Kawasaki Disease

Question 1

A 2 year old boy is brought to the ED with high fevers for 5 days, bilateral conjunctivitis, erythema of his lips and oral mucosa, rash, palpable lymph nodes at the neck

Answer 1

Impression
This constellation of symptoms is in keeping with Kawasaki disease;
CRASH and burn;
C - conjunctivitis (bilateral, non-purulent)
R - Rash
A - Adenopathy (cervical lymph nodes)
S - Strawberry tongue
H - Hands and feet hyperaemia and painful oedema that progresses to desquamation (perianal desquamation is associated)
Burn - Fever persisting for 5 days

Concerned about complications including coronary aneurysms.

DDx to consider;
- infectious: GAS, meningitis, sepsis, toxic shock syndrome, EBV, PIMS-TS/MIS-C post-COVID-19 infection, hand-foot-mouth disease
- inflammatory: juvenile idiopathic arthritis
- SJS/TENS and other cutaneous drug reactions
- The six rashes
o rubella - rash, desquamation
o scarlett - GAS: rash, fever, strawberry
o Measles -
o SSSS -
o parvovirus - slapped cheeck, fe
o roseola: fever, rash

Goals
- ensure HD stability
- initiate definitive/supportive treatment

Question 2

Kawasaki disease - Assessment

Answer 2

Assessment
- A to E assessment to ensure HD stability, systemic evidence of infection

Question 3

Kawasaki disease - History

Answer 3

History
- sx: CRASH and Burn (fever for 5 days); conjunctivitis, rash, adenopathy, strawberry tongue, hand and foot skin involvement, preceding viral/bacterial illness. Recent COVID infection.
- other associated: arthritis, meningitis, sterile pyuria and dysuria, other non-specific sx like Headache/nausea/vomiting
- exclude differentials: recent medications
- RISKS: <5 years, male gender, fam hx of Kawasaki
- paeds history (development, blue book, vaccinations)

Question 4

Kawasaki disease - Examination

Answer 4

Examination
- General appearance + vitals
- Derm:
o polymorphous rash, typically begins with perianal erythema; desquamation in hand and foot distribution
o oral mucosa: cracked lips, strawberry tongue
o eyes: conjunctivitis, usually bilateral
- Lymph nodes: cervical lymphadenopathy (typically cervical)

Question 5

Kawasaki disease - Investigations

Answer 5

Investigations
Is primarily a clinical diagnosis.
- conduct septic screen

Ix to rule out DDX
- bedside: UA, ECG (arrhythmias)
- Bloods: FBC (WCC), ESR/CRP, UEC (hyponatraemia), LFT (raised ALT), blood cultures for differentials
- Imaging: ECHO to identify cardiac complications

Question 6

Kawasaki disease - Management

Answer 6

Management
All patients with suspected Kawasaki require admission for inpatient treatment

Definitive
- IV IG 2g/kg as a single IV infusion
- Aspirin: 3-5mg/kg OD until normal ECHO, or for 6 weeks
o consider risk of Reye’s syndrome
- Corticosteroids: controversial, but use in high-risk patients
- lifelong warfarin for those with coronary aneurysms

Ongoing monitoring for complications (cardiac with coronary aneurysms, ventricular dysfunction), Kawasaki disease shock syndrome

Supportive
- Discharge on daily aspirin and repeat ECHO 6 weeks after initial treatment
- May require indefinite cardiac monitoring and follow-up