Definition & Risk Scores - The Unyielding Fever
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IVIG Resistance: Defined as persistent or recrudescent fever (≥38°C) lasting for ≥36 hours after the completion of the initial IVIG infusion. This affects up to 20% of Kawasaki disease patients.
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Key Laboratory Predictors for Resistance:
- ↑ C-reactive protein (CRP) >8 mg/dL
- ↑ Neutrophil count >80%
- ↓ Serum albumin <3.5 g/dL
- ↓ Sodium <135 mEq/L
- ↑ Alanine transaminase (ALT)
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Clinical Risk Scoring Systems:
| Parameter | Kobayashi Score (≥5 pts) | Egami Score (≥3 pts) |
|---|---|---|
| Age | ≤12 months (1 pt) | ≤6 months (1 pt) |
| Sodium | ≤133 mEq/L (2 pts) | - |
| AST | ≥100 IU/L (2 pts) | - |
| Platelets | ≤30.0 x10⁴/μL (1 pt) | - |
| CRP | ≥10 mg/dL (1 pt) | ≥8 mg/dL (1 pt) |
| Neutrophils | ≥80% (2 pts) | - |
| Albumin | - | ≤3.5 g/dL (1 pt) |
Treatment Algorithm - Plan B Protocols
Management of patients who remain febrile ≥ 36 hours after the initial IVIG infusion.
⭐ Patients with IVIG-resistant Kawasaki disease have a significantly higher risk (up to 25%) of developing coronary artery aneurysms compared to responders.
Complications & Monitoring - The Artery Attack
IVIG resistance significantly ↑ risk of Coronary Artery Aneurysms (CAA), the hallmark complication.
- Risk Profile:
- Untreated: ~25% develop CAA.
- IVIG-Treated: Risk ↓ to <5%.
- IVIG-Resistant: Risk is substantially higher, mandating aggressive follow-up.
- Monitoring:
- Intensified surveillance with serial echocardiography is crucial to track coronary artery dimensions and function.
- Other Complications:
- Myocarditis
- Macrophage Activation Syndrome (MAS)
⭐ Most coronary artery changes develop within the first 4 weeks after fever onset. Early detection is key.

High‑Yield Points - ⚡ Biggest Takeaways
- IVIG resistance is defined as persistent or recrudescent fever ≥36 hours after completing the initial IVIG infusion.
- It is the strongest predictor for the development of coronary artery aneurysms (CAA).
- High-risk patients can be identified by scoring systems like the Kobayashi score.
- The standard second-line treatment is another dose of IVIG (2 g/kg).
- For refractory cases, treatment options include corticosteroids (prednisolone) or infliximab.
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