Clinical status and cardiovascular risk profile of adults with a history of juvenile dermatomyositis

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.