Kawasaki disease

Question 1

What is Kawasaki disease?

Answer 1

A medium size vessel vasculitis

Question 2

Which age group does Kawasaki affect most?

Answer 2

Children under 5 years

Question 3

Which ethnic group is most affected by Kawasaki?

Answer 3

Asian (esp. Chinese and Japanese)

Question 4

What is the incidence of Kawasaki in Japan?

Answer 4

243.1 and 264.8 cases per 100,000 children aged under and over 5 respectively

Question 5

What is the incidence of Kawasaki in Europe?

Answer 5

5-10 per 100,000 children aged under 5

(highest incidence in Ireland at 15.2 per 100,000 children aged under 5)

Question 6

TRUE OR FALSE

Kawasaki affects girls more than boys

Answer 6

FALSE

M>F

Question 7

What is the aetiology of Kawasaki’s?

Answer 7

Idiopathic but believed to be from infective causes

Question 8

Describe the pathogenesis of Kawasaki’s

Answer 8

Start with an infection - e.g., staph or strep

Involves monocytes, IgA, neutrophils and CD8+ cells - mounts an immune response

Immune response can damage collagen and elastic fibres in vessel walls causing structural abnormalities

Can cause aneurysms

Question 9

What are the signs of Kawasaki’s?

Answer 9

Must have fever > 38 degrees Celsius

AND one of the following
(CREAM)
Conjunctivitis (bilateral)
Rash - polymorphous/maculopapular
Erythema, oedema and/or desquamation of hands and feet
Adenopathy - especially cervical lymphadenopathy
Mucosal changes - e.g., strawberry tongue

Question 10

Name DDx for other conditions that can present with a similar rash to Kawasaki’s

Answer 10

Measles – exudate conjunctivitis, koplic spots, rash from head -> trunk

Drug reaction – angioedema, maculopapular rash, urticaria

Streptococcus pharyngitis – scarlet fever, rheumatic fever

Staphylococcus infection – staphylococcus scalded skin syndrome – blisters, toxic shock syndrome

Viral rash

Stevens Johnson syndrome

Question 11

What investigation would you do for a child suspected of having Kawasaki’s?

Answer 11

FBC

ESR

Us&Es

LFT

CRP

Urinalysis

Echocardiogram

Question 12

When should an echocardiogram be done for Kawasaki’s and why?

Answer 12

once diagnosis of Kawasaki has been made

6-10 weeks after

to check for myocardial inflammation and coronary artery aneurysms (CAA)

Question 13

How should Kawasaki’s be managed?

Answer 13

IVIG – within 10 days reduces complications

Aspirin used initially during the fever stage and then switched to antiplatelets

Monitor cardiac function

Question 14

Which class of drug must NOT be prescribed to a child with Kawasaki’s and why?

Answer 14

Corticosteroids

contraindicated as they increase the risk of aneurysms