Joint Trust Guideline for the Diagnosis and Management of

Kawasaki Disease in Children

A Clinical Guideline
For Use in: Children’s Assessment Unit, Buxton Ward
By: Medical and nursing staff in Paediatrics
For: Children with Kawasaki disease
Division responsible for document: Paediatrics
Key words: Kawasaki disease
Name of document author: Dr Shastri
Job title of document author: Consultant Paediatrician
Name of document author’s Line
Mary-Anne Morris
Manager:
Job title of author’s Line Manager: Chief of Service for Paediatrics
Dr Kate Armond Consultant Paediatric
Supported by:
Rheumatologist
Clinical Guidelines and Assessment Panel
(CGAP)
Assessed and approved by the:
If approved by committee or Governance Lead
Chair’s Action; tick here 
Date of approval: 09/12/2022
Ratified by or reported as approved
Clinical Safety and Effectiveness Sub-Board
to (if applicable):
To be reviewed before:
This document remains current after this 09/12/2025
date but will be under review
To be reviewed by: Dr Shastri
Reference and / or Trust Docs ID No: 1154
Version No: 6.2
Compliance links: None
If Yes - does the strategy/policy
deviate from the recommendations of N/A
NICE? If so, why?
This guideline has been approved by the Trust's Clinical Guidelines Assessment Panel as an aid to the
diagnosis and management of relevant patients and clinical circumstances. Not every patient or situation fits
neatly into a standard guideline scenario and the guideline must be interpreted and applied in practice in the
light of prevailing clinical circumstances, the diagnostic and treatment options available and the professional
judgement, knowledge and expertise of relevant clinicians. It is advised that the rationale for any departure
from relevant guidance should be documented in the patient's case notes.

The Trust's guidelines are made publicly available as part of the collective endeavour to continuously improve
the quality of healthcare through sharing medical experience and knowledge. The Trust accepts no
responsibility for any misunderstanding or misapplication of this document.

Joint Clinical Guideline for: Diagnosis and Management of Kawasaki Disease in Children
Author/s: Dr Shastri Author/s title: Consultant Paediatrician
Approved by: CGAP Date approved: 09/12/2022 Review date: 09/12/2025
Available via Trust Docs Version: 6.2 Trust Docs ID: 1154 Page 1 of 16
 Joint Trust Guideline for the Diagnosis and Management of
Kawasaki Disease in Children

Version and Document Control:

Version Date of
Change Description Author
Number Update
6.1 19/04/2022 No clinical changes Hannah Massey
Small amendment to Investigations-
Echocardiogram. 6-8 weeks from Dr Rao Kollipara
onset changed to 2 weeks and 6 (Paediatric
6.2 09/11/2022
weeks from onset. Consultant, JPUH),
2 appendices added to end of Dr Shastri
guideline.

This is a Controlled Document

Printed copies of this document may not be up to date. Please check the hospital intranet
for the latest version and destroy all previous versions.

Contents
1. Glossary...................................................................................................................4
2. Quick reference guideline/s...................................................................................5
2.1 Diagnosis and management of Complete Kawasaki Disease in Children............5
2.2 Evaluation of Suspected Incomplete Kawasaki Disease......................................6
3. Rationale..................................................................................................................7
4. Broad recommendations.......................................................................................7
5. Definition..................................................................................................................7
6. Epidemiology..........................................................................................................7
7. Making a diagnosis.................................................................................................7
8. History and Examination........................................................................................8
8.1 Typical or complete cases.....................................................................................8
8.2 Atypical Or “Incomplete Cases”............................................................................9
9. Complications.......................................................................................................10
10. Investigations........................................................................................................10
11. Management..........................................................................................................11
11.1 Definitive treatment.............................................................................................11
11.2 Supportive management.....................................................................................12
11.3 Management of coronary artery aneurysms.......................................................12
11.4 Long term follow up of uncomplicated cases......................................................12
12. Clinical audit standards.......................................................................................13
13. Summary of development and consultation process undertaken before
registration and dissemination...........................................................................13
14. Distribution list / dissemination method............................................................13

Joint Clinical Guideline for: Diagnosis and Management of Kawasaki Disease in Children
Author/s: Dr Shastri Author/s title: Consultant Paediatrician
Approved by: CGAP Date approved: 09/12/2022 Review date: 09/12/2025
Available via Trust Docs Version: 6.2 Trust Docs ID: 1154 Page 2 of 16
 Joint Trust Guideline for the Diagnosis and Management of
Kawasaki Disease in Children
15. References/ source documents..........................................................................13
Appendix 1 - Kawasaki Disease - Information for Parents..........................................14
Appendix 2 – Management of Kawasaki Disease - ADC Document...........................16
Appendix 3 – Fifteen-Minute Consultation: Kawasaki Disease: How to Distinguish
from other Febrile Illnesses: Tricks and Tips – ADC Document.....................16

Joint Clinical Guideline for: Diagnosis and Management of Kawasaki Disease in Children
Author/s: Dr Shastri Author/s title: Consultant Paediatrician
Approved by: CGAP Date approved: 09/12/2022 Review date: 09/12/2025
Available via Trust Docs Version: 6.2 Trust Docs ID: 1154 Page 3 of 16
 Joint Trust Guideline for the Diagnosis and Management of
Kawasaki Disease in Children
1. Glossary
CRP C- reactive protein
ESR Erythrocyte sedimentation rate
UE Urea and electrolytes
FBC Full blood count
LFT Liver function test
WCC White cell count
Echo Echocardiogram
ECG Electrocardiogram
ASOT Antistreptolysin O titre
ALT Alanine transaminase
ANA Antinuclear antibodies
IVIG Intravenous immunoglobulin
EBV Epstein Barr virus
CMV Cytomegalovirus
NSAID Non-steroidal anti-inflammatory drugs
HLH Hemophagocytic Lymphohistiocytosis

Joint Clinical Guideline for: Diagnosis and Management of Kawasaki Disease in Children
Author/s: Dr Shastri Author/s title: Consultant Paediatrician
Approved by: CGAP Date approved: 09/12/2022 Review date: 09/12/2025
Available via Trust Docs Version: 6.2 Trust Docs ID: 1154 Page 4 of 16
 Joint Trust Guideline for the Diagnosis and Management of
Kawasaki Disease in Children
2. Quick reference guideline/s
2.1 Diagnosis and management of Complete Kawasaki Disease in Children
FEATURES OF KAWASAKI DISEASE
Fever > 5 days duration and
Algorithm A Polymorphous rash
Changes in extremities
Bilateral non exudative bulbar conjunctivitis
Changes in lips and oral cavity
Cervical lymphadenopathy
See text for further details

≥4 CRITERIA

Perform Lab tests

 FBC, UE, LFT
 CRP ESR
 Urine microscopy
 Troponin-I

Echo – positive  Echo

Consult Cardiologist  ECG

Consider if the Patient has High Risk Features. This includes

- Already failed IVIG?
- Severe disease: Those under 12 months, those with persistently
elevated CRP despite IVIG treatment, liver dysfunction,
hypoalbuminaemia and anaemia
- Features of HLH of shock
-Already evolving coronary aneurysms with ongoing inflammation, if
known
If in doubt seek expert advice

No High Risk Features

High Risk Features Present
-Start IVIG 2g/kg over 12 hours
-Start IVIG 2g/kg over 12 hours
-Start aspirin 30-50mg/kg/day in four divided
-Start aspirin 30-50mg/kg/day in four divided doses
doses
And
- Consider methylprednisolone 10-30mg/kg over 2-3
hours once daily for 3 days then oral prednisolone
2mg/Kg daily to 7 days and wean over 2-3 weeks.
-Consider discussion with rheumatology and
cardiology

Disease Defervescence  No Disease Defervescence or Disease

No fever for 48 hours, falling crp, child recrudescence
improving clinically  Give second dose of IVIG 2g/kg over 12 hours
-Reduce aspirin to 3-5mg/kg/day  Consider giving methyprednisalone if not already
given
-Patients need a repeat echo at 2 weeks
 Consider discussion with rheumatology and
booked on ice by the physiology
cardiology team
department and at 6 weeks In Dr Shastri’s  Consider other immunosuppressant such as
cardiac clinic infliximab.

Joint Clinical Guideline for: Diagnosis and Management of Kawasaki Disease in Children
Author/s: Dr Shastri Author/s title: Consultant Paediatrician
Approved by: CGAP Date approved: 09/12/2022 Review date: 09/12/2025
Available via Trust Docs Version: 6.2 Trust Docs ID: 1154 Page 5 of 16
 Joint Trust Guideline for the Diagnosis and Management of
Kawasaki Disease in Children

Evaluation of Suspected Incomplete Kawasaki Disease

FEATURES OF KAWASAKI DISEASE

Algorithm Fever > 5 days duration and
B Polymorphous rash
Changes in extremities
Bilateral non exudative bulbar conjunctivitis
Changes in lips and oral cavity
Consider other diagnoses Cervical lymphadenopathy
Scarlet fever See text for details
Viral exanthem
Stevens Johnson Syndrome
Juvenile idiopathic arthritis < 4 CRITERIA
Staphylococcal scalded skin
Toxic shock
No other diagnosis evident,
findings consistent with KD
Consider other investigations
ASOT, anti DNAse B
Throat/nose swab Investigations
Autoantibody, ANA, ferritin FBC, UE, LFT
Viral serology CRP ESR Fever persists
Urine microscopy

CRP<30 Admit child, antipyretics

CRP>30 mg/L
mg/L and review daily
ESR>40
ESR<40
mm/hr
mm/hr

Fever abates, discharge and review CDW or OPD

Supplementary criteria
3 weeks
Albumin <30g/L
Raised ALT
Platelets >450 x 109/L
Anaemia Typical skin peeling No peeling,
Urine microscopy >50 WBC fingers/toes on history or discharge from FU
examination

>3 <3 Echo for coronary artery aneuryms

criteria criteria

POSITIVE NEGATIVE

Manage as Kawasaki Discharge unless other features

Echo and treat suggest ‘missed Kawasaki’
See Algorithm B, with fever resolved, see
Algorithm B, Box 2 consider discussion with Dr
Box 1. Shastri, or paediatric cardiologist

Joint Clinical Guideline for: Diagnosis and Management of Kawasaki Disease in Children
Author/s: Dr Shastri Author/s title: Consultant Paediatrician
Approved by: CGAP Date approved: 09/12/2022 Review date: 09/12/2025
Available via Trust Docs Version: 6.2 Trust Docs ID: 1154 Page 6 of 16
 Joint Trust Guideline for the Diagnosis and Management of
Kawasaki Disease in Children
3. Rationale
Kawasaki disease is an uncommon disease of childhood that is difficult to diagnose,
having many features that overlap with other commoner diseases in childhood. The early
diagnosis and prompt treatment of this condition is essential to reduce mortality and
morbidity associated with cardiovascular complications.

4. Broad recommendations
1. Kawasaki disease requires a high degree of clinical vigilance as
 It is rare.
 It is diagnosed on clinical criteria rather than diagnostic interventions.
 It has potentially life-threatening complications, including coronary artery vasculitis
with aneurysm formation.
 Treatment is available but must be given early if it is to be effective.

2. A Consultant should assess the child before commencing treatment with IVIG and
aspirin.

5. Definition
Kawasaki disease is an acute self-limiting vasculitis of unknown aetiology that occurs
predominantly in infants and young children.

It affects large-medium vessels and is characterised as an acute febrile illness

associated with features of systemic vasculitis.

6. Epidemiology

a) Incidence: varies considerably worldwide from 3.4 -100 per 100,000 children.
b) Peak age of onset: 1-3 years but can occur in infancy or in later
childhood/adolescence
c) Risk factors:
 M>F 1.5:1
 Race, East Asian > Asian > Black > Caucasian
 Later winter/ early spring peak
d) Pathogenesis: remains unknown although clinical and epidemiological features
strongly suggest an infectious cause
e) Prognosis: In the absence of therapy 15-25% will develop coronary artery
aneurysms which may lead to ischaemic heart disease and sudden death. This
risk is reduced to < 5% with timely use of IVIG (within 10 days of onset of fever)

7. Making a diagnosis
In the absence of a diagnostic test, making a diagnosis depends on recognition of key
clinical features and the exclusion of alternative diagnoses with similar presentations.

Joint Clinical Guideline for: Diagnosis and Management of Kawasaki Disease in Children
Author/s: Dr Shastri Author/s title: Consultant Paediatrician
Approved by: CGAP Date approved: 09/12/2022 Review date: 09/12/2025
Available via Trust Docs Version: 6.2 Trust Docs ID: 1154 Page 7 of 16
 Joint Trust Guideline for the Diagnosis and Management of
Kawasaki Disease in Children
8. History and Examination
8.1 Typical or complete cases
Fever of at least five days duration (defined as recorded temperature of 38 degrees or
above or tactile temperature by parent) and 4 of 5 classical features from the following list

I. Changes in extremities
 Acute: Erythema of palms, soles; oedema of hands, feet
 Subacute: Periungual peeling of fingers and toes in weeks 2 and 3.
 Chronic: Deep transverse grooves (Beau’s lines) may appear across the nails after
1-2 months of onset of fever.

II. Polymorphous rash - the rash may take various forms:

 Nonspecific diffuse maculopapular eruption
 Urticarial exanthem
 Scarlatiniform rash
 Erythroderma
 Erythema multiforme-like rash
 Micropustular eruption (rare)
 It is always non- bullous and non-vesicular
 The rash is usually extensive with the involvement of the trunk and extremities and
accentuation in the perineal region, where early desquamation may occur

III. Bilateral bulbar conjunctivitis without exudate

 Spares the limbus (pale ring immediately surrounding pupil)
 Painless
 Mild iridocyclitis or anterior uveitis may be noted by slit lamp; it resolves rapidly
and rarely associated with photophobia or eye pain

IV. Changes in lips and oral cavity

 Erythema, dryness, fissuring, peeling, cracking and bleeding of lips
 Strawberry tongue with erythema and prominent fungiform papillae
 Diffuse erythema of the oropharyngeal mucosa

V. Cervical lymphadenopathy
 Least common, usually unilateral

Even in the presence of the above, it remains important to exclude other conditions with
similar findings which include:
 Viral exanthems (e.g. measles, adenovirus, enterovirus, EBV)
 Scarlet fever

Joint Clinical Guideline for: Diagnosis and Management of Kawasaki Disease in Children
Author/s: Dr Shastri Author/s title: Consultant Paediatrician
Approved by: CGAP Date approved: 09/12/2022 Review date: 09/12/2025
Available via Trust Docs Version: 6.2 Trust Docs ID: 1154 Page 8 of 16
 Joint Trust Guideline for the Diagnosis and Management of
Kawasaki Disease in Children
 Staphylococcal scalded skin syndrome
 Stevens-Johnson syndrome
 Juvenile idiopathic arthritis (systemic onset)
 Toxic shock syndrome
 Drug hypersensitivity reactions

8.2 Atypical Or “Incomplete Cases”

These have fewer diagnostic features. They can however still develop complications, so
making the diagnosis remains very important. Where fewer than 4 features are present, a
high degree of clinical vigilance is required. The following less common clinical findings
involving other systems should be looked for.

System Finding
Extreme irritability
Aseptic meningitis
Neurological
Sensorineural hearing loss – transient
during acute phase
Musculoskeletal Arthritis and arthralgia, myositis
Diarrhoea, vomiting and abdominal pain
Hepatitis
Gastrointestinal tract Acute acalculous distension of gallbladder
(hydrops) - during first 2 weeks of illness
may be identified by ultrasound scan
Urethritis, meatitis
Genitourinary
Sterile pyuria
Erythema, induration of BCG inoculation
site
Other
Mild anterior uveitis (slit lamp examination
required)

It is also important to remember common diagnostic pitfalls, including

 Rash and mucosal changes may be mistaken for drug reactions
 Sterile pyuria may be mistaken for a partially treated UTI
 CSF pleocytosis may be mistaken for viral meningitis

Supportive evidence should be sought through investigations (see section below).

In addition to incomplete case the diagnostic criterion of a fever for 5 days can also lead
to a delay in treatment. Clinicians should not delay in making a diagnosis of Kawasaki
disease and instituting treatment if:
 5/6 diagnostic criteria of Kawasaki are present before day 5 of fever.
 CAA or coronary dilatation are present.
 There is evidence of persistent elevation of inflammatory markers with no other
explanation in patients where there remains clinical suspicion of Kawasaki’s

Joint Clinical Guideline for: Diagnosis and Management of Kawasaki Disease in Children
Author/s: Dr Shastri Author/s title: Consultant Paediatrician
Approved by: CGAP Date approved: 09/12/2022 Review date: 09/12/2025
Available via Trust Docs Version: 6.2 Trust Docs ID: 1154 Page 9 of 16
 Joint Trust Guideline for the Diagnosis and Management of
Kawasaki Disease in Children
9. Complications
Serious complications are rare but include

Acute phase (<10 days from onset)

 Myocarditis +/- endocarditis
 Pericarditis +/- effusion/tamponade
 Valvular incompetence

Subacute phase
 Coronary artery vasculitis – dilatation +/- aneurysms
o First detectable 10 days
o Peak onset 3-4 weeks
 Coronary artery thrombosis and stenosis
 Systemic large vessel vasculitis (1-2%)
o Cerebral
o Subclavian, axillary, brachial aneurysms
o Peripheral ischaemia/gangrene

10. Investigations
In the presence of less than 4 features of Kawasaki Syndrome, these are useful to aid
the decision to treat ‘incomplete’ disease (see Algorithm A).

i. Bloods
Full blood count
 Mild anaemia
 Leukocytosis with neutrophilia and immature forms
 Thrombocytosis after week 1, peaks in the third week, normalises by 4-8 weeks

Urea and electrolytes, liver function, plasma lipid profile

 Hyponatremia
 Hypoalbuminaemia
 Elevated serum transaminases
 Elevated gamma glutamyl transpeptidase
 Abnormal plasma lipids
 Raised Troponin-I

ii. Urine microscopy and culture

 Sterile pyuria

iii. Others – Pleocytosis of cerebrospinal fluid

Joint Clinical Guideline for: Diagnosis and Management of Kawasaki Disease in Children
Author/s: Dr Shastri Author/s title: Consultant Paediatrician
Approved by: CGAP Date approved: 09/12/2022 Review date: 09/12/2025
Available via Trust Docs Version: 6.2 Trust Docs ID: 1154 Page 10 of 16
 Joint Trust Guideline for the Diagnosis and Management of
Kawasaki Disease in Children

iv. Echocardiogram - should be arranged as soon as possible ideally by Dr Shastri,

however, treatment should not be deferred.
 In acute phase – if positive for coronary artery aneurysm, repeat echo after 2-3
days to monitor progression
 2 weeks and 6 weeks from onset
 12 months from onset

v. ECG – should be done at presentation

 Prolonged PR interval
 Non specific ST and T wave changes
 Arrhythmias

Investigations to exclude/ confirm alternative diagnosis may be considered (discuss with

consultant)
 ASOT and anti-DNAase B
 Blood culture, urine culture
 Nose/throat/skin swab
 Lumbar puncture
 Coagulation screen
 ANA, Rheumatoid factor, ferritin
 Serology for EBV, mycoplasma, CMV, parvovirus, measles etc.

11. Management
11.1 Definitive treatment
Children should be reviewed by a consultant before starting definitive treatment.
Treatment should be started for.
 All children meeting criteria for complete or typical Kawasaki disease without
alternative diagnosis
 All children with sufficient clinical criteria to suspect Kawasaki disease and where
laboratory data is supportive in absence of a definitive alternative diagnosis. (See
Algorithm B)

Treatment should be started within 10 days of the onset of symptoms and if possible,
within 7 days to be most effective, but can be started beyond 10 days in the presence of
ongoing signs (and/or laboratory evidence) of inflammation.

i. First line treatment

 Intravenous immunoglobulin (IVIG) 2 gram/kg IV as single infusion over 12 hours.
IVIG request form to be completed and emailed as an attachment to Colin Green,
D & T Pharmacy Advisor.

Joint Clinical Guideline for: Diagnosis and Management of Kawasaki Disease in Children
Author/s: Dr Shastri Author/s title: Consultant Paediatrician
Approved by: CGAP Date approved: 09/12/2022 Review date: 09/12/2025
Available via Trust Docs Version: 6.2 Trust Docs ID: 1154 Page 11 of 16
 Joint Trust Guideline for the Diagnosis and Management of
Kawasaki Disease in Children
 Aspirin – 30-50mg/kg/day in 4 divided doses. All other NSAIDs should be stopped.
This dose should be continued until the child has been afebrile for >48-72 hours or
for a maximum of 14 days

Thereafter aspirin to continue at 3-5mg/kg/day once daily until review of echocardiogram

6-8 weeks after onset of illness.

NOTE: Corticosteroids have been used as initial therapy or in conjunction with IVIG in the
treatment of Kawasaki disease. However, there is little evidence to support steroids first
line use in most children with Kawasaki disease. It may be appropriate to consider it
along side IVIG for children who have high risk features as shown in algorithm B. This
should be discussed with a specialist.

ii. Second line treatment

Approximately 10% of patients with Kawasaki disease fail to respond with initial IVIG
therapy. Failure to respond usually is defined as persistent or recrudescent fever > 36
hours after completion of initial IVIG infusion.
 Repeat IVIG 2gram/kg as a single infusion over 10-12 hours
 Consider Methylprednisolone 10 (up to maximum 30) mg/kg over 2-3 hours once
daily for 3 days, followed by 2mg/Kg prednisolone for 4 days, weaning over 2-3
weeks
 Discuss with paediatric rheumatology team. The local link is Dr Shastri, if he is
unavailable, discuss with Dr Bale or Dr Armon (paediatric rheumatology team at
Addenbrookes Hospital). If they are unavailable the case can be discussed with
the Great Ormond Street hospital infectious disease team.
 Discuss with Cardiologist at Great Ormond Street Hospital (#6111)

11.2 Supportive management

 Fluid balance to be maintained and monitored closely
 Regular antipyretics/analgesics, all NSAIDs to be stopped when aspirin is
commenced
 Consider need for lansoprazole / omeprazole with aspirin treatment, and definitely
treat with PPI if aspirin and steroids given

11.3 Management of coronary artery aneurysms

 If echocardiogram shows coronary artery aneurysms, the Cardiologist should be
contacted. These children will need long term aspirin therapy
 Children taking long term salicylates should receive annual influenza vaccine
 Patients who require long term aspirin due to CCA should be considered for
varicella zoster virus vaccine. In view of the association between VZV, aspirin and
Reye’s syndrome.

11.4 Long term follow up of uncomplicated cases

 If echocardiogram is normal at 6-8 weeks, then aspirin can be stopped. Repeat
echocardiogram in 12 months

Joint Clinical Guideline for: Diagnosis and Management of Kawasaki Disease in Children
Author/s: Dr Shastri Author/s title: Consultant Paediatrician
Approved by: CGAP Date approved: 09/12/2022 Review date: 09/12/2025
Available via Trust Docs Version: 6.2 Trust Docs ID: 1154 Page 12 of 16
 Joint Trust Guideline for the Diagnosis and Management of
Kawasaki Disease in Children
 Children who have received IVIG should not receive live vaccines for at least three
months.

12. Clinical audit standards

All children with suspected Kawasaki disease should be treated with IVIG within 24 hours
of a consultant confirmed diagnosis.

13. Summary of development and consultation process undertaken before

registration and dissemination
The guideline was drafted by the authors listed above and circulated among the
Paediatric Consultants and other medical staff, who have agreed the final content.

The document was reviewed in 2022 and not clinical changes were required but a short
review date given because a new appendix for nurses is being developed.

This version has been endorsed by the Clinical Guidelines Assessment Panel.

14. Distribution list / dissemination method

Childrens Assessment Unit, Buxton ward

15. References/ source documents

1. Newburger JW et al: Diagnosis, Treatment, and Long-Term Management of

Kawasaki Disease: A Statement for Health Professionals from the Committee on
Rheumatic Fever, Endocarditis and Kawasaki Disease, Council on Cardiovascular
Disease in the Young, American Heart Association Pediatrics 2004;114;1708-1733
2. Baumer JH:Kawasaki Disease: what to do with incomplete cases? Arch Dis Child
Pract Ed 2005; 90:ep102-ep 104
3. Oates-Whitehead RM, Baumer JH, Haines L, Love S, Maconochie IK, Gupta A,
Roman K, Dua JS, Flynn I. Intravenous immunoglobulin for the treatment of
Kawasaki disease in children. Cochrane Database of Systematic Reviews 2003,
Issue 4. Art. No:CD004000. DOI: 10.1002/14651858.CD004000
4. Furukawa et al Effects of steroid pulse therapy on immunoglobulin-resistant
Kawasaki disease. Arch Dis Child 2008 Feb;93(2):142-6.
5. Eleftheriou D, Levin M, Shingadia D, etal. Management of Kawasake disease.
Archives of Disease in Childhood 2014;99:74-83.

Also see the Patient Information leaflet (Appendix one)

Joint Clinical Guideline for: Diagnosis and Management of Kawasaki Disease in Children
Author/s: Dr Shastri Author/s title: Consultant Paediatrician
Approved by: CGAP Date approved: 09/12/2022 Review date: 09/12/2025
Available via Trust Docs Version: 6.2 Trust Docs ID: 1154 Page 13 of 16
 Joint Trust Guideline for the Diagnosis and Management of
Kawasaki Disease in Children
Appendix 1 - Kawasaki Disease - Information for Parents
What is Kawasaki disease?
Kawasaki disease is an illness which affects children’s blood vessels, causing them to be
inflamed (red, hot, swollen) It affects young children the most (80% of children are under
5 years of age). It is very rare in this country, affecting 150-200 children per year in the
UK. Boys are more likely to have this illness than girls.

What causes Kawasaki disease?

Although the cause is not yet fully understood, it is thought to result from an infection.
What are the symptoms of Kawasaki Disease?
The main symptom is fever that is higher than 38 degrees centigrade, lasting for at least
5 days. The other symptoms are:
 Soreness of the mouth and lips.
 Reddening of the tongue with raised bumps (strawberry tongue).
 Rashes on the body, hands and feet.
 Swelling of the hands and feet.
 Reddening of the eyes.
 Swollen glands in the neck.
 Significant discomfort and irritability in young children.
Once the fever improves, the red eyes and swollen glands will disappear. However,
during the third week of illness the skin around the hands and feet may peel.
Are there any complications of Kawasaki disease?
Most children make a full recovery without any problems but for some children there is a
risk of developing swelling or ‘ballooning’ of the blood vessels of the heart (termed
coronary aneurysms). This may cause other heart problems. The risk of aneurysms is
reduced if children are treated within 10 days of the start of the illness.
What tests will your child undergo in hospital?
There is no single test for Kawasaki disease; however, some tests are carried out to help
to confirm the diagnosis. These are:
 Blood tests - to look for signs of anaemia, raised markers of inflammation and
increased platelet count (cell which helps in blood clot formation in blood vessels)
 ECG (electrocardiogram) - to look for any abnormalities of heart rhythm.
 Echocardiogram (heart scan or echo) - To look for how well the heart is
functioning and for changes in the blood vessels in the heart (coronary artery
aneurysms)
What is the treatment for Kawasaki disease?
As soon as this illness is suspected, your child will begin a course of treatment including:
 An intravenous dose of immunoglobulin (a purified human blood plasma product)
given in a drip over 12 hours. This will reduce the inflammation and if started
within 10 days of the onset of illness, helps to prevent coronary artery damage.
 Aspirin given every 6-8 hours (to prevent clot formation in the coronary arteries)
Most children will respond to this treatment and their fever should improve within
48 hours.

Joint Clinical Guideline for: Diagnosis and Management of Kawasaki Disease in Children
Author/s: Dr Shastri Author/s title: Consultant Paediatrician
Approved by: CGAP Date approved: 09/12/2022 Review date: 09/12/2025
Available via Trust Docs Version: 6.2 Trust Docs ID: 1154 Page 14 of 16
 Joint Trust Guideline for the Diagnosis and Management of
Kawasaki Disease in Children
What happens after the first two days of treatment?
If your child does not have any further fever, then the following will change:
 The aspirin dose will be reduced to once daily.
 Your child will be discharged when their general condition improves.
 An echocardiogram will be repeated after 6-8 weeks, if this is normal then the
aspirin will be stopped.
 A further echocardiogram will be done after 1 year.

What happens if the treatment does not work?

If your child has persistent fever within the first 48 hours of treatment, then your medical
team will:
 Give another dose of intravenous immunoglobulin.
 They may add steroid medication (IV and / or oral).
 Consider consulting a cardiologist (Heart Specialist) for further advice.

What happens if there are problems seen on the echocardiogram?

If there are any signs of swelling of the coronary arteries or any signs that the heart is not
functioning as well as it should, your medical team will consult a paediatric cardiologist
(children’s heart specialist) for further advice. Your child may need further tests to look at
heart function and may have to have long term treatment with aspirin.

What happens after discharge from hospital?

Following discharge, you may notice that your child is tired and does not eat well for
nearly 2 weeks. This is quite normal, and most children make a full recovery. However, if
your child has any of the following symptoms please contact the number below:
 Shallow, rapid breathing.
 Stomach pains.
 Vomiting (with or without blood).
 Return of fever or other symptoms of Kawasaki disease.
It is recommended that your child has the flu vaccine if they are on long term aspirin
treatment, this can be arranged with your GP. Please contact them directly to discuss
this. Your child will also receive a follow up appointment in the children’s outpatient clinic
in approximately 6-8 weeks. This will be to discuss your child’s progress with a consultant
paediatrician or senior paediatric trainee (doctors who are expert in the care of children).

How to contact the Jenny Lind Children’s Department

 There is a doctor and nurse available in the department 24 hours a day 7 days a
week in the Children’s Assessment Unit on: 01603 289774.
 For outpatient appointment enquiries please contact: 01603 287055.
 For questions about your child’s medication please contact: 01603 286286 and
ask for the Pharmacy Helpline
 The main number for the hospital is: 01603 286286

Joint Clinical Guideline for: Diagnosis and Management of Kawasaki Disease in Children
Author/s: Dr Shastri Author/s title: Consultant Paediatrician
Approved by: CGAP Date approved: 09/12/2022 Review date: 09/12/2025
Available via Trust Docs Version: 6.2 Trust Docs ID: 1154 Page 15 of 16
 Joint Trust Guideline for the Diagnosis and Management of
Kawasaki Disease in Children

Appendix 2 – Management of Kawasaki Disease - ADC Document

Appendix 3 – Fifteen-Minute Consultation: Kawasaki Disease: How to

Distinguish from other Febrile Illnesses: Tricks and Tips – ADC Document

Joint Clinical Guideline for: Diagnosis and Management of Kawasaki Disease in Children
Author/s: Dr Shastri Author/s title: Consultant Paediatrician
Approved by: CGAP Date approved: 09/12/2022 Review date: 09/12/2025
Available via Trust Docs Version: 6.2 Trust Docs ID: 1154 Page 16 of 16