11/14/24, 10\:58 AM Kawasaki Disease

Kawasaki Disease

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Kawasaki disease\: acute, self-limiting systemic vasculitis a
Incidence\: 8 per 100,000 children under 5 in the UK; >200 per 100,000 in Japan.
A
cardiac death.
Aetiology\: unknown; suspected infectious trigger causing immune-mediated response in genetically susceptible children.
Risk factors\: Asian ethnicity, age \<5 years, male sex (1.5\:1 male to female ratio).
Clinical features\: fever ≥5 days plus 4/5 key features (mucositis, conjunctivitis, rash, peripheral changes, cervical
lymphadenopathy).
Natural history\:
Acute phase (1-2 weeks)\: high fever, irritability, rash, mucositis, peripheral erythema/oedema.
Subacute phase (2-4 weeks)\: afebrile, desquamation, highest risk for cardiac complications.
Convalescent phase (4-8 weeks)\: asymptomatic, resolution of clinical features, possible coronary artery aneurysm
improvement.
Di
idiopathic arthritis, Stevens-Johnson syndrome.
Investigations\:
Beside\: ECG (arrhythmias, ST-T changes), urinalysis (sterile pyuria).
Laboratory\: FBC (anaemia, raised WBC, platelets), U&Es, LFTs, ESR/CRP, ASOT.
Imaging\: echocardiogram (coronary artery aneurysms, valvular disease, thrombosis, myocarditis, pericardial e
Management\: hospital admission, oral aspirin (high-dose until fever resolves, then low-dose), intravenous immunoglobulin
(IVIG) within 10 days, possibly corticosteroids or in
Complications\: coronary artery aneurysms, myocarditis, pericarditis, arrhythmias, valvular disease, coronary artery
thrombosis, myocardial infarction, sudden cardiac death.
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A comprehensive topic overview

Introduction

Kawasaki disease is an acute self-limiting systemic vasculitis of unknown cause that mainly a
age of
1
It is found worldwide but has a preponderance for children of East Asian origin. In the UK, it a
under the age of 2 3
In Japan, the incidence is over 200 per 100,000 children under the age of
Kawasaki disease is a medium-sized artery vasculitis, particularly a
treatment are important to reduce the risk of major cardiac complications, including coronary artery aneurysms and
sudden cardiac death.
1
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Aetiology

The aetiology of Kawasaki disease remains unknown. The leading theory is that an infectious trigger causes an immune-
mediated response in a genetically susceptible child. However, no de
identi
3

Risk factors

Risk factors for Kawasaki disease include\:
4
Asian ethnicity, especially Japanese
Aged \<5 years (80% of cases occur in those under the age of
Male sex (male to female ratio 1.5\:1)

Clinical features

Kawasaki disease should be considered in any child with a fever lasting 5
there is no diagnostic test.
It is a clinical diagnosis, and
The diagnosis of Kawasaki disease is based on the American Heart Association diagnostic criteria.
6
American Heart Association diagnostic criteria
Presence of fever (usually ≥39°C) for at least
1. Mucositis\: erythema and cracking of the lips, strawberry tongue and/or oral erythema
2. Conjunctivitis\: bilateral conjunctivitis without exudate
3. Rash\: maculopapular, erythroderma or erythema multiforme
4. Peripheral changes\: erythema, oedema and/or desquamation of the hands and feet
5. Cervical lymphadenopathy
It is important to ask parents or carers whether any of these features have been present since the onset of the fever, as
some may have resolved by the time of assessment.
5
Some children do not ful
incomplete Kawasaki disease.

Clinical examination

All children presenting with fever should be thoroughly examined, covering all major body systems. Examination
suggesting Kawasaki disease are the features in the diagnostic criteria above. Some examples are shown in Figure 1.
Figure 1. Key examination
7
Kawasaki disease. (A) bilateral
conjunctivitis without exudate; (B)
strawberry tongue with erythema and
cracking of the lips; (C) erythematous
perineal rash; (D) erythema of the hands;
(E) erythema and oedema of the feet; (F)
desquamation of the hands; (G) erythema
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at the site of previous BCG vaccination;

Natural history

(H) perianal erythema and desquamation.
Kawasaki disease usually follows three phases\:
3
1. Acute phase (1-2 weeks from fever onset)\: high fever, irritability, rash, mucositis, peripheral erythema and oedema.
2. Subacute phase (2-4 weeks from fever onset)\: afebrile, most clinical features begin to resolve, desquamation of the hands
and feet. The highest risk period for developing cardiac complications.
3. Convalescent phase (4-8 weeks from fever onset)\: asymptomatic period, clinical features have resolved. Coronary artery
aneurysms often improve but may get worse.

Di

Fever is a common paediatric presentation. The di
rheumatological conditions. Possible di
1,4
Scarlet fever or acute rheumatic fever (Group A streptococcus)
Toxic shock syndrome or staphylococcal scalded skin syndrome (S t a p h a u r e u s )
Paediatric multisystem inCOVID-19 (PIMS-TS)
Viral exanthems\: erythema infectiosum (parvovirus B19) or infectious mononucleosis (EBV)
Measles
Systemic-onset juvenile idiopathic arthritis
Steven-Johnson syndrome / toxic epidermal necrolysis

Investigations

There is no diagnostic test for Kawasaki disease, and it is a clinical diagnosis. All patients should undergo an
echocardiogram. Other investigations may be useful in suspected incomplete Kawasaki disease or to exclude alternative
diagnoses.

Beside investigations

Relevant bedside investigations include\:
6
ECG\: may show arrhythmias or ST-T changes if myocarditis or myocardial infarction occurs
Urinalysis\: may show sterile pyuria

Laboratory investigations

Relevant laboratory investigations include\:
6
Baseline blood tests (FBC, U&E, LFTs)\: there may be anaemia, raised white cells and raised platelets. LFTs may be
deranged if hepatitis occurs.
ESR and/or CRP\: often signi
Anti-streptolysin O titre (ASOT)\: to exclude group A streptococcal infection
Consider PIMS-TS blood tests if suspected as a di
ferritin).

Imaging

An echocardiogram is essential. It should be performed as soon as Kawasaki disease is suspected but should not delay
treatment (e.g. if the child presents out-of-hours or to a hospital without a suitably quali
Typical
6
Coronary artery aneurysms (as shown in Figure 2)
Valvular disease (e.g. mitral regurgitation)
Coronary artery thrombosis
Poor ventricular function/evidence of myocarditis
Pericardial epericarditis
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The echocardiogram can be normal in the
echocardiograms are needed to monitor disease progression. Their timings depend on the initial
guided by cardiology.
Figure 2. Angiogram showing a left
anterior descending artery aneurysm in a
patient with Kawasaki disease.
8

Management

Children with Kawasaki disease should be admitted to hospital. Early treatment aims to prevent coronary artery aneurysms.
Oral aspirin and intravenous immunoglobulin (IVIG) are the main treatments used.
2

Aspirin

Aspirin is thought to reduce the risk of coronary artery aneurysms and thrombosis through its antiplatelet and anti-
in
Typically, high-dose aspirin is given until the fever has resolved for 48 hours, then low-dose aspirin for approximately 6
weeks (duration will be guided by cardiology).
4
Reye’s syndrome
Aspirin is usually contraindicated in children under 16 due to the risk of Reye’s syndrome, a rare acute
encephalopathy associated with liver failure. However, in Kawasaki disease, the bene
1

Intravenous immunoglobulin (IVIG)

An infusion of IVIG within the
improvement should be seen within 36 hours.
A second dose of IVIG may be given if an improvement is not seen. Other treatments, including corticosteroids or
in
4

Complications

Most children with Kawasaki disease recover well, and the overall mortality rate is \<0.5%. 4
However, complications can
include\:
4
Coronary artery aneurysms\: 15-25% of untreated children develop aneurysms, but the risk is much lower with early
treatment. Giant aneurysms may rupture, causing cardiac tamponade.
Myocarditis/pericarditis
Arrhythmias
Valvular disease (e.g. mitral regurgitation)
Coronary artery thrombosis/myocardial infarction
Sudden cardiac death
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References

Patient UK. K a w a s a k i D i s e a s e . Published 2020. Available from\: [LINK]
Eleftheriou D, Levin M, Shingadia D et al. M a n a g e m e n t o f K a w a s a k i d i s e a s e . Arch Dis Child. 2014;99(1)\:74-83. Available from\:
[LINK]
Rife E and Gedalia A. K a w a s a k i D i s e a s e \: a n U p d a t e . Curr Rheumatol Rep. 2020; 22(10)\:75. Available from\: [LINK]
BMJ Best Practice. K a w a s a k i d i s e a s e . Published 2019, updated 2022. Available from\: [LINK]
National Institute for Health and Care Excellence. N G 1 4 3 \: F e v e r i n u n d e r 5 s \: a s s e s s m e n t a n d i n i t i a l m a n a g e m e n t . Published
2019, updated 2021. Available from\: [LINK]
McCrindle BW, Rowley AH, Newburger JW et al. D i a g n o s i s , T r e a t m e n t , a n d L o n g- T e r m M a n a g e m e n t o f K a w a s a k i D i s e a s e \: A
S c i e n t i
Available from\: [LINK]
Dong Soo Kim. Kawasaki. Licence\: [CC BY-SA 4.0]
mprice18. KawasAngio. Licence\: [CC BY 3.0].

Reviewer

Dr Catriona Anderson
Consultant Paediatric Rheumatologist
University of Edinburgh / NHS Lothian

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Contents

Introduction
Aetiology
Risk factors
Clinical features
Di
Investigations
Management
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