Kawasaki disease - A common childhood vasculitis

Kawasaki disease (KD) is an acute self-limiting vasculitis of children predominantly affecting the medium sized arteries. The disease was first described by Dr. Tomisaku Kawasaki in 1967 from Japan. KD has now been reported from more than 60 countries and is the commonest cause of acquired heart disease in children in the developed countries. Japan reports the highest incidence of KD at 265/100,000 children below 5 years, followed by Korea and Taiwan. In North America and Europe, the incidence of KD is much lower (9-25/100,000 children below 5) and appears to have plateaued down over the last few decades. The reasons for these differences in epidemiology are not clearly understood. KD has been increasingly reported from India over the last 20 years. At Chandigarh, an incidence of 4.54/100,000 children below 15 years was reported in 2011. However, this was likely to be an underestimate. The etiology of KD remains unknown. Although a genetic basis of KD seems plausible, an intercurrent infectious process seems to act as a trigger for the inflammatory cascade. Like many other vasculitides, the diagnosis of KD is essentially clinical and is based on a set of criteria first elaborated by Dr. Kawasaki himself. However, several children (especially infants) with KD can have incomplete and atypical presentations. This can result in diagnostic and therapeutic delays. Approximately 15-25% children with KD can develop coronary artery abnormalities (CAAs) if left untreated. Two dimensional echocardiography remains the gold standard in detecting CAAs in patients with KD. Dual source CT coronary angiography is a recent advance in accurate detection of CAAs with minimal radiation risk. Intravenous immunoglobulin (2 g/kg) remains the drug of choice and is administered as an infusion. Other therapeutic agents that have been used include infliximab, cyclosporine, glucocorticoids, and statins. KD has been associated with several long-term sequelae.