Kawasaki Disease

1. Kawasaki Disease and
Cardiac Complications

2. “
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Presented by: Nischal Nihaal Kumar

3. What is Kawasaki Disease ?
▫ By definition, kawasaki
disease(KD) is a systemic
inflammatory disorder manifesting
as a vasculitis with a predilection
for the coronary arteries
▫ KD was first described by
Dr.Tomisaku Kawasaki in 1967.
▫ Highest incidence is seen in Asians
and native pacific islanders.
▫ Untreated children develop
coronary artery abnormalities
(CAA) including aneurysms.
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4. Etiology
▫ Idiopathic
▫ Autoimmune
▫ Infectious agents
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5. Epidemiology
▫ There is a higher susceptibility of Kawasaki
disease in boys when compared to girls of the
same age.
▫Children of Asian and Pacific Islander ancestry
have the highest rates of KD among all racial
groups.
▫KD in other countries like South Korea & United
Kingdom seems to be increasing as well.
▫Several ways have been formulated to determine
which patients with KD are at highest risk for CAA
for instance, the Kobayashi score.
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6. From the graphs:
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Country Incidence of KD IVIG Resistance
Japan 264.8/100,000 17.0%
Taiwan 55.9/100,000 12.5%
Germany 7.2/100,000 26.8%

7. Pathology
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8. 8
Pathophysiology
1. Myointimal proliferation (affected vessels may try to
heal to normal but become thicker
2. Scarring and calcification
3. Coronary thrombosis
4. Stenosis
5. MI
6. Death
Enlarged heart with giant coronary
artery aneurysms of the right
coronary and left anterior
descending arteries.

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Clinical Phases of KD
1. Acute febrile phase
• Fever
2. Subacute phase
• Desquamation
• Thrombocytosis
• Development of CAA
• Highest risk of sudden death in patients who develop aneurysms.
3. Convalescent phase
• Begins when all clinical signs of the illness have disappeared.
• Beau’s lines are seen

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Clinical Manifestations
▫ High grade fevers lasting more than 5 days
5 principal clinical criteria of KD are:
C- conjunctivitis (bilateral)
R- rash of various forms
E- edema and erythema
A- adenopathy
M- mucous membrane changes

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1. Conjunctivitis
▫ It usually begins after the onset
of fever during the acute stage
of the disease.
▫ Presents with limbal sparing
▫ It typically involves the bulbar
conjunctivae, is not
accompanied by suppuration
and is not painful.

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2. Rash
▫ rash of various forms
(maculopapular, erythema
multiforme, scarlatiniform or
less often psoriatic-like,
urticarial or micropustular)
▫ It can be polymorphic, not itchy
and usually develops up to the
fifth day of fever.

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3.Edema and Erythema
▫ There will be edema of the
hands and feet.
▫ Desquamation of skin in the
perineal region is common in
the acute phase.
▫ Desquamation of the fingers
and toes beings 2-3 weeks after
the onset of the illness.

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4. Adenopathy
▫ There will be nonsuppurative
cervical lymphadenopathy
which is usually unilateral.
▫ The affected node size may be
>1.5cm.
▫ Affected lymph nodes are painless
or minimally painful.

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5. Mucous membrane
changes
▫ Erythema of the oral and
pharyngeal mucosa is also seen
with strawberry tongue red and
dry cracked lips.
▫ These oral manifestations are
caused by the necrotizing
microvasculitis with fibrinoid
necrosis.

16. “
Kawasaki disease. Strawberry
tongue in patient with
mucocutaneous lymph
node syndrome.
Kawasaki disease. Congestion of
bulbar conjunctiva in a patient with
mucocutaneous lymph node
syndrome.
Indurative edema of the
hands in a patient with
mucocutaneous lymph node
syndrome.
Desquamation of the fingers in a patient with
mucocutaneous lymph node syndrome.

17. Cardiac Involvement in KD
▫ Myocarditis, pericarditis, valvular regurgitation, shock
▫ Coronary artery abnormalities
▫ Aneurysms of medium-sized noncoronary arteries
▫ Peripheral gangrene
▫ Aortic root enlargement
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18. Cardiac Complications
Kawasaki disease can lead to the following complications in the
Cardiovascular system:
1. Myocarditis
▫ Refers to the inflammation of the heart muscle (myocardium).
▫ KD Myocarditis often develops as a result of sub acute inflammation
of interstitial tissue of myocardium and is usually concentrated
around the coronaries.
▫ Manifests as tachycardia disproportionate to fever along with
diminished left ventricular systolic function.
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19. Cardiac Complications
2. Pericarditis
▫ Pericarditis with a small pericardial effusion can also occur during the
acute illness.
3. Valvular regurgitation
▫ Mitral regurgitation of at least mild severity is evident on
echocardiography in 10–25% of patients at presentation but
diminishes over time, except among rare patients with coronary
aneurysms and ischemic heart disease.
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20. Cardiac Complications
3. Coronary artery abnormalities
▫ Almost all the morbidity and mortality in KD occur inpatients with large or giant
coronary artery aneurysms.
▫ Coronary artery aneurysms occur as a sequel of the vasculitis in 20-25% of
untreated children.
▫ It is first detected at a mean of 10 days of illness and the peak frequency of
coronary artery dilation or aneurysms occur within four weeks of onset.
▫ Specifically, large or giant aneurysms are associated with the greatest risk of
thrombosis or stenosis, angina, and myocardial infarction.
▫ Rupture of a giant aneurysm is a rare complication.
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21. Let’s go digital!
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Coronary angiograms in 6 yr old boy with
Kawasaki disease.
MRI of coronary and peripheral artery aneurysms in
Kawasaki disease.

22. Laboratory Findings
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23. Diagnosis
▫ There is no diagnostic test for KD.
▫ For classic KD the diagnostic criteria require the presence of fever for at
least 4 days and at least 4 of 5 of the other principal characteristics of the
illness
▫ In atypical or incomplete KD , patients have persistent fever but <4 of the 5
characteristic clinical signs.
▫ Echocardiography should be performed at diagnosis and again after 1-2
week of illness.
▫ it is recommended that any infant age ≤6 mo with fever for ≥7 days without
explanation undergo echocardiography to assess the coronary arteries.
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24. Differential Diagnosis
Viral Infections
▫ Adenovirus
▫ Enterovirus
▫ Measles
▫ Epstein-Barr virus
▫ Cytomegalovirus
Bacterial Infections
▫ Scarlet fever
▫ Rocky Mountain
spotted fever
▫ Leptospirosis
▫ Bacterial cervical
lymphadenitis ±
retropharyngeal
phlegmon
▫ Meningococcemia
▫ Urinary tract infection
Rheumatologic Disease
▫ Systemic-onset juvenile idiopathic
arthritis
▫ Behçet disease
▫ Rheumatic fever
Other
▫ Toxic shock syndromes
▫ Serum sickness
▫ Staphylococcal scalded skin
syndrome
▫ Macrophage activation syndrome
▫ Drug hypersensitivity reactions
▫ Stevens-Johnson syndrome
▫ Aseptic meningitis
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25. Investigations
1.Full Blood Count
2. Echocardiogram
3. Urinalysis
4. Angiography
5. ECG
6. Temporal Artery
Biopsy
7. X-Ray
8. Coronary artery
catheterization
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26. Treatment
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27. Treatment
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28. Treatment Options for IVIG-Resistant Patients With
Kawasaki Disease
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29. Prognosis
▫ The prognosis for patients with CCA depends on the severity of coronary
disease; therefore, recommendations for follow-up and management are
stratified according to coronary artery status.
▫ The vast majority of patients with KD return to normal health; timely
treatment reduces the risk of coronary aneurysms to <5%.
▫ All children with a history of KD should be counseled regarding a heart-
healthy diet, adequate amounts of exercise, tobacco avoidance, and
intermittent lipid monitoring.
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30. References
Kliegman, R., Stanton, B., St. Geme, J., Schor, N., Behrman, R. and Nelson,
n.d. Nelson textbook of pediatrics. 21st ed. Elsevier, pp.5370-5391.
Slideshare.net. 2021. Kawasaki disease. [online] Available at:
https://www.slideshare.net/ikramdr01/kawasaki-disease-173273953
[Accessed 26 May 2021].
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5856963/
[Accessed 26 May 2021].
Youtube.com. 2020. Before you continue to YouTube. [online] Available at:
https://www.youtube.com/watch?v=EtNI4byIAsI&t=313s
[Accessed 26 May 2021].
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31. Thanks!
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