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OBJECTIVE To compare cardiac function in patients with juvenile dermatomyositis (JDM) with matched controls, and examine associations between pathological electrocardiography (ECG), echocardiographic abnormalities and disease variables in patients with JDM. METHODS A total of 59 patients with JDM, examined a median 16.8 years (range 2-38 years) after(More)
Idiopathic inflammatory myopathies (IIM) include the main subgroups polymyositis (PM), dermatomyositis (DM), inclusion body myositis (IBM) and juvenile DM (JDM). The mentioned subgroups are characterised by inflammation of skeletal muscles leading to muscle weakness and other organs can also be affected as well. Even though clinically significant heart(More)
OBJECTIVE To describe the clinical, laboratory, and histopathologic features, current treatment, and outcome of patients with macrophage activation syndrome (MAS) complicating systemic juvenile idiopathic arthritis (JIA). METHODS In this multinational, multicenter study, pediatric rheumatologists and hemato-oncologists entered patient data collected(More)
OBJECTIVE To describe cumulative organ damage in juvenile dermatomyositis (JDM) patients and to identify patient characteristics and early disease variables that predict organ damage. METHODS An inception cohort of 60 patients diagnosed with JDM from 1970 to 2006 was examined, median 16.8 (2.0-38.1) years after disease onset. Disease activity was measured(More)
OBJECTIVE To compare cytokine profiles in patients with juvenile dermatomyositis (JDM) after medium to long-term follow-up with matched controls, and to examine associations between cytokine levels and disease activity, disease duration and organ damage. METHODS Fifty-four JDM patients were examined median 16.8 years (2-38) after disease onset (follow-up)(More)
OBJECTIVES The aims of this study were to examine disease activity by the Paediatric Rheumatology International Trials Organization (PRINTO) criteria for inactive disease and the Myositis Disease Activity Assessment Tool (MDAAT) in JDM patients after long-term follow-up and to identify predictors of these outcomes. METHODS A retrospective inception cohort(More)
OBJECTIVES To compare pulmonary function in patients with juvenile dermatomyositis (JDM) with that of matched controls; and to examine associations between pulmonary function impairment, high-resolution CT (HRCT) abnormalities and other disease variables in patients with JDM. METHODS A total of 59 patients with JDM clinically examined a median 16.8 years(More)
OBJECTIVE To compare muscle strength, physical health, and HLA-DRB1 allele carriage frequencies in patients with longstanding juvenile dermatomyositis (DM) with that of controls, and to determine the presence of and risk factors for muscle weakness and magnetic resonance imaging (MRI)-detected muscle damage in juvenile DM patients. METHODS Fifty-nine(More)
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(More)
OBJECTIVES Low heart rate variability (HRV) is a well-established predictor of cardiac death. The aim of this study was to investigate arrhythmias and HRV in patients with JDM, and associations between HRV and inflammatory markers, echocardiographic measurements and disease parameters. METHODS Fifty-five patients with JDM were examined 2-34 years (median(More)