Kawasaki disease is an acute vasculitis that primarily affects children under 5 years of age. It is characterized by fever for at least 5 days and at least 4 of 5 clinical features including rash, conjunctivitis, mouth changes, swelling of hands and feet, and cervical lymphadenopathy. If left untreated, it can lead to coronary artery aneurysms in 15-25% of cases. Treatment involves intravenous immunoglobulin and aspirin which are most effective within the first 10 days of symptoms to reduce the risk of aneurysms and complications like myocardial infarction.

1. Presented by George Greige
Kawasaki disease

2. Outline
• Epidemiology
• Etiology
• Clinical features
• Diagnostics
• Treatment
• Complications
• References
• Q&A

3. Epidemiology

4. • Sex: ♂ > ♀ (1.5:1)
• Age: primarily children < 5 years
• Peak incidence: occurs mostly in late winter and spring
• Prevalence
‣ Approx. 20 per 100,000 children
‣ Highest rate in children of Asian and Paci
fi
c-Islander descent
• Mortality: < 0.5%
Age: primarily children < 5 years (most common cause of acquired coronary artery disease in children)
Mortality: < 0.5% is Mostly due to myocardial infarction or arrhythmia
Epidemiological data refers to the US, unless otherwise speci
fi
ed.

5. Etiology

6. • The exact cause of Kawasaki disease remains uncertain
• However, it is associated with infectious and genetic factors (the prevalence is
higher in patients of Asian descent and in siblings of a ected children)
Kawasaki disease does not appear to be contagious despite an association with infectious factors.

7. Clinical features

8. • Clinical diagnosis requires fever for at least 5 days and one of the following:
‣ ≥ 4 other speci
fi
c symptoms
‣ < 4 speci
fi
c symptoms and involvement of the coronary arteries

9. Specific symptoms include:
• Erythema and edema of hands and feet, including the palms and soles
• Possible desquamation of
fi
ngertips and toes after 2–3 weeks
• Polymorphous rash, originating on the trunk
• Painless bilateral “injected” conjunctivitis without exudate
• Oropharyngeal mucositis:
‣ Erythema and swelling of the tongue
‣ Cracked and red lips
• Cervical lymphadenopathy
Erythema and edema of hands and feet, including the palms and soles (the
fi
rst week)
Possible desquamation of
fi
ngertips and toes after 2–3 weeks
Polymorphous rash, originating on the trunk
Painless bilateral “injected” conjunctivitis without exudate
Oropharyngeal mucositis:
Erythema and swelling of the tongue (strawberry tongue) (More pink than red)
Cracked and red lips
Cervical lymphadenopathy (mostly unilateral)
“CRASH and BURN”: Conjunctivitis, Rash, Adenopathy, Strawberry tongue, Hands and feet, and BURN (fever ≥ 5 days) are the most common features of Kawasaki
disease.

11. Typical
fi
ndings in Kawasaki disease
(A) Bilateral, nonpurulent conjunctivitis
(B) Stomatitis with bright red lips
(C) Erythema and edema of the hands
(D) Erythema and edema of the feet
(E) Truncal rash
(F) Cervical lymphadenopathy
(G) Ultrasound of an enlarged cervical lymph node: increased perfusion
(H) Desquamation of
fi
ngertips
(I) Deep grooves in the nail (Beau lines)

12. Strawberry tongue in Kawasaki disease
The tongue appears enlarged and is covered by a grey-white exudate.
Prominent papillae are also visible.

13. Non-specific symptoms include:
• Diarrhea
• Fatigue
• Abdominal pain
• Arthritis
• Hoarseness
• Breathing di
ffi
culties
Nonspeci
fi
c symptoms may precede the onset of Kawasaki disease (e.g., diarrhea, fatigue, abdominal pain, arthritis, hoarseness, breathing di
ffi
culties)

14. Diagnostics

15. Kawasaki disease is a clinical diagnosis which is
supported by the following diagnostic findings:
• Laboratory
fi
ndings:
‣ ↑ ESR and CRP
‣ Leukocytosis
‣ Thrombocytosis
‣ ↑ AST, ALT
• Echocardiography:
• For evaluating coronary artery aneurysms
• Minimal evaluation should be performed at diagnosis, at 2 weeks, and at 6–8 weeks
after onset

16. Treatment

17. • IV immunoglobulin (IVIG)
‣ High single-dose to reduce the risk of coronary artery aneurysms
‣ Most e
ff
ective if given within the
fi
rst 10 days following disease onset
• High-dose oral aspirin
• IV glucocorticoids
IV immunoglobulin (IVIG)
High single-dose to reduce the risk of coronary artery aneurysms
Most e
ff
ective if given within the
fi
rst 10 days following disease onset
High-dose oral aspirin: initial high doses of aspirin are given for the anti-in
fl
ammatory e
ff
ect until the fever has subsided. Treatment is then continued with a lower dosage
for its antiplatelet e
ff
ect until ESR has normalized (1–2 months).
Kawasaki disease is a rare exception to the contraindication of giving children aspirin, which is associated with Reye syndrome.
IV glucocorticoids: may be considered in addition to standard treatment, esp. in cases of treatment-refractory disease, as they lower the risk of coronary involvement
To avoid the risk of Reye syndrome, children should not be treated with aspirin, especially if a viral infection is suspected. Kawasaki disease is an exception to this rule.

18. Complications

19. • Coronary artery aneurysm
• Myocardial infarction
• Myocarditis
• Ventricular dysfunction
• Arrhythmias
Coronary artery aneurysm:
The risk of developing coronary artery aneurysm in untreated patients is 15–25%.
The risk of aneurysms is highest during the second and third weeks following symptom onset.
Rupture or thrombosis of the aneurysm can be lethal.
Myocardial infarction
Myocarditis
Ventricular dysfunction:
Most commonly occurs within the
fi
rst 2 weeks of onset of Kawasaki disease
Often seen in infants with untreated Kawasaki disease
Most commonly associated with lymphocytic myocarditis: Lymphocytes and macrophages in
fi
ltrate arterial walls → vasculitis and dilation of small arteries → tissue
edema and myocardial lesions
Manifests with symptoms (e.g., tachypnea or diaphoresis with feeding, exertional dyspnea) and physical examination
fi
ndings of congestive heart failure (e.g.,
hepatomegaly, S3 gallop, rales or crackles on lung auscultation)
Increased risk of sudden death
Arrhythmias
I listed the most important complications. The selection is not exhaustive.

20. Coronary artery aneurysm in Kawasaki disease
Volume rendering of a thorax CT (anterior view, with contrast) showing aorta and coronary vessels
The proximal part of the left anterior descending branch of the left coronary artery (LCA) is dilating (green overlay), and there is signi
fi
cant variation of vessel diameters.
This abnormal morphology suggests an aneurysm of the LCA.

21. References

22. AMBOSS

23. Q&A