Acute febrile illness lasting 5 or more days.
Typical signs include fever, polymorphic rash, injected eyes, and mucosal erythema with strawberry tongue.
Swelling and erythema of the hands and feet occur in the acute stage, followed by desquamation in the second week.
Unilateral non-purulent cervical lymphadenopathy is present in about 40% of cases.
Coronary aneurysms develop in 20% to 25% of untreated patients.
Standard treatment includes intravenous immunoglobulin and/or aspirin. In resistant cases, corticosteroids or a tumour necrosis factor (TNF)-alpha inhibitor may be necessary.
Kawasaki disease (KD) is an acute, febrile, self-limiting, systemic vasculitis of unknown origin that almost exclusively affects young children. In an immunogenetically pre-disposed host, one or more infectious agents may play a role in triggering the clinical manifestations of the disease. Clinically, it is characterised by fever, polymorphic rash, conjunctivitis, mucosal erythema with strawberry tongue, induration of the hands and feet, and unilateral cervical lymphadenopathy. Morbidity and mortality depend on coronary aneurysms that develop in 20% to 25% of untreated patients. KD is the leading cause of acquired heart disease in children under 5 years of age in the US and other developed countries.
History and exam
Key diagnostic factors
- presence of risk factors
- polymorphous rash
- conjunctival injection
- skin changes in the peripheral extremities
- enlarged cervical lymph nodes
- coronary artery aneurysms
- fever and extreme irritability
Other diagnostic factors
- pericarditis with effusion
- congestive heart failure
- joint pain or oedema
- neurological manifestations
- gastrointestinal manifestations
- urological manifestations
- other dermatological manifestations
- Asian ancestry
- age 3 months to 4 years
- male sex
1st investigations to order
- serum erythrocyte sedimentation rate (ESR)
- serum CRP
Investigations to consider
- serum LFTs
- chest x-ray
- ultrasonography of the gallbladder
- ultrasonography of the testes
- lumbar puncture
- magnetic resonance angiography
- cardiac catheterisation and angiography
presentation ≤10 days from onset; or presentation >10 days from onset with risk factors for complications
presentation >10 days from onset without risk factors for complications
after initial episode: Z score always <2; no involvement at any time
after initial episode: Z score ≥2.0 to <2.5; dilation only
after initial episode: Z score ≥2.5 to <5.0; small aneurysm
after initial episode: Z score ≥5 to <10 (with absolute luminal dimension <8 mm); medium aneurysm
after initial episode: Z score ≥10 or absolute luminal diameter ≥8 mm; large or giant aneurysm
- Staphylococcal or streptococcal infection
- Systemic juvenile idiopathic arthritis (systemic JIA)
- Scarlet fever
- Fever in under 5s: assessment and initial management
- Diagnosis, treatment, and long-term management of Kawasaki disease
- Log in or subscribe to access all of BMJ Best Practice
Use of this content is subject to our disclaimer