Abstract

ObjectiveTo investigate cardiac involvement in patients with sporadic inclusion body myositis (IBM) by cardiac magnetic resonance tomography (CMR).MethodsA case series of 20 patients with IBM underwent basic cardiac assessment and CMR including functional imaging, native and contrast-enhanced T1-weighted, and late gadolinium enhancement (LGE) imaging.ResultsAll IBM patients reported no cardiac symptoms. Echocardiography was normal in 16/17 IBM patients. In CMR, IBM patients had normal ejection fractions (mean LVEF 63 ± 7%) and ventricular mass. They had reduced left (mean 55 versus 88 ml) and right ventricular stroke volumes (mean 54 versus 86 ml) and increased early myocardial enhancement (pathological T1 Ratio in 44% versus 5%), as compared to age- and gender-matched controls. Since arterial hypertension was more often observed in IBM patients, hypertensive heart disease can also be causative for these changes. Late gadolinium enhancement did not differ statistically from healthy controls. There was no apparent association between elevated biomarkers, echocardiography and CMR.ConclusionCMR revealed subtle changes in cardiac geometry and tissue characterization in IBM patients when compared to a gender- and age-matched control group. Findings in CMR indicated a higher extent of diffuse myocardial fibrosis as well as smaller left ventricular stroke volumes. These alterations may be due to a higher prevalence of arterial hypertension in the IBM cohort.

Highlights

  • Sporadic inclusion body myositis (IBM) is the most common and disabling inflammatory myopathy among persons > 50 years of age [1, 2]

  • Cardiac magnetic resonance (CMR) has been used as an objective diagnostic tool in the detection of cardiac involvement in IBM in two singular case reports [8, 10], detecting cardiomyopathy and fatty replacement of heart muscle, respectively

  • Thirteen patients were treated with immunoglobulins (IVIG) every 6–8 weeks during the CMR acquisition, one of the patients was on therapy with corticosteroids and mycophenolate mofetil during the 3 months before CMR

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Summary

Introduction

Sporadic inclusion body myositis (IBM) is the most common and disabling inflammatory myopathy among persons > 50 years of age [1, 2]. Epidemiologic studies found no significant increase of cardiovascular disease in IBM patients since cardiac abnormalities would be expected in healthy controls of this age group [5, 14]. Cardiac magnetic resonance (CMR) imaging has been shown to be a valuable tool in the diagnostic work-up of inflammatory myocarditis [15, 16] and for the detection of cardiac involvement in systemic muscle diseases [17,18,19]. CMR has been used as an objective diagnostic tool in the detection of cardiac involvement in IBM in two singular case reports [8, 10], detecting cardiomyopathy and fatty replacement of heart muscle, respectively. The aim of this study was to characterize potential cardiac involvement in IBM using a comprehensive CMR protocol

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