Background/Purpose: Juvenile Dermatomyositis (JDM) is a multisystem pediatric autoimmune disease characterized by chronic inflammation of muscle and skin. Premature atherosclerosis is an important cause of mortality in adults with rheumatic diseases, which is attributed to chronic inflammation, hypertension and dyslipidemia (low HDL and elevated triglyceride). Older patients who had JDM in childhood have higher risk of atherosclerosis evident by increased intima media thicknes and cardiovascular damage. There are limited data on the prevalence of dyslipidemia in children with JDM.

Methods: This was a retrospective study conducted at The CureJM Center, Ann & Robert H. Lurie Children’s Hospital. We included all JDM patients (n= 210, 73% female) who had either a random (n= 65) or fasting lipid profile (n= 145) and a disease activity score close to the time of sampling. Based on the 2011 AAP guidelines for cardiovascular health, the lipid profile data was divided into three groups: acceptable, borderline and abnormal. One way ANOVA was conducted to compare the means of these three groups using SPSS. IRB approval was obtained (IRB# 2012-14858).

Results: 210 JDM patients (145 with fasting lipid profile) were included in this study. 32% of the patients had elevated fasting triglyceride (TG) level. One third of the subjects had low HDL (Table 1). Elevated fasting TG is associated with higher Skin, Muscle and Total Disease Activity Score (DAS) (Table 2). Surprisingly, there was no significant correlation between abnormal fasting TG level and lipodystrophy, which can be explained by the high prevalence of abnormal TG, not only in lipodystrophy patients, but also in the other groups, who were each taking a range of immunosuppressive medications.

Conclusion: We found that many JDM patients have elevated fasting TG (32%) and/or low HDL (30%), which is similar to the reported dyslipidemia in other rheumatic diseases. Of note, dyslipidemia in this study was associated with an increased Total Disease Activity Score, raising the possibility that chronic inflammation may contribute to the generation of dyslipidemia in JDM. On the other hand, this association could be due to immunosuppressive medications such as corticosteroids. We suggest annual monitoring of lipid profile in JDM patients with early institution of dietary intervention and exercise, for this high prevalence of dyslipidemia is associated with an increased risk of cardiovascular disease in adults with JDM in childhood.

Table 1: Prevalence of Dyslipidemia in JDM Patients

Table 2: Association of Fasting Triglyceride Level and Disease Activity Score

* P value <0.05 ** P value <0.01 *** P value <0.001