Clinical Criteria for the Risk of Pediatric CAA in Historical Perspective

Abstract

The objective of this paper is to examine the clinical criteria for the risk of pediatric coronary artery aneurysms from a historical perspective. In the late 1970s IPN was re-categorized as the fatal outcome of KD. Pathologists welcomed the connection between the observable rash/fever sign complex described by Kawasaki and coronary artery abnormalities (CAA). This had both research and clinical implications. Investigations shifted from exploring the mechanisms of the vasculitis to a search for an infectious agent predicted to be responsible for the sign complex. By the early 1980s, the CDC case definition, based on Kawasaki's clinical signs, was adopted as the diagnostic criteria for authorizing diagnoses and treatment to prevent coronary artery abnormalities (CAA). However, the KD case definition was designed as an epidemiological tool to authenticate the existence of the syndrome for research purposes rather than as a diagnostic tool for the detection of CAA. Based on clinical experience described in the literature and confirmed by our experience at San Diego Children's Hospital, we have found increasing numbers of children with CAA who failed to meet the KD clinical criteria. Treatment of these atypical cases is often delayed and they develop CAA. The goal of a clinical case definition should be to alert physicians to institute immediate treatment. Instead, the current case definition often serves to construct a barrier to effective intervention. This paper (re)examines the historical evidence that persuaded clinicians that IPN and KD are the same disorder. We suggest that while the merging of these two syndromes initially served a useful diagnostic purpose, continued reliance on the KD sign complex has resulted in delay of treatment of CAA in atypical cases that earlier would have been included in the designation of IPN.