Kawasaki disease/mucocutaneous lymph node syndrome

M30.3

DESCRIPTION

Kawasaki disease is an acute systemic vasculitis of unknown aetiology occurring predominantly in children. It involves the small and medium arteries. Most serious complication is coronary artery aneurysms.

DIAGNOSTIC CRITERIA

Clinical

  • There is no diagnostic test.
  • Confirm diagnosis by the presence of fever for ≥ 5 days, lack of another known disease process to explain the illness and the presence of 4 of the 5 criteria listed below:
  1. bilateral bulbar conjunctival injection without exudates;
  2. changes of the lips and oral cavity: reddening of the oral mucosa, pharynx, lips, strawberry tongue, cracking of lips;
  3. polymorphous rash, primarily on the trunk;
  4. cervical lymphadenopathy (lymph nodes >1.5 cm diameter);
  5. changes of the extremities, including reddening of the palms and soles, oedema of the hands and/or feet and desquamation of the finger tips and toes.
  • A high index of suspicion is required especially in younger children who may present without all the above or may have incomplete/atypical Kawasaki.
  • Important differential diagnosis:
    • aseptic/bacterial meningitis,
    • viral or drug eruption,
    • bacterial adenitis,
    • diseases mediated by staphylococcal or streptococcal toxins,
    • rickettsial diseases.

Investigations

  • C-reactive protein.
  • FBC: leucocytosis and thrombocytosis (thrombocytosis usually only occurs in second week of illness).
  • Urine test strip: transient pyuria.
  • ESR: elevated.
  • Cardiology assessment, including echocardiography to detect coronary artery aneurysms: 100% sensitivity, 97% specificity, done at beginning and 6 weeks after disease improvement.

GENERAL AND SUPPORTIVE MEASURES

  • Routine supportive care.
  • Maintain hydration with oral fluids.

MEDICINE TREATMENT

As soon as diagnosed and preferably within first 10 days from onset of fever after specialist consultation.

  • Immunoglobulin, IV, 2 g/kg as a single dose administered over 12 hours.
    • Repeat dose, if necessary, if temperature does not normalise or rash does not resolve within 24 hours.

If fever continues after 2 doses:

  • Methylprednisolone, IV, 30 mg/kg/dose. Specialist consultation.

All children:

  • Aspirin (high dose), oral, 20 mg/kg/dose 6 hourly for 72 hrs or until fever settles.

Follow with:

  • Aspirin, oral, 3–5mg/kg/day until ESR and platelet count are normal if there are no coronary artery aneurysms.
    • If coronary aneurysms are present continue for at least 2 years after aneurysms have resolved or lifelong if coronary aneurysms persist.

REFERRAL

  • All patients for confirmation of diagnosis.
  • For echocardiography to confirm presence of coronary artery aneurysms.