Background/Purpose: Dyslipidemia has been infrequently investigated in pediatric population with autoimmune rheumatic diseases. However, lipid abnormalities in these diseases may occur due to multiple risk factors such as body composition, chronic inflammation, autoantibodies, lipodystrophy, sedentarism and therapy, especially glucocorticoid. The objectives of this study was to evaluate the presence of dyslipidemia in Juvenile Dermatomyositis (JDM) and its possible risk factors.

Methods: 25 JDM patients were compared to 25 healthy controls according to demographic data, body composition, fasting lipoproteins, glycemia, insulin, antibodies and muscle enzymes. The following JDM scores were assessed: Childhood Myositis Assessment Scale (CMAS), Manual Muscle Testing (MMT), Disease Activity Score (DAS), Myositis Disease Activity Assessment Analogue Scale (MYOACT) and Myositis Intention to Treat Activity Index (MYTAX). 

Results: : Abnormal lipid profile was found in nine patients and four controls (36% vs. 16%, p=0.196). JDM patients demonstrated significant higher levels of triglycerides (TG) [80 (31-340) vs. 61 (19-182) mg/dL, p=0.011] and higher frequency of abnormal levels of high density lipoproteins (HDL) (28% vs. 4%, p=0.04) when compared to controls. JDM patients with dyslipidemia demonstrated significant lower median of HDL levels compared to those without this condition [29 (0-49) vs. 50 (39-72) mg/dL, p=0.0005] and also had significant higher TG levels [128 (31-340) vs. 69 (46-138) mg/dL, p=0.011]. JDM with dyslipidemia demonstrated a higher frequency of low HDL levels (77% vs. 0%, p=0.0001), and also a higher frequency of increased levels of TG (44% vs. 0%, p=0.01), and TC (33% vs. 0%, p=0.03). Positive anti-LPL antibody was detected in just one JDM patient with abnormal lipid profile. JDM with dyslipidemia had higher ESR (26 vs. 14.5mm/1^sthour, p=0.006), CRP (2.1 vs. 0.4mg/dL, p=0.01), DAS (6 vs. 2, p=0.008), MYOACT (0.13 vs. 0.01, p=0.012), MYTAX (0.06 vs. 0, p=0.018), and lower scores of CMAS (47 vs. 52, p=0.024) and MMT (78 vs. 80, p=0.001) compared to JDM without dyslipidemia. Positive correlations were detected between TG levels and CRP(r=0.697, p=0.001), DAS (r=0.610, p=0.001), MYOACT (r=0.661, p=0.001), MYTAX (r=0.511, p=0.008), and negative correlations with CMAS (r=-0.506, p=0.009) and MMT (r=-0.535, p=0.005). No differences were found between these groups regarding body composition, lipodystrophy, anti-LPL antibodies, and treatment (current and cumulative doses of prednisone, methotrexate and hydroxichloroquine) (p>0.05), except by higher frequency of cyclosporine current use in patients with dyslipidemia (33% vs. 0%, p=0.03).

Conclusion: Dyslipidemia in JDM patients was characterized by increased levels of TG and low levels of HDL, and disease activity and cyclosporine use were the mainly factors associated to these metabolic abnormalities.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/dyslipidemia-in-juvenile-dermatomyositis-the-role-of-disease-activity/