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

J Pediatr. 2011 Nov;159(5):795-801. doi: 10.1016/j.jpeds.2011.05.015. Epub 2011 Jul 23.

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.

Publication types

  • Research Support, N.I.H., Extramural
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adiponectin / analysis
  • Adult
  • Age Factors
  • Atherosclerosis / diagnostic imaging
  • Body Fat Distribution
  • Body Mass Index
  • Brachial Artery / diagnostic imaging*
  • Carotid Intima-Media Thickness*
  • Case-Control Studies
  • Cholesterol, HDL / blood*
  • Creatine Kinase / analysis
  • Dermatomyositis / blood*
  • Dermatomyositis / physiopathology*
  • Diastole / physiology
  • Female
  • Humans
  • Hypertension / physiopathology*
  • Lipodystrophy / etiology
  • Male
  • Pilot Projects
  • Risk Assessment
  • Risk Factors
  • Skinfold Thickness
  • Systole / physiology
  • Triglycerides / blood

Substances

  • Adiponectin
  • Cholesterol, HDL
  • Triglycerides
  • Creatine Kinase