Kawasaki Disease

Last updated 20.09.12

 

Index

 

Definition

-  Large, medium & small vessel vasculitis, esp coronary arteries

 

Features

-  Large, medium & small vessel vasculitis, esp coronary arteries

-  Children <4 yrs

-  Associated w mucocutaenous lymph node syndrome

-  Most important cause of acquired cardiac disease in children

 

Dx

-  5/7 fever + 4+ of

-  Bilateral conjunctival injection (non-exudative)

-  Injected or fissured lips or pharynx

-  Strawberry tongue

-  Erythema of palms/soles, hands/feet

-  Periunguinal desquamation

-  Cervical lymphadenopathy >1.5cm (usually unilateral)

 

Assocaited S/S

-  As above +

-  Coronary aneurysms

 

Ix

-  Echo

 

Mx

-  IgG 2g/kg IV (8-12hr infusion)

-  Reduces development of coronary aneurysms

-  Repeat dose if fever persists >36hrs

-  If persists after x2 doses --> Methylpred 30mg/kg

-  Aspirn

-  High dose initial 30mg/kg/day to 100mg/kg/day divided into 4 doses

-  Until 48hrs post resolution of fever

-  Low dose maintenance 3-5mg/kg PO Daily

-  Up to 3 months duration

 

 

 

References

 

Therapeutic  Guidelines – Vasculitis in Children 2010