Kawasaki Disease: History Definition Criteria Diagnosis Complications Management Follow-up

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

11. Differential Diagnosis

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