title: In juvenile dermatomyositis, cardiac systolic dysfunction is present after long-term follow-up and is predicted by sustained early skin activity.
abstract: OBJECTIVE
  To compare systolic cardiac function in patients with juvenile dermatomyositis (JDM) with matched controls and examine associations between systolic and diastolic cardiac function and disease variables.
  
  
  METHODS
  Fifty-nine patients, examined at follow-up, median 16.8 years (2-38 years) after disease onset, were compared with 59 age-matched and sex-matched controls. Echocardiography was performed and analysed blinded to patient information. We used mitral annulus displacement to assess the relative long-axis shortening of the left ventricle (long-axis strain) and early diastolic tissue velocity (e'), as markers for systolic and diastolic function, respectively. Disease activity and organ damage were assessed at follow-up by clinical examination and retrospectively by chart review.
  
  
  RESULTS
  Long-axis strain was reduced in patients compared with controls (16.6% (2.5) vs 17.7% (2.0), mean (SD), p=0.001), whereas no difference was seen between patients with active and inactive disease. Disease duration correlated with systolic and diastolic function (rsp=-0.50 and rsp=-0.73, both p<0.001) and so did Myositis Damage Index (MDI) 1 year (rsp=-0.36 and rsp=-0.46) and MDI at follow-up (rsp=-0.33 and rsp=-0.60), all p<0.01. High early disease activity score (DAS) in skin (DAS skin 1 year), but not in muscle, predicted systolic (standardised β=-0.28, p=0.011, R(2)=48%) and diastolic dysfunction (β=-0.36, p<0.001, R(2)=72%) at follow-up.
  
  
  CONCLUSIONS
  Long-axis strain was reduced in JDM patients compared with controls, suggesting systolic dysfunction. Impaired systolic and diastolic function was predicted by DAS skin 1 year. This indicates a common pathway to two different cardiac manifestations in JDM, perhaps with similar pathogenesis as skin affection.
authors.affiliation: Institute for Experimental Medical Research, Oslo University Hospital-Ullevål and University of Oslo, Oslo, Norway Medical Faculty, Institute for Clinical Medicine, University of Oslo, Oslo, Norway.
authors.forename: Thomas
authors.initials: T
authors.lastname: Schwartz
authors.affiliation: Section of Rheumatology, Oslo University Hospital-Rikshospitalet, Oslo, Norway Norwegian Competence Centre of Pediatric and Adolescent Rheumatology, Oslo University Hospital-Rikshospitalet, Oslo, Norway.
authors.forename: Helga
authors.initials: H
authors.lastname: Sanner
authors.affiliation: Department of Cardiology, Oslo University Hospital-Ullevål, Oslo, Norway.
authors.forename: Ola
authors.initials: O
authors.lastname: Gjesdal
authors.affiliation: Medical Faculty, Institute for Clinical Medicine, University of Oslo, Oslo, Norway Section of Rheumatology, Oslo University Hospital-Rikshospitalet, Oslo, Norway.
authors.forename: Berit
authors.initials: B
authors.lastname: Flatø
authors.affiliation: Institute for Experimental Medical Research, Oslo University Hospital-Ullevål and University of Oslo, Oslo, Norway Department of Cardiology, Oslo University Hospital-Ullevål, Oslo, Norway.
authors.forename: Ivar
authors.initials: I
authors.lastname: Sjaastad
mesh_terms: D000293:Adolescent; D000328:Adult; D000367:Age Factors; D016022:Case-Control Studies; D002648:Child; D003882:Dermatomyositis / Q000150:complications* / Q000453:epidemiology / Q000503:physiopathology; D003971:Diastole / Q000502:physiology; D005500:Follow-Up Studies; D006801:Humans; D008875:Middle Aged; D009664:Norway / Q000453:epidemiology; D015588:Observer Variation; D011379:Prognosis; D015203:Reproducibility of Results; D012720:Severity of Illness Index; D013599:Systole / Q000502:physiology; D013997:Time Factors; D014463:Ultrasonography; D018487:Ventricular Dysfunction, Left / Q000000981:diagnostic imaging / Q000453:epidemiology / Q000209:etiology* / Q000503:physiopathology; D055815:Young Adult
keywords: Cardiovascular Disease; Dermatomyositis; Disease Activity; Inflammation