Vasculitis is a feature of many different diseases and syndromes of childhood (Hicks, 1988). In some, it is the predominant manifestation of the condition; in others, it may be one aspect of a multisystem disease. Of the various vasculitic syndromes seen in children, Kawasaki disease (mucocutaneous lymph node syndrome) is comparatively common and is of some importance since, unlike many vasculitides, there is good evidence pointing to an infective initiating agent.

In this chapter the clinical and laboratory features of Kawasaki disease are described with current views on management and prognosis. In addition, the evidence supporting endothelial involvement is outlined, providing further support for the general importance of endothelial cell pathology in the variety of immunologically mediated diseases described in other chapters of this volume.

Kawasaki disease–clinical and therapeutic aspects

This childhood systemic vasculitis was first described in Japan in 1967 (Kawasaki, 1967). Since then over 80000 cases have been reported from that country alone (Yanagawa & Nakamura, 1986), although it is of worldwide distribution, affecting predominantly infants and young children under five years of age (Tizard et al., 1991a). There is an ethnic bias towards Oriental or Afro-Caribbean children, a male preponderance, some seasonality, and occasional epidemics (Hicks & Melish, 1986; Rowley, Gonzalez-Crussi & Shulman, 1988).

Clinical features

The principal manifestations are outlined in Table 9.1, based on the diagnostic guidelines prepared by the Japan Kawasaki Disease Research Committee (1984).