Controversies in diagnosis and management of Kawasaki disease

Abstract

Kawasaki disease (KD) is a common medium vessel systemic vasculitis that usually occurs in small children. It has a predilection for the coronary arteries, but other medium sized arteries can also be involved. The etiology of this disorder remains a mystery. Though typical presentation of KD is quite characteristic, it may also present as incomplete or atypical disease in which case the diagnosis can be very challenging. As both incomplete and atypical forms of KD can be associated with serious coronary artery complications, the pediatrician can ill afford to miss these diagnoses. The American Heart Association has enunciated consensus guidelines to facilitate the clinical diagnosis and treatment of this condition. However, there are still several issues that remain controversial. Intravenous immunoglobulin remains the cornerstone of management but several other treatment modalities, especially glucocorticoids, are increasingly finding favour. We review here some of the contemporary issues, and the controversies thereon, pertaining to management of KD.

Keywords: Kawasaki disease; Diagnosis; Intravenous immunoglobulin; Treatment; Controversies

Core tip: The diagnosis of Kawasaki disease poses several challenges for the treating pediatricians as it is based on a set of criteria that are entirely clinical. To further complicate matters, several children present with incomplete and atypical forms of the disease. It is known that children with incomplete and atypical Kawasaki disease do not have milder form of the disease, rather the rate of coronary and non-coronary complications may even be higher in these subgroups as the diagnosis often gets delayed. While intravenous immunoglobulin remains the cornerstone of management, several children require additional form of therapy thereby further challenging the clinical skills and judgment of the pediatricians.