In juvenile dermatomyositis, cardiac systolic dysfunction is present after long-term follow-up and is predicted by sustained early skin activity. Issue 10 (23rd July 2013)

• Record Type: Journal Article
• Authors: Schwartz, Thomas; Sanner, Helga; Gjesdal, Ola; Flatø, Berit; Sjaastad, Ivar
• Is Part Of: Annals of the rheumatic diseases. Volume 73:Issue 10(2014)
• Page Start: 1805
• Page End: 1810
• Publication Date: 2013-07-23
• Subjects: Dermatomyositis -- Disease Activity -- Cardiovascular Disease -- Inflammation; Rheumatism -- Periodicals; 616.723005
• DOI: 10.1136/annrheumdis-2013-203279
• Languages: English
• ISSNs: 0003-4967

Abstract

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.