Kawasaki Disease

Question 1

What is Kawasaki disease?

Answer 1

Kawasaki disease is also known as mucocutaneous lymph node syndrome. It is a systemic, medium-sized vessel vasculitis.

Question 2

Who is commonly affected in Kawasaki disease?

Answer 2

It affects young children, typically under 5 years. There is no clear cause or trigger. It is more common in Asian children, particularly Japanese and Korean children. It is also more common in boys.

Question 3

What is a key complciation of Kawasaki disease?

Answer 3

A key complication is coronary artery aneurysm.

Question 4

What are the clinical features of Kawasaki disease?

Answer 4

A key feature that should make you consider Kawasaki disease is a persistent high fever (above 39ºC) for more than 5 days. Children will be unhappy and unwell. The key skin findings are a widespread erythematous maculopapular rash and desquamation (skin peeling) on the palms and soles.

Other features include:

• Strawberry tongue (red tongue with large papillae)
• Cracked lips
• Cervical lymphadenopathy
• Bilateral conjunctivitis

Question 5

What investigations should be ordered for Kawasaki disease?

Answer 5

There are several investigations that can be helpful in Kawasaki disease:

• Full blood count can show anaemia, leukocytosis and thrombocytosis
• Liver function tests can show hypoalbuminemia and elevated liver enzymes
• Inflammatory markers (particularly ESR) are raised
• Urinalysis can show raised white blood cells without infection
• Echocardiogram can demonstrate coronary artery pathology

Question 6

Briefly describe the disease course of Kawasaki disease

Answer 6

There are three phases to Kawasaki disease:

• Acute phase
  
  • The child is most unwell with the fever, rash and lymphadenopathy
  • This lasts 1 – 2 weeks
• Subacute phase
  
  • The acute symptoms settle, the desquamation and arthralgia occur and there is a risk of coronary artery aneurysms forming
  • This lasts 2 – 4 weeks
• Convalescent stage
  
  • The remaining symptoms settle, the blood tests slowly return to normal and the coronary aneurysms may regress
  • This last 2 – 4 weeks

Question 7

Briefly describe the management of Kawasaki disease

Answer 7

There are two first line medical treatments given to patients with Kawasaki disease:

• High dose aspirin to reduce the risk of thrombosis
• IV immunoglobulins to reduce the risk of coronary artery aneurysms

Question 8

What monitoring is required following Kawasaki disease?

Answer 8

Patients will need close follow up with echocardiograms to monitor for evidence of coronary artery aneurysms.

Question 9

Why is aspirin usually avoided in children?

Answer 9

Reye’s syndrome.