Kawasaki Disease
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This document provides information about Kawasaki disease: - It is an acute febrile illness that predominantly affects children under 5 years old. It causes systemic inflammation of medium-sized arteries. - Diagnosis is based on fever for at least 5 days plus four out of five criteria including conjunctivitis, rash, swelling of hands and feet, adenopathy, and strawberry tongue. - Treatment involves intravenous immunoglobulin (IVIG) and aspirin to reduce inflammation and prevent coronary artery abnormalities.
![Aspirin
Benefit:
Eases pain and discomfort.
High dose reduces swelling.
Low dose prevents blood clot forming.
Dose:
High dose [Anti-inflammatory]: (80-100 mg/kg/day) for 2 wks
or until afebrile, followed by..
Low dose [Antiplatelets]: (2-5 mg/kg) for 6 wks.
Side effects:
Reye syndrome.](https://image.slidesharecdn.com/kawasaki-220727144113-0fda412c/85/Kawasaki-Disease-20-320.jpg)
Kawasaki Disease
- 1. Dr Ehab Zahran MBBCh, MSc, MRCPCH
- 2. An acute febrile self-limiting illness of unknown cause that Predominantly affects children <5 years of age. It was first described in Japan by Tomisaku Kawasaki in 1967.
- 3. Epidemiology Incidence: 30:100000. Commonnest in: Asian descent, especially in Japan and Korea. Gender: Male:Female = 1.6:1 Recurrence: 3-5% Season: Winter and Spring.
- 4. Pathogenesis Systemic inflammation of medium-sized arteries Multiple organ and tissue involvement Hepatitis, Gastroenteritis, Meningitis, Pneumonia, Pancreatitis, Puyria, Lymphadenopathy
- 5. Criteria Fever >5 days + Four of: Conjunctivitis : Non-purulent. Rash : Polymorphous. Edema : Swelling/Erythema of hands & feet. Adenopathy : Cervical - >1.5 cm - Non-tender. Mucosal : Strawberry tongue + Cracked dry lips. *Absence of another diagnosis: Scarlet fever or Measles. *Coronary a. aneurysm + 1 feature = Diagnostic.
- 7. Incomplete Kawasaki Disease Fever >5 days + 2-3 Criteria OR Fever >7 days with no other explanation CRP <30 - ESR <40 + Fever + Peeling CRP >30 - ESR >40 + Anemia + High Plts/ALT/WBCs - Low Albumin
- 9. High Risk Patients *Hemophagocytic lymphohistiocytopenia: Persistent fever, HSM, High D-dimers/TG/Fibrinogen/Ferritin. Failed IVIG <1 year old Severe inflammation (High CRP after IVIG) *Hemophagocytic lymphohistiocytopenia Shock Coronary aneurysm Kobayashi score >5
- 12. Workup (None is diagnostic) Test Result Laboratory CBC Neutrophilia - Thrombocytopenia (early) CRP - ESR - ALT - LFT - Ferritin - D-Dimers High Albumin - Sodium Low Imaging Echo Coronary Aneurysm Others CSF Lymphocytosis Throat swab GABHS ECG ST-depression - Heart block
- 13. Complications Complication Frequency Shock 7% Decreased cardiac contractility Almost all Coronary artery involvement Frequent GI abnormalities Common Non-coronary vascular involvement Rare Macrophage activation syndrome Rare Renal disease Rare Sensoryneural hearing loss Rare
- 16. Predicting CA Aneurysms in Kawasaki Disease
- 17. Management Non-Medical • Restriction of daily activity for 6 weeks (Echo repeat). Medical • Aspirin. • IVIG. • Steroids. Precautions • Vaccines. • Follow up.
- 18. IVIG Benefit: Lowers the incidence of CA abnormalities. Dose: Dose: 2 g/kg (over 10-12 hours). Side effects: Hemolytic anemia, Aseptic meningitis.
- 19. IVIG Treatment of IVIG-resistant cases: • IVIG second infusion. • IVIG + Prednisolone. • Infliximab. • Cyclosporine. • Cyclophosphamide. • Plasma exchange.
- 20. Aspirin Benefit: Eases pain and discomfort. High dose reduces swelling. Low dose prevents blood clot forming. Dose: High dose [Anti-inflammatory]: (80-100 mg/kg/day) for 2 wks or until afebrile, followed by.. Low dose [Antiplatelets]: (2-5 mg/kg) for 6 wks. Side effects: Reye syndrome.
- 21. Steroids Benefits: Lower the incidence of CA abnormalities. Lower risk of IVIG resistance. Dose: Methylprednisolone for 1 week, followed by.. Prednisolone (wean over 2-3 wks). Indications: High risk patients. Persistent fever after IVIG.
- 24. Vaccines Avoid live vaccines for 3 months following treatment with IVIG.
- 26. Thank You