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In juvenile dermatomyositis, cardiac systolic dysfunction is present after long-term follow-up and is predicted by sustained early skin activity
  1. Thomas Schwartz1,2,
  2. Helga Sanner3,4,
  3. Ola Gjesdal5,
  4. Berit Flatø2,3,
  5. Ivar Sjaastad1,5
  1. 1Institute for Experimental Medical Research, Oslo University Hospital-Ullevål and University of Oslo, Oslo, Norway
  2. 2Medical Faculty, Institute for Clinical Medicine, University of Oslo, Oslo, Norway
  3. 3Section of Rheumatology, Oslo University Hospital-Rikshospitalet, Oslo, Norway
  4. 4Norwegian Competence Centre of Pediatric and Adolescent Rheumatology, Oslo University Hospital-Rikshospitalet, Oslo, Norway
  5. 5Department of Cardiology, Oslo University Hospital-Ullevål, Oslo, Norway
  1. Correspondence to Dr Thomas Schwartz, Institute for Experimental Medical Research, Oslo University Hospital-Ullevål and University of Oslo, Oslo 0407, Norway; thomas.schwartz{at}medisin.uio.no

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, R2=48%) and diastolic dysfunction (β=−0.36, p<0.001, R2=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.

  • Dermatomyositis
  • Disease Activity
  • Cardiovascular Disease
  • Inflammation

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