute disease episode, then assess after 3 months, 6 months, and 12 months, and follow up every 6-12 months thereafter.

Assess cardiovascular risks (blood pressure, fasting lipid profile, BMI, waist circumference, dietary and activity assessment, and smoking) 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 ischaemia (stress echocardiography, stress with MRI, stress nuclear medicine, or PET) every 1-3 years, or if the patient has symptoms suggestive of ischaemia 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 ischaemia 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.

Consider – additional antiplatelet agent

Back
ONGOING
|
after initial episode: Z score ≥5 to <10 (with absolute luminal dimension <8 mm); medium aneurysm
|
current or persistent
Consider – 

additional antiplatelet agent

Additional treatment recommended for SOME patients in selected patient group

Consider dual antiplatelet therapy with an additional antiplatelet agent such as clopidogrel (if the patient is not already on it). Treatment should continue at least until aneurysm regression is demonstrated.

If the patient is not able to tolerate or is resistant to aspirin, and is therefore already on clopidogrel, consider adding dipyridamole as an alternative. Dipyridamole is no longer generally used for long-term thromboprophylaxis. It is an alternative for aspirin in patients who are taking ibuprofen, resistant or allergic to aspirin, or at risk of Reye syndrome.[1]

Primary options

clopidogrel: consult specialist for guidance on dose

Secondary options

dipyridamole: consult specialist for guidance on dose

1st line – antiplatelet agent

Back
ONGOING
|
after initial episode: Z score ≥5 to <10 (with absolute luminal dimension <8 mm); medium aneurysm
|
regression to small aneurysm
1st line – 

antiplatelet agent

Risk stratification for long-term management is based primarily on maximal coronary artery Z scores, and takes into account both past and current involvement.

Treat with low-dose aspirin. Treatment should continue at least until aneurysm regression is demonstrated.

If the patient cannot tolerate or is resistant to aspirin, clopidogrel is an alternative.

Primary options

aspirin: 3-5 mg/kg/day orally

Secondary options

clopidogrel: consult specialist for guidance on dose

Plus – follow-up + advice

Back
ONGOING
|
after initial episode: Z score ≥5 to <10 (with absolute luminal dimension <8 mm); medium aneurysm
|
regression to small aneurysm
Plus – 

follow-up + advice

Treatment recommended for ALL patients in selected patient group

Promote healthy lifestyle and activity at every visit.

Follow up patients every year.

Assess cardiovascular risk (blood pressure, fasting lipid profile, BMI, waist circumference, dietary and activity assessment, and smoking) every year. Obtain a follow-up fasting lipid profile.

Assess for inducible myocardial ischaemia (stress echocardiography, stress with MRI, stress nuclear medicine, or PET) every 2-3 years, if the patient has symptoms suggestive of ischaemia 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 ischaemia 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.

Consider – additional antiplatelet agent

Back
ONGOING
|
after initial episode: Z score ≥5 to <10 (with absolute luminal dimension <8 mm); medium aneurysm
|
regression to small aneurysm
Consider – 

additional antiplatelet agent

Additional treatment recommended for SOME patients in selected patient group

Consider dual antiplatelet therapy with an additional antiplatelet agent such as clopidogrel (if the patient is not already on it). Treatment should continue at least until aneurysm regression is demonstrated.

If the patient is not able to tolerate or is resistant to aspirin, and is therefore already on clopidogrel, consider adding dipyridamole as an alternative. Dipyridamole is no longer generally used for long-term thromboprophylaxis. It is an alternative for aspirin in patients who are taking ibuprofen, resistant or allergic to aspirin, or at risk of Reye syndrome.[1]

Primary options

clopidogrel: consult specialist for guidance on dose

Secondary options

dipyridamole: consult specialist for guidance on dose

1st line – antiplatelet agent

Back
ONGOING
|
after initial episode: Z score ≥5 to <10 (with absolute luminal dimension <8 mm); medium aneurysm
|
regression to normal Z score or dilation only
1st line – 

antiplatelet agent

Risk stratification for long-term management is based primarily on maximal coronary artery Z scores, and takes into account both past and current involvement.

Treat with low-dose aspirin. Treatment should continue at least until aneurysm regression is demonstrated.

If the patient cannot tolerate or is resistant to aspirin, clopidogrel is an alternative.

Primary options

aspirin: 3-5 mg/kg/day orally

Secondary options

clopidogrel: consult specialist for guidance on dose

Plus – follow-up + advice

Back
ONGOING
|
after initial episode: Z score ≥5 to <10 (with absolute luminal dimension <8 mm); medium aneurysm
|
regression to normal Z score or dilation only
Plus – 

follow-up + advice

Treatment recommended for ALL patients in selected patient group

Promote healthy lifestyle and activity at every visit.

Follow up every 1-2 years. Consider not performing routine 2D echocardiography unless there is evidence of inducible myocardial ischaemia, the patient has symptoms suggestive of ischaemia, or the patient has signs suggestive of ventricular dysfunction.

Assess cardiovascular risk (blood pressure, fasting lipid profile, BMI, waist circumference, dietary and activity assessment, and smoking) every 2 years. Obtain a follow-up fasting lipid profile.

Assess for inducible myocardial ischaemia (stress echocardiography, stress with MRI, stress nuclear medicine, or PET) every 2-4 years, if the patient has symptoms suggestive of ischaemia or has signs suggestive of ventricular dysfunction.

Further imaging with angiography may not be needed in the absence of evidence of inducible myocardial ischaemia.

Advise that participation in competitive sports or high-intensity activities should be guided by results from testing for inducible myocardial ischaemia or exercise-induced arrhythmias.

No restrictions regarding contraception and pregnancy are applicable to this group of patients.

Consider – additional antiplatelet agent

Back
ONGOING
|
after initial episode: Z score ≥5 to <10 (with absolute luminal dimension <8 mm); medium aneurysm
|
regression to normal Z score or dilation only
Consider – 

additional antiplatelet agent

Additional treatment recommended for SOME patients in selected patient group

Do not use an additional antiplatelet agent unless there is evidence of inducible myocardial ischaemia.

Primary options

clopidogrel: consult specialist for guidance on dose

Secondary options

dipyridamole: consult specialist for guidance on dose

after initial episode: Z score ≥10 or absolute luminal diameter ≥8 mm; large or giant aneurysm

1st line – antiplatelet agent

Back
ONGOING
|
after initial episode: Z score ≥10 or absolute luminal diameter ≥8 mm; large or giant aneurysm
|
current or persistent
1st line – 

antiplatelet agent

Risk stratification for long-term management is based primarily on maximal coronary artery Z scores, and takes into account both past and current involvement.

Patients with evidence of large or giant aneurysms, or of coronary obstruction, are at high risk for developing myocardial ischaemia.

Treat with low-dose aspirin. Treatment should continue at least until aneurysm regression is demonstrated.

If the patient cannot tolerate or is resistant to aspirin, clopidogrel is an alternative.

Primary options

aspirin: 3-5 mg/kg/day orally

Secondary options

clopidogrel: consult specialist for guidance on dose

Plus – anticoagulant

Back
ONGOING
|
after initial episode: Z score ≥10 or absolute luminal diameter ≥8 mm; large or giant aneurysm
|
current or persistent
Plus – 

anticoagulant

Treatment recommended for ALL patients in selected patient group

Patients with evidence of large or giant aneurysms, or of coronary obstruction, are at high risk for developing myocardial ischaemia. These patients need warfarin (to keep the INR at 2-3), or a low molecular weight heparin (LMWH) such as enoxaparin (to keep anti-factor Xa level at 0.5 to 1.0 units/mL), in addition to antiplatelet therapy.

LMWH is preferred as an alternative to warfarin for infants and toddlers in whom blood drawing for INR testing is difficult.[1]

Primary options

warfarin: 0.2 mg/kg orally as a loading dose, followed by 0.1 mg/kg/day, titrate dose to achieve an INR of 2-3

OR

enoxaparin: 1 to 1.5 mg/kg subcutaneously every 12 hours

Plus – follow-up + advice

Back
ONGOING
|
after initial episode: Z score ≥10 or absolute luminal diameter ≥8 mm; large or giant aneurysm
|
current or persistent
Plus – 

follow-up + advice

Treatment recommended for ALL patients in selected patient group

Promote healthy lifestyle and activity at every visit.

Assess patients at 1, 2, 3, 6, 9 and 12 months after the acute episode and every 3-6 months thereafter.

Assess cardiovascular risk (blood pressure, fasting lipid profile, BMI, waist circumference, dietary and activity assessment, and smoking) every 6-12 months. Obtain a follow-up fasting lipid profile.

Assess for inducible myocardial ischaemia (stress echocardiography, stress with MRI, stress nuclear medicine, or PET) every 6-12 months, if the patient has symptoms suggestive of ischaemia 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 ischaemia 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.

Consider – additional antiplatelet agent

Back
ONGOING
|
after initial episode: Z score ≥10 or absolute luminal diameter ≥8 mm; large or giant aneurysm
|
current or persistent
Consider – 

additional antiplatelet agent

Additional treatment recommended for SOME patients in selected patient group

Consider dual antiplatelet therapy with aspirin and an additional antiplatelet agent such as clopidogrel (if the patient is not already on it) together with warfarin or low molecular weight heparin in the setting of very extensive or distal coronary artery aneurysms or if there is a history of coronary artery thrombosis.

If the patient is not able to tolerate or is resistant to aspirin, and is therefore already on clopidogrel, consider adding dipyridamole as an alternative. Dipyridamole is no longer generally used for long-term thromboprophylaxis. It is an alternative for aspirin in patients who are taking ibuprofen, resistant or allergic to aspirin, or at risk of Reye syndrome.[1]

Primary options

clopidogrel: consult specialist for guidance on dose

Secondary options

dipyridamole: consult specialist for guidance on dose

Consider – beta-blocker

Back
ONGOING
|
after initial episode: Z score ≥10 or absolute luminal diameter ≥8 mm; large or giant aneurysm
|
current or persistent
Consider – 

beta-blocker

Additional treatment recommended for SOME patients in selected patient group

Beta-blockers are not part of the standard treatment of KD. They may be considered on an individual basis for high-risk patients with large or giant aneurysms, or for patients who develop myocardial ischaemia.[1]

Consult a cardiology specialist for guidance on drug selection and dose.

1st line – discontinue anticoagulant and start dual antiplatelet therapy

Back
ONGOING
|
after initial episode: Z score ≥10 or absolute luminal diameter ≥8 mm; large or giant aneurysm
|
regression to medium aneurysm
1st line – 

discontinue anticoagulant and start dual antiplatelet therapy

Risk stratification for long-term management is based primarily on maximal coronary artery Z scores, and takes into account both past and current involvement.

Discontinue anticoagulation (warfarin or low molecular weight heparin) and substitute with an additional antiplatelet agent.

Treat with low-dose aspirin plus clopidogrel. Treatment should continue at least until aneurysm regression is demonstrated.

If the patient cannot tolerate or is resistant to aspirin, clopidogrel is an alternative.

Primary options

aspirin: 3-5 mg/kg/day orally

and

clopidogrel: consult specialist for guidance on dose

Secondary options

clopidogrel: consult specialist for guidance on dose

and

dipyridamole: consult specialist for guidance on dose

Plus – follow-up + advice

Back
ONGOING
|
after initial episode: Z score ≥10 or absolute luminal diameter ≥8 mm; large or giant aneurysm
|
regression to medium aneurysm
Plus – 

follow-up + advice

Treatment recommended for ALL patients in selected patient group

Promote healthy lifestyle and activity at every visit.

Assess the patient every 6-12 months.

Assess cardiovascular risk (blood pressure, fasting lipid profile, BMI, waist circumference, dietary and activity assessment, and smoking) every 12 months. Obtain a follow-up fasting lipid profile.

Assess for inducible myocardial ischaemia (stress echocardiography, stress with MRI, stress nuclear medicine, or PET) every 12 months or if the patient has symptoms suggestive of ischaemia 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 ischaemia 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.

Consider – beta-blocker

Back
ONGOING
|
after initial episode: Z score ≥10 or absolute luminal diameter ≥8 mm; large or giant aneurysm
|
regression to medium aneurysm
Consider – 

beta-blocker

Additional treatment recommended for SOME patients in selected patient group

Beta-blockers are not part of the standard treatment of KD. They may be considered on an individual basis for high-risk patients with large or giant aneurysms, or for patients who develop myocardial ischaemia.[1]

Consult a cardiology specialist for guidance on drug selection and dose.

1st line – antiplatelet agent

Back
ONGOING
|
after initial episode: Z score ≥10 or absolute luminal diameter ≥8 mm; large or giant aneurysm
|
regression to small aneurysm
1st line – 

antiplatelet agent

Risk stratification for long-term management is based primarily on maximal coronary artery Z scores, and takes into account both past and current involvement.

Treat with low-dose aspirin. Treatment should continue at least until aneurysm regression is demonstrated.

If the patient cannot tolerate or is resistant to aspirin, clopidogrel is an alternative.

Primary options

aspirin: 3-5 mg/kg/day orally

Secondary options

clopidogrel: consult specialist for guidance on dose

Plus – follow-up + advice

Back
ONGOING
|
after initial episode: Z score ≥10 or absolute luminal diameter ≥8 mm; large or giant aneurysm
|
regression to small aneurysm
Plus – 

follow-up + advice

Treatment recommended for ALL patients in selected patient group

Promote healthy lifestyle and activity at every visit.

Assess the patient every 6-12 months.

Assess cardiovascular risks (blood pressure, fasting lipid profile, BMI, waist circumference, dietary and activity assessment, and smoking) every 12 months. Obtain a follow-up fasting lipid profile.

Assess for inducible myocardial ischaemia (stress echocardiography, stress with MRI, stress nuclear medicine, or PET) every 1-2 years, if the patient has symptoms suggestive of ischaemia 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 ischaemia 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.

Consider – discontinue dual antiplatelet therapy

Back
ONGOING
|
after initial episode: Z score ≥10 or absolute luminal diameter ≥8 mm; large or giant aneurysm
|
regression to small aneurysm
Consider – 

discontinue dual antiplatelet therapy

Additional treatment recommended for SOME patients in selected patient group

Dual antiplatelet therapy is generally not indicated, but additional patient and coronary artery risk factors may be considered when making decisions regarding thromboprophylaxis.

If patients remain on dual antiplatelet therapy, restrict or modify risk of bodily contact or trauma.

Consider – beta-blocker

Back
ONGOING
|
after initial episode: Z score ≥10 or absolute luminal diameter ≥8 mm; large or giant aneurysm
|
regression to small aneurysm
Consider – 

beta-blocker

Additional treatment recommended for SOME patients in selected patient group

Beta-blockers are not part of the standard treatment of KD. They may be considered on an individual basis for high-risk patients with large or giant aneurysms, or for patients who develop myocardial ischaemia.[1]

Consult a cardiology specialist for guidance on drug selection and dose.

1st line – antiplatelet agent

Back
ONGOING
|
after initial episode: Z score ≥10 or absolute luminal diameter ≥8 mm; large or giant aneurysm
|
regression to normal Z score or dilation only
1st line – 

antiplatelet agent

Risk stratification for long-term management is based primarily on maximal coronary artery Z scores, and takes into account both past and current involvement.

Treat with low-dose aspirin. Treatment should continue at least until aneurysm regression is demonstrated.

If the patient cannot tolerate or is resistant to aspirin, clopidogrel is an alternative.

Primary options

aspirin: 3-5 mg/kg/day orally

Secondary options

clopidogrel: consult specialist for guidance on dose

Plus – follow-up + advice

Back
ONGOING
|
after initial episode: Z score ≥10 or absolute luminal diameter ≥8 mm; large or giant aneurysm
|
regression to normal Z score or dilation only
Plus – 

follow-up + advice

Treatment recommended for ALL patients in selected patient group

Promote healthy lifestyle and activity at every visit.

Follow up every 1-2 years. Consider not performing routine 2D echocardiography unless there is evidence of inducible myocardial ischaemia, the patient has symptoms suggestive of ischaemia, or the patient has signs suggestive of ventricular dysfunction.

Assess cardiovascular risk (blood pressure, fasting lipid profile, BMI, waist circumference, dietary and activity assessment, and smoking) every 2 years. Obtain a follow-up fasting lipid profile.

Assess for inducible myocardial ischaemia (stress echocardiography, stress with MRI, stress nuclear medicine, or PET) every 2-5 years, if the patient has symptoms suggestive of ischaemia or signs suggestive of ventricular dysfunction. Further imaging with angiography may not be needed in the absence of evidence of inducible myocardial ischaemia.

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.

Consider – intensify or discontinue thromboprophylaxis

Back
ONGOING
|
after initial episode: Z score ≥10 or absolute luminal diameter ≥8 mm; large or giant aneurysm
|
regression to normal Z score or dilation only
Consider – 

intensify or discontinue thromboprophylaxis

Additional treatment recommended for SOME patients in selected patient group

Patient and coronary artery characteristics may influence decisions about thromboprophylaxis.

If patients are on dual antiplatelet therapy, restrict or modify risk of bodily contact or trauma.

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Please note that formulations/routes and doses may differ between drug names and brands, drug formularies, or locations. Treatment recommendations are specific to patient groups. See disclaimer

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