Myositis
Abstract Number 1243 - Abnormal Cardiovascular Risk Profile in Adult Patients with Juvenile Dermatomyositis
Authors: Micah J. Eimer1, Luciana Young1, Kathy Abbott2, Roopa Seshadri2, Aneel Gursahaney2, Beverly Smulevitz1, Elisa Rhew1, Rosalind Ramsey-Goldman1, David McPherson1, Lauren M. Pachman1. 1Northwestern University Medical School, Chicago, IL; 2Children's Memorial Research Center, Chicago, IL
PURPOSE: Juvenile Dermatomyositis (JDM), the most common pediatric inflammatory myopathy, is a systemic vasculopathy, in which nailfold end row (ERL) capillary dropout is associated with chronic inflammation. Pathologic calcifications occur in 20-40% of patients. The long term cardiovascular effects of JDM are unknown. Thus, this study sought to determine if adults with JDM have evidence of cardiovascular pathology placing them at higher risk for cardiovascular events.
METHODS: Eight adults (mean age 35y ± 8.2 ) who had a history of definite JDM and eight controls (mean age 47y ± 10) were studied after obtaining informed consent. All patients underwent measurement of carotid intima- media thickness (IMT), brachial vascular reactivity (BVR), fasting lipid profile, calculation of an ultrasound derived carotid arterial calcium score (CaS) and intima medial brightness (IMB). In the JDM patients, a disease activity score for skin (DAS-S) and weakness (DAS-W) and assessment of end fold capillary loops (ERL) were performed.
RESULTS: The JDM patients were younger than the control patients, but this difference did not reach statistical significance (p=0.09). Despite that, there were differences between the two groups with regard to indices of atherosclerotic burden (IMT) and arterial intima-medial brightness (IMB). In addition there was a trend toward impaired endothelial function (BVR) among patients with JDM. JDM patients with the most severe dystrophic calcification appeared to have more marked abnormalities of HDL, IMB and BVR.
|
JDM |
Controls |
P Value |
|
|
IMT (cm) |
0.7 ± 0.08 |
0.6 ± 0.09 |
0.03 |
|
IMB |
58.6 ± 11.7 |
40.6 ± 14.5 |
0.04 |
|
BVR (% change) |
4.1 ± 3.4 |
8.3 ± 4.9 |
0.08 |
|
CaS |
11.3 ± 19.2 |
0.25 ± 0.7 |
0.12 |
|
Systolic BP (mmHg) |
130.5 ± 8.05 |
122.8 ± 10.3 |
0.31 |
|
Diastolic BP (mmHg) |
79.4 ± 8.9 |
72.4 ± 7.6 |
0.21 |
Of the markers of JDM disease activity, DAS-weakness correlated the strongest with the mean IMB (r = 0.80, p=0.02). The number of end fold capillary loops showed a significant inverse correlation with the level of HDL.
CONCLUSION: In this study, adult patients with a history of JDM demonstrated abnormalities of the cardiovascular system which may suggest an increased risk for future cardiovascular events. DAS-weakness and ERL were the best rheumatologic predictors of abnormal cardiovascular testing. Further studies with a larger patient group may help to identify predictors of cardiovascular disease in JDM.
COMMENT: Atherosclerosis is increasingly recognized as a condition mediated by systemic inflammation. Some pivotal studies in patients with systemic inflammatory diseases (SLE and RA) have demonstrated an increased risk of cardiovascular events in these populations, as high as 50-fold in one study. This study of adult patients with a history of myositis in childhood explores the atherosclerosis risk burden in this population. This study is small and lacks data about disease activity and duration as well as prednisone use, covariates that would be helpful in statistical modeling analyses. The surprising finding of elevated HDL in patients with increased nailfold capillary abnormalities allowed the further exploration of the increasingly recognized role of pathogenic HDL – which has been seen in association with lupus-related cardiovascular studies. Further study with larger numbers of patients and perhaps coronary calcification scoring with helical CT scans should be considered.