Kawasaki Disease/Mucocutaneous Lymph Node Syndrome
Description Diagnostic criteria General and supportive measures Medicine treatment Referral ICD-10 code: M30.3 DESCRIPTION Kawasaki disease is an acute systemic vasculitis of unknown aetiology occurring predominantly in children. It involves small and medium arteries. The most serious complication is coronary artery aneurysms. Important: MIS-C, a complication of SARS-CoV-2, can mimic Kawasa
Description
Diagnostic criteria
General and supportive measures
Medicine treatment
Referral
ICD-10 code: M30.3
DESCRIPTION
Kawasaki disease is an acute systemic vasculitis of unknown aetiology occurring predominantly in children. It involves small and medium arteries. The most serious complication is coronary artery aneurysms.
Important:
MIS-C, a complication of SARS-CoV-2, can mimic Kawasaki disease.
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:
- Bilateral bulbar conjunctival injection without exudates.
- Changes of the lips and oral cavity: reddening of the oral mucosa, pharynx, lips, strawberry tongue, cracking of lips.
- Polymorphous rash, primarily on the trunk.
- Cervical lymphadenopathy (lymph nodes > 1.5 cm diameter).
- 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 disease.
- Important differential diagnoses:
- 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 the 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 initially 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 the 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, 3–5 mg/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 the presence of coronary artery aneurysms.
Standard Treatment Guidelines And Essential Medicines List For South Africa, Hospital Level, Paediatrics, 2023 Edition
Chapter 12: Rheumatology And Vasculitides, 12.3 Kawasaki Disease/Mucocutaneous Lymph Node Syndrome