PE21.3 | Kawasaki Disease — Summary & Reflection

KEY TAKEAWAYS

Kawasaki disease is an acute medium-vessel vasculitis predominantly affecting children under 5 years, with unknown aetiology and a critical 10-day treatment window. Complete Kawasaki = fever ≥5 days + ≥4 CREAM criteria (Conjunctivitis, Rash, Extremity changes, Adenopathy, Mucositis). Incomplete Kawasaki = fever + 2–3 criteria + supplemental lab/echo evidence — carries equal CAA risk and must not be dismissed. Pathogenesis involves innate immune-mediated coronary arteritis with MMP-driven aneurysm formation. Treatment: IVIG 2 g/kg single infusion within 10 days (reduces CAA risk from 15–25% to <5%) + high-dose aspirin during febrile phase, then low-dose aspirin for 6–8 weeks (or indefinitely with aneurysms). IVIG resistance (fever ≥36h post-infusion) → second IVIG or infliximab. Echocardiography at diagnosis, 2 weeks, and 6–8 weeks; Z-scores guide long-term management. Giant aneurysms (Z ≥10) require lifelong cardiac surveillance. Defer live vaccines 11 months post-IVIG.

REFLECT

Consider the parents of a child who had Kawasaki disease diagnosed on day 9 of illness — one day before the 10-day window closed. The child received IVIG and developed no coronary aneurysm. How would you discuss with these parents the importance of early fever evaluation in young children, and how would you help them recognise Kawasaki disease early in the future if the condition recurs (recurrence rate ~1–3%)? Conversely, consider a child whose Kawasaki disease was missed and who developed a giant aneurysm. What system-level barriers (diagnostic unfamiliarity, limited echo availability, multiple clinic visits before specialist assessment) contributed to the delay, and what changes in clinical training and referral practice would prevent similar cases?