Kawasaki Disease



Background

Kawasaki disease (KD) is a disease of exclusion and the diagnosis and treatment of possible cases must be discussed with senior medical staff

Diagnosis

There is no diagnostic test and diagnosis is based on clinical criteria and the exclusion of other diseases. Infection must be considered. The criteria may present sequentially such that an ‘incomplete’ case can evolve with time to become ‘complete’. This makes the definite exclusion of Kawasaki disease (KD) difficult and the disease should be considered in any irritable child with a fever for 5 or more days.

Diagnostic Criteria

Fever >five days plus 4 of the following:

  • Conjunctivitis: non-exudative
  • Cervical lymphadenopathy - > one >1.5cm node
  • Rash
  • Changes to lips or oral mucosal - erythema or cracking or swelling of lips, strawberry tongue
  • Changes of extremities - erythema and/or painful oedema and /or desquamation from the 2nd week

Atypical KD has a similar morbidity, thus senior advice should be sought in cases with 2 or more features of KD.


Differential Diagnosis

  • Toxic Shock Syndrome
  • Scalded skin syndrome
  • Scarlet fever
  • EBV, CMV, Mycoplasma
  • Adenovirus, SARS-CoV-2, measles
  • Stephen-Johnson syndrome
  • Drug reaction
  • Polyarteritis nodosa
  • Juvenile idiopathic arthritis
  • Malignancy e.g. lymphoma

Initial investigations

KD is associated with many non-specific laboratory findings.

  • Acute phase proteins raised
  • Neutrophilia, ESR raised
  • Thrombocytosis towards the end of the second week and therefore is not useful diagnostically
  • LFTs may be deranged
  • Pyuria, CSF pleocytosis

Other investigations

  • FBC and Film
  • ESR,CRP
  • Renal profile
  • LFT
  • Coagulation
  • Autoimmune profile

Other investigations

  • ASOT, anti DNA serology (mycoplasma, enterovirus, adenovirus, measles, parvovirus, EBV, CMV)
  • Blood Cultures
  • Urine MC&S
  • ECG and echocardiogram
  • Consider CXR

Consider


Treatment

Aspirin

  • Given during the acute phase of the illness at 30-50 mg/kg/day until afebrile

Immunoglobulin

  • Early recognition and treatment with IVIG has been shown to reduce the occurrence of coronary artery aneurysms
  • For maximum benefit it should be given before day 10 of the illness but should not be withheld if Dx after this time
  • If you suspect KD then it should be treated regardless of what the echo shows
  • Recommended dose is 2g/Kg over 12 hours except where there is cardiac compromise when a smaller volume in divided doses may be preferable

Steroids

  • No steroids unless x2 fail of IVIG

Mx algorithm


References

Brogan PA, Bose A, Burgner D et al Kawasaki disease:an evidence based approach to diagnosis, treatment, and proposals for future research. Arch Dis Child 2002; 86:286-290


Content by Dr Sarah Bridges, Dr Íomhar O' Sullivan. Last review Dr ÍOS 16/06/21.