Kawasaki disease

This is an acute systemic vasculitis involving small and medium arteries with a predilection for coronary arteries. Peak occurrence in the winter and spring has been reported with peak incidence in children aged 9–11 months with a range of 6 months to 5 years. It is the second commonest vasculitic illness of childhood and associated with the development of systemic vasculitis complicated by coronary and peripheral arterial aneurysms and is the commonest cause of acquired heart disease in children in the UK (Brogan et al., 2002). The causative agent is unknown but clinical and epidemiological features are strongly suggestive of an infectious trigger, although there is no correlation with any specific viruses (Brogan et al., 2002; Harnden et al., 2009).

There is no diagnostic test for this illness seen in young children, so diagnosis is based on clinical criteria which are:

Fever of 5 days duration plus four of the following:

1. Conjunctivitis which is bilateral, bulbar and non-suppurative;
2. Lymphadenopathy which is cervical and | >1.5 cm;
3. Polymorphic rash with no vesicles or crusts;
4. Changed lip or oral mucosa with red cracked lips, ‘strawberry’ tongue or diffuse erythema of the oropharynx;
5. Changes of the extremities initially with erythema and oedema of palms and soles. At the convalescent stage, peeling of skin from the fingertips is seen.

It can be diagnosed with less than four of these features if coronary artery aneurysms are detected (Brogan et al., 2002). Management

1. This aims to reduce inflammation and prevent the occurrence of coronary arterial aneurysms (CAA) and arterial thrombosis.
2. Early treatment with aspirin and intravenous immunoglobulin has been shown to reduce the occurrence of CAA.