Background/Purpose: Kawasaki disease (KD) is an acute systemic vasculitis predominantly affecting children under 5 years old. Although primarily known for its cardiac complications, the long-term immune-related outcomes remain poorly understood. Recent evidence suggests a potential link between KD and subsequent development of autoimmune conditions, possibly due to immune dysregulation and Th1/Th2 cytokine imbalance. The aim is to assess the long-term risk of pediatric rheumatic diseases in children with a history of KD compared to the general pediatric population.
 

Methods: This comprehensive 20-year retrospective cohort cohort study analyzed Clalit Health Services data in Israel between 2002-2022. KD patients (N&#3f2126) were compared with matched controls (N&#3f10630) with follow-up assessments conducted at multiple intervals (2, 5, 10, 15, and 20 years). Primary outcomes included juvenile idiopathic arthritis (JIA), systemic lupus erythematosus (SLE), and Henoch-Schönlein purpura (HSP) development.

Results: Children with KD demonstrated a significantly higher risk of developing pediatric rheumatic diseases compared to controls. JIA incidence was markedly elevated at 2 years (117.83 vs. 9.41 per 100,000 person-years; HR 12.51, 95% CI: 2.43-64.50, P=0.003), with this significance persisting throughout the 20-year follow-up (HR 4.38, 95% CI: 1.59-12.09, P=0.004). SLE showed consistently significantly higher incidence from early follow-up (HR 15.01, 95% CI: 1.56-144.30, P=0.019) at 2 years, through 15 years (HR 5.01, 95% CI: 1.01-24.80, P=0.049), though not at 20-years. HSP risk was elevated at 2 years (HR 5.01, 95% CI: 1.45-17.30, P=0.011), with an approximately two-fold increased risk thereafter, without statistical significance.
 

Conclusion: Our findings reveal a crucial message for clinicians caring for children with KD: the battle doesn’t end when the fever subsides. These children face significantly higher risks of developing pediatric rheumatic diseases, with JIA risk up to 12.5 times higher at 2 years post-diagnosis and consistently

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/risk-assessment-for-pediatric-rheumatic-diseases-in-children-with-a-history-of-kawasaki-disease-a-long-term-retrospective-comparative-big-data-cohort-study/