Kawasaki Disease

Question 1

What is Kawasaki?

Answer 1

AI vasculitis

Question 2

Epidemiology: age? race?

Answer 2

• Predominantly in children <5 years

- Esp Asian children (esp. Japanese)

Question 3

Dx criteria for Kawasaki

Answer 3

4 out of 5 of CRASH and Burn:
- Conjunctivitis - bilateral, non-purulent
- Rash - polymorphous: vesicular/petechial less likely
- Adenopathy (cervical): unilat, >1.5cm (large!), non-purulent, painful
- Strawberry tongue
- Hand swelling/erythema

- and Burn: 5 days of daily fevers (typically Rx-res)

• NB: not all criteria necessary at same time, and sometimes are not fully met in those with the disease.

Question 4

Clinical features of Kawasaki aside from Dx criteria

Answer 4

• General: extreme irritability, arthritis
• Derm: bright red, cracked lips
• Neuro/ENT: aseptic meningitis, OM
• GIT: diarrhoea, mild hepatitis GB hydrops
• Renal: sterile pyuria
• Peripheral
  
  • Hands and soles erythema -> desquamation
  • Early desquamation at periungual region
• Other sign - “BCG sign”: redness and ulceration at site of vaccine often first sign

Question 5

Why is Dx often delayed with kawasaki?

Answer 5

Looks like many viral exanthems at the beginning

Question 6

What Ix for kawasaki (remembering classically clinical Dx anyway)?

Answer 6

• ASOT / Anti DNAase B
• Echo (at least twice: at initial presentation and, if negative, again at 6 - 8 weeks).
  
  • Often do early ECG because cardiologists don’t want to do the early echo
• Platelet count (marked thrombocytosis common in second week of illness)
• Other FBE: neutrophilia, anaemia

Question 7

What is a serious complication of kawasaki, and which age is more likely to develop it?

Answer 7

coronary artery aneurysm(25% if untreated - 2-4% if treated) -> AMI -> death

- Smaller aneurysm may present after weeks/months
- Giant aneurysms present early 
- Infants < 12m

Question 8

Rx for kawasaki

Answer 8

• high-dose aspirin (6-8 weeks)
  
  • anti-inflamm - anti-thrombotic doses
  • 3 - 5 mg/kg once a day for at least 6 to 8 weeks i.e. higher dose, more prolonged course

• intravenous immunoglobulin
• 2 g/kg over 10 hours
• Check effect by same/next day echo
• Fever should defervesnce within 24h of IVIG
preferably within the first 10 days of the illness but should also be given to patients diagnosed after 10 days of illness if there is evidence of ongoing inflammation - eg fever, raised ESR/CRP

Question 9

F/U for kawasaki

Answer 9

• Annual echos (some say for 6-8 years, some say for lifetime)

Question 10

List some DDx for kawasaki

Answer 10

• Staph infection e.g. scalded skin syndrome, TSS
• Strep infection e.g. scarlet fever
• Measles
• viral exanthems
• SJS
• Drug reaction
• Juvenile RA