Background/Purpose: Juvenile systemic lupus erythematosus (JSLE) and juvenile dermatomyositis (JDM) are associated with chronic inflammation that can accelerate atherosclerosis. There is no specific guidance regarding the best way to stratify adolescents/young adults (AYA) based on cardiovascular disease (CVD)-risk for personalised management.Objective: To evaluate the performance of validated CVD-risk scores in JSLE/JDM, comparing risk prediction with long term atherosclerotic burden indicators.

Methods: A retrospective cohort analysis of 155 AYA with JSLE (n=76) and JDM (n=79) using descriptive statistics/regression analysis was conducted. CVD-risk was assessed at last follow-up using validated adult scores: Framingham Risk Score (FRS), Atherosclerotic Cardiovascular Disease (ASCVD) and QRISK3, and the Pathobiological Determinants of Atherosclerosis in Youth (PDAY) score (validated from age 14).

Results: AYA with JSLE vs. JDM were 84% vs.72% females (P=0.08), 60% vs. 29% Non-White (P < 0.001), with age at last follow-up of 22.3±5.4 vs.18.3±5.5 years (P < 0.001), and median age at disease onset 13.0 (11-16) and 7.5 (5-10) years (P < 0.001). At last assessment, AYA with JSLE had more hypertension (13% vs. 3%, P=0.02), and higher total cholesterol (P=0.01) and LDL-cholesterol (P=0.02) levels compared to JDM, while there were no differences in BMI, smoking or diabetes prevalence. The cumulative dose of steroids was higher in JSLE vs. JDM (P < 0.001). The PDAY score was the only one with adequate performance, stratifying 43.5% and 32.8% of JSLE patients as ‘high’ and ‘very high’ risk, respectively, while only 15% of AYA with JDM were classified as ‘high’ risk (P < 0.001). PDAY scores remained significantly higher in JSLE vs. JDM after adjusting for age/disease duration (P < 0.001). PDAY did not correlate with FRS, ASCVD or QRISK3 scores in either cohort. When last visit was dichotomized by any major flare, AYA with active JSLE or damage (PedSDI >1) had higher PDAY score (mean 11 vs. 7, P=0.004, and mean 10 vs. 6, P=0.02, respectively). In a multivariable logistic model, four independent predictors of high CVD-risk were identified in JSLE: older age (OR ~1.5 per 5-year increase, P=0.03), longer disease duration (OR ~1.4 per 5-year increase, P=0.04), higher LDL level (OR ~2.0 per 1 mmol/L increase, P=0.02), and presence of active JSLE (OR ~3.3 for any BILAG A vs. no BILAG A flare at last visit, P=0.01). Too few AYA with JDM had ‘high’ CVD-risk based on PDAY score for robust modeling; all were males (N&#3f12), one was an ex-smoker and N&#3f9 had low HDL-cholesterol during active disease. None of the typical JDM features (muscle enzyme levels, MMT8 score or CDASI skin disease score) showed an association with PDAY, supporting that traditional factors drove the high CVD-risk observed in JDM.

Conclusion: Traditional 10-year CVD-risk assessments validated in adults consistently classified all AYA with JSLE and JDM as low risk, while PDAY score was the only one useful for risk stratification in AYA with JSLE/JDM. This study is the first to highlight higher CVD-risk in JSLE vs. JDM in young adulthood, after adjustment for age/disease duration, as well as disease-related factors driving high CVD-risk in JSLE compared to traditional ones in JDM in young adulthood.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/comparative-assessment-of-cardiovascular-risk-and-its-predictors-in-a-large-cohort-of-young-adults-with-juvenile-systemic-lupus-erythematosus-and-juvenile-dermatomyositis/