Key result
IVIG resistance predicts ~889% greater odds of coronary aneurysms in children with Kawasaki disease.
Why the study?
Long-term prognosis in Kawasaki disease depends on coronary sequelae, yet Western longitudinal data integrating acute-phase features, treatment, and long-term coronary outcomes remain limited.
Cohort (n=533)
No
In a 40-year Italian cohort of children with Kawasaki disease, younger age, delayed treatment, IVIG resistance, and pericardial effusion independently predicted coronary aneurysm formation, emphasizing the need for prompt, risk-based management.
Answers are grounded in this paper and cite their sources.
Captured external expert commentary on this paper, strongest first. Original sources are linked where available.
“In this issue of Pediatrics, Bonanni et al report their remarkable 4-decade experience with Kawasaki disease (KD) at a children's hospital referral center in Florence, Italy. This group had the foresight to establish a registry for clinical research and long-term follow-up in the early 1980s. The long-term experience in Italy, with increasing recognition of cases, adoption of IVIG therapy and other care delivery improvements culminated in better coronary outcomes.”
“As with peer diagnostic and treatment programs, Bonanni et al now wrestle with the challenges of patients who present with coronary dilation or aneurysms, remain febrile after initial IVIG therapy, or develop progressive coronary dilation despite treatment. They have developed a model for early identification of patients at high risk of developing coronary abnormalities. Age less than 6 months, late treatment, and IVIG resistance are well-recognized risk factors for aneurysm development in Asia and the Americas, but pericardial effusion has not been a part of any of the risk scores that have emerged.”
May aid risk stratification for aneurysms in Kawasaki disease; extends observational predictors but leaves open impact of targeted therapies.
BACKGROUND Kawasaki disease (KD) is the leading cause of acquired heart disease in children in developed countries. Long-term prognosis depends on coronary sequelae, yet Western longitudinal data integrating acute-phase features, treatment, and long-term coronary outcomes remain limited. METHODS We conducted a longitudinal observational cohort study including consecutive children diagnosed with KD between 1982 and 2025 at a single tertiary pediatric referral center in Italy. All patients underwent clinical and echocardiographic assessment during the acute phase, followed by longitudinal annual follow-up. Coronary involvement (dilation or aneurysm) was assessed by echocardiography. RESULTS Among 533 children (median age, 26 months; 59.8% male), 117 (22.0%) developed acute coronary involvement, including 27 aneurysms and 90 dilation. Intravenous immunoglobulin (IVIG) use increased from 53.3% in the 1980s to 99% after 2020. The highest rates of IVIG resistance (30.0%) and coronary lesions (27.2%) occurred between 2011 and 2020, followed by a decline in 2021–2025. Persistent coronary abnormalities were observed exclusively in patients with medium-giant aneurysms. Independent predictors of aneurysm were age less than 6 months (odds ratio [OR], 7.64), IVIG resistance (OR, 9.89), IVIG administration after day 10 (OR, 5.27), and pericardial effusion (OR, 3.16). No persistent coronary sequelae were observed in high-risk patients treated with interleukin-1 inhibitors. CONCLUSIONS In this 40-year Italian cohort, coronary aneurysms were uncommon but exclusively determined long-term coronary sequelae. Younger age, delayed treatment or IVIG resistance, and pericardial effusion independently predicted aneurysm formation, highlighting major and potentially modifiable gaps in care delivery. These findings support specialist, risk-based management with prompt escalation to reduce long-term coronary morbidity.
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Bonanni et al. (2026) conducted a cohort in Kawasaki disease (n=533). IVIG resistance, age <6 months, delayed IVIG, and pericardial effusion vs. Absence of these risk factors was evaluated on aneurysm (OR 9.89). In children with Kawasaki disease, IVIG resistance, age <6 months, delayed IVIG, and pericardial effusion independently predicted coronary aneurysm formation.
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