Clinical Status and Cardiovascular Risk Profile of Adults with a History of Juvenile Dermatomyositis

Abstract

Objective A pilot study of adults who had onset of juvenile dermatomyositis (JDM) in childhood, before current therapeutic approaches, to characterize JDM symptoms and subclinical cardiovascular disease. Study design Eight adults who had JDM assessed for disease activity and 8 healthy adults (cardiovascular disease controls) were tested for carotid intima media thickness and brachial arterial reactivity. Adults who had JDM and 16 age-, sex-, and body mass index-matched healthy metabolic controls were evaluated for body composition, blood pressure, fasting glucose, lipids, insulin resistance, leptin, adiponectin, proinflammatory oxidized high-density lipoprotein (HDL), and nail-fold capillary end row loops. Results Adults with a history of JDM, median age 38 years (24–44 years) enrolled a median 29 years (9–38 years) after disease onset, had elevated disease activity scores, skin (7/8), muscle (4/8), and creatine phosphokinase (2/8). Compared with cardiovascular disease controls, adults who had JDM were younger, had lower body mass index and HDL cholesterol (P = .002), and increased intima media thickness (P = .015) and their brachial arterial reactivity suggested impairment of endothelial cell function. Compared with metabolic controls, adults who had JDM had higher systolic and diastolic blood pressure, P = .048, P = .002, respectively; lower adiponectin (P = .03); less upper arm fat (P = .008); HDL associated with end row loops loss (r = −0.838, P = .009); and increased proinflammatory oxidized HDL (P = .0037). Conclusion Adults who had JDM, 29 years after disease onset, had progressive disease and increased cardiovascular risk factors.

Metabolic Abnormalities and Cardiovascular Risk Factors in Children with Myositis

“…Our study and others (2, 5, 37) indicate that metabolic abnormalities are frequent in patients with juvenile myositis, with a high frequency of the metabolic syndrome that could predispose to early cardiovascular disease. Our study, however, is limited primarily to a population of patients with severe, chronically active disease.…”

“…There are only four reports characterizing asymptomatic atherosclerosis in pediatric patients with rheumatic disease. Pachman reported premature carotid artery disease in young adults with JDM (37). Reports in pediatric SLE revealed significantly increased carotid artery intima-media thickness (38) and significant myocardial perfusion abnormalities on thallium scanning (39), while a third report failed to show abnormalities in endothelial function (40).…”

Increased expression of vascular cell adhesion molecule 1 in muscle biopsy samples from juvenile dermatomyositis patients with short duration of untreated disease is regulated by miR‐126

“…The miRNA miR‐126 inhibits ischemia‐induced neovascularization (40), also present in juvenile DM, and also targets insulin receptor substrate 1, which is involved in the development of insulin resistance associated with mitochondrial dysfunction (41). Mitochondrial dysfunction has been previously described in the muscle of children with juvenile DM (4, 12), as has the development of insulin resistance in older patients with juvenile DM (42), which may be associated with observed premature cardiovascular damage (43). The effect of miR‐126 on both VCAM‐1 and endothelial cell structure and function adds another layer of complexity to the pathophysiology of juvenile DM and the evolution of the inflammatory process as defined by the duration of untreated disease.…”

Cardiovascular risk in pediatric-onset rheumatological diseases

“…A recent study from Sweden showed that adults with polymyositis/dermatomyositis had a standardized incidence ratio of 1.92 for the development of coronary heart disease as compared with age- and sex-matched controls [105]. The only study to examine the risk of atherosclerosis in JDM compared CIMT and FMD of eight adults with a history of JDM with eight healthy adults [106]. The CIMT was higher in JDM patients despite the fact that they were younger and had lower body mass index than controls.…”

“…The previous mentioned study of CIMT was also performed to document risk factors for atherosclerosis in JDM [106]. These same patients had higher blood pressure values, higher prevalence of abnormal HDL-C levels and lower adiponectin levels compared with controls.…”