ARD Online First, published on July 23, 2013 as 10.1136/annrheumdis-2013-203279 Clinical and epidemiological research
EXTENDED REPORT
In juvenile dermatomyositis, cardiac systolic dysfunction is present after long-term follow-up and is predicted by sustained early skin activity Thomas Schwartz,1,2 Helga Sanner,3,4 Ola Gjesdal,5 Berit Flatø,2,3 Ivar Sjaastad1,5 Handling editor Tore K Kvien 1
Institute for Experimental Medical Research, Oslo University Hospital-Ullevål and University of Oslo, Oslo, Norway 2 Medical Faculty, Institute for Clinical Medicine, University of Oslo, Oslo, Norway 3 Section of Rheumatology, Oslo University HospitalRikshospitalet, Oslo, Norway 4 Norwegian Competence Centre of Pediatric and Adolescent Rheumatology, Oslo University HospitalRikshospitalet, Oslo, Norway 5 Department of Cardiology, Oslo University HospitalUllevål, Oslo, Norway Correspondence to Dr Thomas Schwartz, Institute for Experimental Medical Research, Oslo University Hospital-Ullevål and University of Oslo, Oslo 0407, Norway;
[email protected]. no Received 11 January 2013 Revised 15 May 2013 Accepted 29 June 2013
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 (e0 ), 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