What is Kawasaki disease?

Kawasaki disease (KD) is an acute self-limiting vasculitis (blood vessel inflammation) of medium-sized vessels that predominantly affects children under 5 years (peak incidence 6-24 months). It is the most common cause of acquired heart disease in children in high-income countries. The cause is unknown — likely triggered by an infectious agent in genetically susceptible children, causing an excessive inflammatory response. Without treatment, approximately 15-25% of affected children develop coronary artery aneurysms (CAA) — ballooning of the coronary arteries that can lead to myocardial infarction or sudden death years or decades later. With timely IVIG treatment, the CAA risk falls to approximately 3-5%.

How is Kawasaki disease diagnosed?

KD diagnosis is clinical — there is no single diagnostic test (no blood test, swab or imaging that confirms KD). Diagnosis requires: fever for ≥5 days AND at least 4 of the 5 following features: (1) Bilateral conjunctival injection (red eyes — non-purulent, no discharge). (2) Oral changes: "strawberry tongue" (bright red with prominent papillae); red, cracked, dry lips; red oropharynx. (3) Polymorphous (varied appearance) rash on the trunk. (4) Changes to extremities: redness and swelling of palms and soles (acute), then desquamation (skin peeling) beginning at fingertips (subacute). (5) Unilateral cervical lymphadenopathy ≥1.5cm. Echocardiography (to detect early coronary involvement) and blood tests (elevated CRP, ESR, WBC; elevated platelets in subacute phase) support the diagnosis.

What is the treatment for Kawasaki disease?

IVIG (intravenous immunoglobulin) 2g/kg as a single infusion over 10-12 hours — this must be given within the first 10 days of fever onset to maximise protection against coronary artery aneurysm. Combined with: high-dose aspirin (30-50mg/kg/day in 4 divided doses) during the acute febrile phase, then switched to low-dose (3-5mg/kg/day once daily) during the subacute and convalescent phases. The response is usually dramatic — fever resolves within 24-36 hours of IVIG in most patients. IVIG-resistant KD (fever persisting/recurring ≥36 hours after IVIG): second dose of IVIG 2g/kg OR infliximab (anti-TNF) OR corticosteroids — choice depends on risk score and centre experience.

What are the long-term consequences of KD?

For the majority (approximately 95-97% who received timely IVIG) with no coronary abnormalities at 6-8 weeks: the prognosis is excellent, with no increased cardiovascular risk compared to the general population. For the approximately 3-5% who develop coronary artery dilation or aneurysm: Small/medium aneurysms: often regress within 1-2 years; may have intimal thickening; low-dose aspirin continued until resolution. Giant aneurysms (≥8mm diameter or Z-score ≥10): high risk of coronary thrombosis and MI; require lifelong management — anticoagulation (warfarin ± aspirin or LMWH), lifelong cardiology follow-up, possible coronary angiography and intervention in adulthood. Adults who had unrecognised or untreated KD in childhood may present with premature myocardial infarction of unknown cause in their 20s-40s.

How is Kawasaki disease different from scarlet fever?

Both conditions can cause fever, rash and strawberry tongue in young children — making clinical distinction important since their management is completely different. Key differences: Scarlet fever (group A streptococcal pharyngitis): age typically 5-15 years; starts with sore throat, then fine sandpaper-like scarlatiniform rash; perioral pallor; strawberry tongue; positive throat culture or rapid strep test; responds promptly to penicillin/amoxicillin. Kawasaki disease: peak age under 5 years; no identifiable infectious agent; polymorphous (not scarlatiniform) rash; prominent eye (conjunctivitis), extremity and lymph node features; negative throat culture; coronary artery involvement; treated with IVIG, not antibiotics. Both diagnoses should be considered in a young child with prolonged fever and mucocutaneous features.

How does GMJ News cover Kawasaki disease?

GMJ News covers KD as the leading acquired paediatric heart disease in HICs, the 5 diagnostic criteria, strawberry tongue, IVIG 2g/kg within 10 days, aspirin protocol, coronary artery Z-scores, IVIG-resistant KD (infliximab), long-term CAA management, MIS-C post-COVID, echocardiographic surveillance, and late-presenting adult consequences. Kawasaki disease is directly relevant to PHIG's paediatric health programme and Georgia's paediatric cardiology capacity.

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Kawasaki Disease

GMJ News knowledge hub · last reviewed September 2026 · Georgian Medical Journal

Summary Statistics Glossary GMJ news FAQ Documents Organizations Research

Kawasaki disease (KD) — an acute self-limiting vasculitis of medium-sized vessels predominantly affecting children under 5 — is the most common cause of acquired heart disease in children in high-income countries, threatening approximately 15-25% of untreated children with coronary artery aneurysms that can lead to myocardial infarction and sudden death decades later, making timely diagnosis and treatment a paediatric emergency (WHO). Intravenous immunoglobulin (IVIG) 2g/kg as a single infusion within the first 10 days of illness — combined with high-dose aspirin — reduces coronary aneurysm risk from approximately 20-25% to approximately 3-5%, representing one of the most dramatic treatment responses in all of paediatric medicine; the key diagnostic challenge is that no single test confirms KD — it requires recognition of the clinical criteria in a febrile child.

Key messages

Most common cause of acquired heart disease in children in HICs

Kawasaki disease (KD) is the most common cause of acquired heart disease in children in high-income countries — surpassing rheumatic fever in industrialised nations. The primary danger: coronary artery aneurysms (CAA), occurring in 15-25% of untreated children, that can lead to myocardial infarction or sudden death decades later.

IVIG 2g/kg within 10 days — the life-saving treatment

A single dose of IVIG (intravenous immunoglobulin) 2g/kg infused over 10-12 hours — given within the first 10 days of fever onset — reduces coronary artery aneurysm risk from approximately 20-25% to approximately 3-5%. Combined with high-dose aspirin (30-50mg/kg/day in the acute phase). One of the most dramatic and satisfying treatment responses in paediatric medicine.

No single diagnostic test — clinical criteria only

KD diagnosis is clinical — no pathognomonic test. Diagnosis requires fever ≥5 days PLUS ≥4 of 5 features: Conjunctival injection (bilateral, non-purulent); Mouth changes (strawberry tongue, red cracked lips, diffuse oropharyngeal redness); Rash (polymorphous, non-vesicular); Extremity changes (erythema/oedema of hands/feet, then desquamation); Cervical lymphadenopathy (≥1.5cm, usually unilateral). OR fever ≥5 days + <4 features + coronary artery changes (incomplete/atypical KD).

Strawberry tongue — the memorable diagnostic clue

The "strawberry tongue" of Kawasaki disease — a bright red tongue with prominent papillae (resembling a strawberry) — combined with red, cracked, dry lips and erythematous oropharynx constitutes the "oral changes" criterion and is one of the most visually memorable findings in paediatric medicine. Also seen in scarlet fever (group A strep — also presents with fever, rash, strawberry tongue; treated with penicillin).

Coronary artery aneurysm — the long-term legacy

Giant CAA (≥8mm or Z-score ≥10): the most serious form — risk of coronary thrombosis, stenosis, MI and sudden death over decades. Long-term anticoagulation (warfarin ± aspirin) and cardiology follow-up required for life. Even after treatment with IVIG, approximately 3-5% develop some degree of coronary dilation — requiring echocardiography surveillance for months to years. The Z-score system (adjusted for body surface area) is used to classify aneurysm severity.

Infliximab for IVIG-resistant KD

Approximately 10-20% of KD patients fail to defervesce after initial IVIG and require a second dose of IVIG (2g/kg). Infliximab (anti-TNF) is used for IVIG-resistant KD — evidence from randomised trials showing faster defervescence and reduced CRP. Corticosteroids + IVIG may reduce CAA risk in high-risk patients (Japanese Cardiovascular Risk Score ≥5).

Key statistics

15-25%

coronary artery aneurysm risk in untreated KD children

AAP/AHA

3-5%

CAA risk with timely IVIG + aspirin treatment

AAP/AHA 2017

2g/kg

IVIG single dose within 10 days — the standard KD treatment

AAP/ESC

most common cause of acquired heart disease in children in HICs

AAP/AHA

Fever ≥5 days

plus ≥4 of 5 features — the classic diagnostic criteria

AAP/EULAR

10-20%

of KD patients are IVIG-resistant → second IVIG or infliximab

AAP/Cardiology

Kawasaki disease — diagnostic features frequency and coronary outcome

Source: AAP/AHA. IVIG within 10 days reduces CAA from 25% to 3-5%.

Glossary of key terms

Coronary artery Z-score
AHA/PaediatricsA standardised measurement of coronary artery diameter adjusted for body surface area (BSA) — allowing meaningful comparison across different patient sizes. Z-score >2.0: dilation (mild). Z-score 2.0-2.5: dilation (equivocal). Z-score 2.5-5.0: small aneurysm. Z-score 5.0-10.0: medium aneurysm. Z-score ≥10.0 or absolute diameter ≥8mm: giant aneurysm — highest risk of thrombosis, stenosis, MI and sudden death. Z-scores are calculated from normative tables using BSA and echocardiographic measurements of LAD, LCx, RCA.

Incomplete/atypical Kawasaki disease
AAP 2017Incomplete KD: fever ≥5 days + ≤3 diagnostic features + laboratory evidence of systemic inflammation (CRP ≥3.0 mg/dL or ESR ≥40 mm/h) + echocardiographic evidence of coronary involvement OR echocardiographic Z-score ≥2.5 for LAD or RCA. Recognised as a distinct entity especially in infants <6 months (who have the highest CAA risk) and in older children. Infants <6 months with unexplained prolonged fever should always have echocardiography to exclude KD. The consequences of missed KD (untreated CAA) are severe — a low threshold for incomplete KD diagnosis is essential.

IVIG mechanism
Pharmacology/ImmunologyThe mechanism by which IVIG prevents coronary artery aneurysm in KD is not fully understood. Proposed mechanisms: immunomodulation — anti-idiotypic antibodies neutralising pathogenic antibodies; Fc receptor blockade on macrophages; increased regulatory T-cell activity; anti-inflammatory cytokine modulation; suppression of IL-1, TNF and other pro-inflammatory mediators driving coronary vasculitis. The 2g/kg single infusion protocol was established empirically — lower doses (400mg/kg × 4 days — older protocol) are inferior.

Aspirin in KD — changing role
AAP 2017/CardiologyAspirin has two roles in KD: (1) High-dose (anti-inflammatory): 30-50mg/kg/day in 4 divided doses during the acute febrile phase — reduces inflammation; combined with IVIG. (2) Low-dose (antiplatelet): 3-5mg/kg/day (baby aspirin) once daily during the subacute and convalescent phases — prevents thrombosis in coronary dilation. Duration: 6-8 weeks after illness onset (if no CAA); indefinitely (if persistent CAA). Note: American Heart Association 2017 guidelines allow the option of low-dose aspirin throughout (omitting high-dose) — no high-quality RCT demonstrates superiority of high-dose vs low-dose during the acute phase.

MIS-C (Multisystem Inflammatory Syndrome in Children)
WHO/CDCA KD-like illness occurring 2-6 weeks after COVID-19 infection in children — first described in April 2020. Overlapping features with KD: fever; rash; conjunctivitis; mucous membrane changes; gastrointestinal symptoms; elevated inflammatory markers. Distinct features: older age at presentation (peak 9-10 years vs 2-3 years for KD); more frequent cardiac involvement (myocarditis, shock); more severe presentation (often requiring ICU); different geography (less prevalent in East Asia than classic KD). Treatment: IVIG ± corticosteroids; similar to KD management. COVID-19 vaccine has dramatically reduced MIS-C incidence.

Echocardiographic surveillance post-KD
AAP/CardiologyAll children with confirmed KD require serial echocardiography: at diagnosis; at 2 weeks; at 6-8 weeks post-illness. For those with coronary abnormalities: more frequent surveillance (3 monthly, 6 monthly, then annually depending on severity). No coronary involvement at 6-8 weeks: coronary involvement very unlikely — standard annual cardiology review recommended. Giant aneurysms: lifelong cardiology follow-up; anticoagulation; coronary angiography + exercise testing as adults; coronary intervention or bypass if stenosis develops. Adults with undiagnosed KD in childhood (before echocardiography was standard) may present with premature ischaemic heart disease of unknown cause.

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