Limitations of Echocardiographic Periarterial Brightness in the Diagnosis of Kawasaki Disease

2005 
Kawasaki disease (KD) is a diffuse vasculitis that has surpassed rheumatic fever as the major cause of acquired cardiovascular disease in children. Manifestations include fever, conjunctivitis, rash, lymphadenopathy, swelling of the hands and feet (followed by desquamation), and, in about 20% of cases, coronary artery involvement. The latter may take the form of discrete aneurysms or diffuse dilation and is the basis of virtually all of the mortality and longterm morbidity. Early treatment with intravenous gamma globulin substantially reduces the risk of aneurysm development 1,2 and is considered the standard of care for patients with a new diagnosis. Unfortunately, there is no diagnostic laboratory test and the diagnosis is made on the basis of fever of at least 5 days’ duration and 4 of 5 signs of conjunctivitis, mucosal changes, swollen hands and feet, rash, and cervical lymphadenopathy. 3 These findings are not specific for KD, and echocardiography is typically used in an attempt to confirm the diagnosis. Coronary artery dilation (ectasia) and aneurysm formation are quite specific for KD, but only occur in about a fifth of cases and may not be apparent early in the disease. Several investigators have alluded to “periarterial brightness” 4,5 or “prominence” 6 as an additional echocardiographic feature of KD, presumably reflecting inflammation or swelling of the coronary arterial wall. If these findings are reproducible, they might aid in the diagnosis of KD, especially in the 80% of patients who do not have obvious coronary dilation or aneurysm. The purpose of this study was to determine the intraobserver and interobserver variability in detection of periarterial brightness, and to determine the usefulness of this finding in distinguishing children with confirmed KD from control patients.
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