Systemic-Artery Aneurysms Common in Kawasaki Disease

By Will Boggs MD

November 19, 2019

NEW YORK (Reuters Health) - Systemic-artery aneurysms are common in children with Kawasaki disease, but most resolve during follow-up, researchers report.

Coronary-artery aneurysms are recognized complications of Kawasaki disease, but less is known about the incidence and outcomes of systemic-artery aneurysms associated with Kawasaki disease.

Dr. Fang Liu from Children's Hospital of Fudan University, in Shanghai, China, and colleagues screened 162 children who presented with Kawasaki disease, based on the presence of risk factors for systemic-artery aneurysms, using full-body magnetic-resonance angiography (MRA) or peripheral angiography.

Overall, 23 children (16 boys and seven girls) had systemic artery aneurysms, for an incidence of 14.2% of those screened, the team reports in Pediatrics, online November 15.

Based on an overall population of 1,148 patients with Kawasaki disease, the researchers estimated the incidence of systemic-artery aneurysms to be 2%.

All patients with systemic artery aneurysms had concomitant coronary-artery aneurysms, and 38.6% (17/44) of children with giant coronary-artery aneurysms had systemic-artery aneurysms.

Among children with coronary-artery aneurysms, those who had systemic-artery aneurysms presented at a younger median age (5 months vs. 15 months for those without) and had a longer duration of fever (12 days vs. eight days).

Of the 85 systemic-artery aneurysms in 18 children who were managed for a median six months, 92% (79/85) had some degree of regression, including 80% (68/85) that regressed to normal.

Aneurysm diameters returned to normal in all patients with small or medium systemic-artery aneurysms and in three of five patients with large systemic-artery aneurysms.

In contrast, only seven of 18 medium to giant coronary-artery aneurysms showed regression during follow-up.

"With this study, we are the first to report the incidence and early outcomes of systemic artery aneurysms in the current IVIg era, showing that although the incidence of systemic artery aneurysms may not be as dramatically reduced as we expected, systemic artery aneurysms have a relatively high regression rate, which correlated with the size of systemic artery aneurysms and was higher than that of coronary artery aneurysms during short-term follow-up," the authors conclude.

"Early screening for systemic artery aneurysms by noninvasive imaging modalities should be considered in patients with Kawasaki disease with medium or giant coronary artery aneurysms," they add.

Dr. Renu Peterson of Saint Louis University School of Medicine, in Missouri, who recently reported a case of systemic-arterial aneurysm complicated by thrombosis in an infant with Kawasaki disease, told Reuters Health by email, "I found the frequent incidence of systemic-artery aneurysm quite interesting, as well as the fact that these were seen even in patients without giant coronary-artery aneurysms, which was the case in our case report."

She emphasized "the importance of having a high index of suspicion for systemic-artery aneurysms, particularly in young patients (infants), those with refractory disease, and in those patients with giant coronary-artery aneurysms. If there is evidence of altered perfusion in the extremities of these patients, it is important to consider systemic-artery aneurysms and perform imaging for further evaluation. These aneurysms, if large, can thrombose and could require urgent treatment."

Dr. Liu did not respond to a request for comments.


Pediatrics 2019.


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