Almost one in three children with Kawasaki Disease (KD) are not diagnosed on presentation and guidelines for evaluation appear to be underused, an Australian study has found.
The findings of underdiagnosis and undertreatment of children with KD add to concerns that the incidence of the condition may be increasing, according to the authors of a small observational study conducted at a NSW hospital.
A review of 40 children with suspected KD managed at the John Hunter Children’s Hospital in NSW in 2015 and 2016 found that 29% with the condition were not diagnosed on presentation.
Those who were correctly diagnosed on presentation received aspirin and IV immunoglobulin, but the others were only identified later based on echocardiography and chart review. One in four (25%) of the children were found to have coronary artery abnormalities on echocardiography.
The review found that 30% of the children were incompletely assessed according to the American Heart Association (AHA) guidelines that have been recommended for evaluation of suspected KD since 2004. In many cases KD was not considered because children had a concurrent diagnosis such as viral upper respiratory tract infection and gastroenteritis. Also, the laboratory tests recommended in AHA pathway were rarely completed.
The study investigators noted that the presence of a polymorphous rash and extremity changes was much more common in those with confirmed KD, and had a positive predictive value of 96%. They suggested these features should be given more weight in diagnostic pathways.
They also remarked that the numbers of cases seen at the hospital suggested an incidence of KD twice as high as the previous rates of 22–26 vs 9.34 per 100,000 patients seen in Australian epidemiological studies.
Possible explanations for the higher incidence might include increased immigration from Asian countries that have high rates of KD, they suggested.
“The diagnostic accuracy of clinician assessment and treatment compliance for KD appears suboptimal,” they concluded.
The authors said the findings showed the need to encourage the use of the AHA guidelines for evaluation of KD, perhaps through education and audits within hospitals.
“This study reinforces the need to provide empiric therapy when KD is suspected, as excluding KD on the basis of an alternate diagnosis, or using echocardiography after discharge to rule out KD, may result in inadequate care.”
The study was published in Heart Lung and Circulation.