Kawasaki Disease
| We're mainly worried about development of coronary artery aneurysms. | |
|---|---|
| Etiology | Unclear cause. Self limiting, multi-system vasculitis of childhood. |
| Epidemiology | Age <5. M:F = 1.5:1 Most common cause of acquired heart disease in developed countries. |
| Clinical presentation | >5 days fever - Bilateral conjunctival injection without exudate. - Erythema and cracking of lips, strawberry tongue, or erythema of oral and pharyngeal mucosa. - Oedema and erythema in the hands and feet. - Polymorphous rash. - Cervical lymphadenopathy. Ddx: Staph/Strep infection, measles, SJS, PIMS-TS |
| Pathogenesis | Proposed mech - necrotising arteritis → chronic vasculitis → myofibroblastic proliferation |
| Diagnostic investigations | Echo - initial and repeat → role = quantify coronary artery involvement with Z-score |
| Management | - IVIG single dose - can be given with delayed diagnosis/presentation - Aspirin until afebrile for 72h → low dose if no CA involvement - DAPT with clopidogrel if aspirin contra-indicated, or as dual agent if Z-score > 5 - 2nd, 3rd line: Steroids, Infliximab, etanercept Peak onset of MI etc in first few months after illness...! Management similar to |
Higher risk groups:
- age <1
- CRP ↑
- liver dysfunction
- ↓ albumin, anaemia
- CA Z-score >2.5 on baseline echo