Kawasaki Disease (AHA 2024)

Algorithm Steps

1. ▶

   Start

   Suspected Kawasaki Disease

   Fever ≥5 days + clinical features

   1. ●

      Action

      Diagnostic Criteria

      Classic KD: Fever ≥5 days + ≥4 of 5 features

      • PRINCIPAL FEATURES:
      • 1. Bilateral non-purulent conjunctival injection
      • 2. Oral mucous membrane changes (red lips, strawberry tongue)
      • 3. Peripheral extremity changes (erythema, edema, desquamation)
      • 4. Polymorphous rash (non-vesicular)
      • 5. Cervical lymphadenopathy (≥1.5 cm, usually unilateral)
      • INCOMPLETE KD: Fever ≥5 days + 2-3 features + supportive labs/echo
      1. ●

         Action

         Echocardiogram

         Assess coronary arteries

         • Obtain echo at diagnosis
         • Measure LAD and RCA z-scores
         • Z-SCORE INTERPRETATION:
         • • Normal: z <2
         • • Dilation: z 2-2.5
         • • Small aneurysm: z 2.5-5
         • • Medium aneurysm: z 5-10
         • • Giant aneurysm: z ≥10 or ≥8mm
         • Also assess LV function, valves, pericardium
         1. ◆

            Decision

            Risk Stratification (AHA 2024)

            High-risk features?

            • HIGH-RISK CRITERIA:
            • • Age ≤6 months
            • • Z-score ≥2.5 in LAD or RCA at diagnosis
            • High-risk patients may benefit from intensified initial therapy
            1. ●

               Action

               Standard Treatment

               IVIg + Aspirin

               • IVIg 2 g/kg IV over 10-12 hours
               • Single infusion preferred
               • ASPIRIN:
               • • High-dose: 30-50 mg/kg/day or 80-100 mg/kg/day divided q6h
               • • Continue until afebrile 48-72 hours
               • • Then low-dose: 3-5 mg/kg/day (max 81 mg)
               • Continue low-dose aspirin 6-8 weeks minimum
               • (longer if CAA present)
               1. ◆

                  Decision

                  Response to IVIg?

                  Afebrile within 36-48 hours?

                  1. ✓

                     Outcome

                     Follow-Up Care

                     Based on coronary artery involvement

                     • REPEAT ECHO:
                     • • 2 weeks after treatment
                     • • 6-8 weeks after illness onset
                     • • More frequent if CAA present
                     • NO CAA: Low-dose aspirin 6-8 weeks
                     • CAA PRESENT:
                     • • Continue aspirin indefinitely
                     • • Add anticoagulation for giant aneurysms
                     • • Cardiology follow-up
                     • • Activity restrictions based on severity
                  2. ⚠

                     Warning

                     IVIg-Refractory KD

                     Persistent or recurrent fever >36h after IVIg

                     • OPTIONS:
                     • • Second dose IVIg 2 g/kg
                     • • Infliximab 5-10 mg/kg if not already given
                     • • Methylprednisolone pulse 30 mg/kg x3 days
                     • • Cyclosporine for refractory cases
                     • Consult pediatric cardiology/rheumatology
            2. ●

               Action

               High-Risk: Intensified Therapy

               Consider adjunctive treatment

               • IVIg 2 g/kg + Aspirin
               • +
               • ADJUNCTIVE OPTIONS:
               • • Corticosteroids: Methylprednisolone 2 mg/kg/day or pulse
               • • Infliximab: 5-10 mg/kg IV (single dose)
               • Limited data for anakinra, cyclosporine