Kawasaki disease

KD is an acute, self-limiting illness. The immediate outcome has improved dramatically, with a decrease in the frequency of coronary artery aneurysms to less than 3% following the introduction of intravenous immunoglobulin (IVIG) therapy. Overall, the mortality rate is less than 0.5%.

However, in untreated patients it is associated with significant morbidity and mortality. Delayed diagnosis, particularly of incomplete KD, and KD in very young children, poses a challenge as these patients have a high risk of aneurysms. The long-term prognosis of children with giant aneurysms remains a concern due to their associated risk of ischaemia or thrombosis. Management of such patients includes thromboprophylaxis and careful identification of evolving stenoses. Selected patients may need invasive revascularisation procedures.

Clinicians should therefore focus on early diagnosis and rapid escalation of treatment when there is no immediate response to IVIG. It remains to be seen whether other anti-inflammatory agents, such as new immunosuppressive therapies or new anti-cytokine biologicals, will further improve management and outcome of KD.