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Study guide · Rheumatology

Kawasaki disease: criteria, incomplete KD, IVIG and aspirin

Kawasaki disease is an acute vasculitis of young children, most under 5 years. Without treatment about 1 in 4 children develop coronary artery aneurysms; IVIG within 10 days of fever onset lowers that risk sharply.

For medical students, residents, nurses and pediatricians. Written from current guidelines; trees are from Pedemy's flashcards.

Main decision tree

Kawasaki disease: fever, principal features, treatment

Decision tree: Kawasaki disease: fever, principal features, treatment
From Pedemy's flashcards. Tap to open full size; explained in “Recognize classic Kawasaki disease” below.

Key points

  • Classic KD: fever for 5 days or more plus at least 4 of the 5 principal features.
  • Incomplete KD: fever ≥5 days with 2–3 features (or an infant with ≥7 days of unexplained fever) → check CRP and ESR.
  • CRP ≥3 mg/dL or ESR ≥40 mm/h, then 3 or more supplemental lab findings or a positive echo → treat.
  • Treatment: IVIG 2 g/kg as one infusion, ideally within 10 days of fever onset, plus aspirin.
  • After the fever settles, low-dose aspirin 3–5 mg/kg once daily until the 4–6 week echo is normal.
  • Echo at diagnosis, at 1–2 weeks and at 4–6 weeks after treatment.
  • Delay measles and varicella vaccines 11 months after 2 g/kg IVIG; give the yearly inactivated flu vaccine while on aspirin.

Recognize classic Kawasaki disease

Think of KD in any young child with fever for 5 days or more. The 5 principal features often come and go, so ask about features that have already settled.

  • Eyes: bilateral bulbar conjunctival injection without pus, often sparing the limbus.
  • Mouth: red, cracked lips, strawberry tongue, red throat without exudate.
  • Rash: polymorphous, often worse in the groin and perineum; not vesicular or bullous.
  • Hands and feet: red, swollen palms and soles early; peeling around the nails at 2–3 weeks.
  • Neck: one cervical node of 1.5 cm or more, usually on one side.
Other cluesWhy it matters
Gallbladder hydropsRight upper quadrant pain or mass on ultrasound
Sterile pyuriaUrine WBCs with a negative culture
Redness at the BCG scarA specific clue in BCG-vaccinated infants
Marked irritabilityCommon, especially in infants

Incomplete Kawasaki disease

Decision tree: Childhood vasculitis: the incomplete Kawasaki pathway
Childhood vasculitis: the incomplete Kawasaki pathway · from Pedemy's flashcards. Tap to open full size.

Incomplete KD is not milder disease: infants under 6 months often present this way and have the highest aneurysm risk.

Use the lab pathway when fever has lasted 5 days or more with 2 or 3 features, or 7 days or more in an infant with no other cause.

  • CRP ≥3.0 mg/dL or ESR ≥40 mm/h: look for supplemental findings.
  • Supplemental findings: anemia for age, platelets ≥450,000 after day 7, albumin ≤3.0 g/dL, raised ALT, WBC ≥15,000, urine ≥10 WBC/hpf.
  • 3 or more supplemental findings, or a positive echo → treat as KD.
  • CRP and ESR below those cut-offs: re-examine daily; get an echo if peeling appears.
  • Positive echo: LAD or RCA z score ≥2.5, an aneurysm, or several other suggestive findings.

Treatment: IVIG and aspirin

Decision tree: Kawasaki disease: treatment
Kawasaki disease: treatment · from Pedemy's flashcards. Tap to open full size.

Children at high risk of IVIG resistance or with aneurysms at diagnosis may also get corticosteroids or another added therapy; this is a cardiology decision.

  • IVIG 2 g/kg as a single infusion, best within the first 10 days of fever.
  • Still give IVIG after day 10 if fever or raised inflammatory markers persist, or if there are coronary changes.
  • Aspirin: moderate or high dose while febrile, then low dose 3–5 mg/kg once daily.
  • Stop low-dose aspirin at 4–6 weeks if the echo shows no coronary changes.
  • Avoid ibuprofen in children taking aspirin for coronary aneurysms: it blocks aspirin's antiplatelet effect.

If fever persists after IVIG

About 10–20% of children still have fever 36 hours or more after the IVIG infusion ends. This is IVIG resistance and raises the aneurysm risk.

Options are a second dose of IVIG 2 g/kg, corticosteroids or infliximab, chosen with the cardiology and rheumatology teams.

Echo follow-up and vaccines

  • Echo at diagnosis, again 1–2 weeks after treatment, and 4–6 weeks after treatment.
  • Children with aneurysms need closer imaging, antithrombotic therapy and lifelong cardiology care.
  • Measles- and varicella-containing vaccines: wait 11 months after 2 g/kg IVIG, because the antibodies in IVIG block the vaccine response.
  • Long-term aspirin carries a Reye syndrome risk with influenza or varicella: give the inactivated flu vaccine, not the live nasal one.

Look-alikes

ConditionWhat points away from KD
MIS-CRecent SARS-CoV-2 infection or exposure; older children; prominent GI symptoms, shock and myocardial dysfunction
Scarlet feverPositive group A strep test; sandpaper rash; improves quickly on antibiotics
MeaslesCough, coryza, Koplik spots; rash starts on the face
AdenovirusExudative pharyngitis or purulent conjunctivitis
Drug reaction, SJSNew medicine; mucosal erosions, target or blistering lesions
IgA vasculitisPalpable purpura on the legs and buttocks with normal platelets

Common questions

Can Kawasaki disease be diagnosed before day 5 of fever?

Yes. With 4 or more principal features, an experienced clinician can diagnose and treat on day 4.

What is the IVIG dose for Kawasaki disease?

2 g/kg given as one infusion, ideally within 10 days of fever onset.

How long do children with Kawasaki disease take aspirin?

Low-dose aspirin, 3–5 mg/kg once daily, usually continues until the echo 4–6 weeks after onset is normal. Children with aneurysms continue longer.

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Sources

Written for Pedemy in our own words from the sources above; checked November 2026. For education only, not medical advice; check doses and local protocols before treating a patient. Pedemy is independent and not affiliated with or endorsed by the organizations cited.

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