Abstract

BackgroundThe structural and functional changes of the skeletal muscles in idiopathic inflammatory myopathies (IIM) caused by inflammation and immune changes can be severely disabling. The objective of this study was to assess the effect of a 24-week program combining a supervised training of activities of daily living (ADL), resistance, and stability with home exercise for improving muscle function, compared to a daily home-based exercise representing the regular outpatient care.MethodsFifty-seven patients with IIM were consecutively and non-selectively enrolled in an intervention (IG, n = 30) or control (CG, n = 27) group. Both groups were provided a standard-of-care pharmacological treatment and follow-up. Only the IG underwent the supervised intervention twice a week for 1 h per session. At baseline, 12, 24, and 48 weeks, all patients were assessed by an assessor blinded to the intervention for primary outcomes: muscle strength (Manual Muscle Testing of eight muscle groups [MMT-8]) and endurance (Functional Index-2 [FI-2]), and secondary outcomes: stability and body composition. Secondary outcomes also included questionnaires evaluating disability (Health Assessment Questionnaire [HAQ]), quality of life (Short Form 36 [SF-36]), depression (Beck’s Depression Inventory-II [BDI-II]), and fatigue (Fatigue Impact Scale [FIS]), and analysis of the systemic and local inflammatory response and perceived exertion to assess the safety of the intervention.ResultsTwenty-seven patients in the IG and 23 in the CG completed the entire program and follow-up. At week 24, compared to deterioration in the CG, we found a significant improvement in the IG in muscle strength (mean % improvement compared to baseline by 26%), endurance (135%), disability (39%), depression (26%), stability (11%), and basal metabolism (2%) and a stabilization of fitness for physical exercise. The improvement was clinically meaningful (a 24-week change by >20%) in most outcomes in a substantial proportion of patients. Although the improvement was still present at 48 weeks, the effect was not sustained during follow-up. No significant increase in the systemic or local expression of inflammatory markers was found throughout the intervention.ConclusionsThis 24-week supervised intervention focused on ADL training proved to be safe and effective. It not only prevented the progressive deterioration, but also resulted in a significant improvement in muscle strength, endurance, stability, and disability, which was clinically meaningful in a substantial proportion of patients.Trial registrationISRCTN35925199 (retrospectively registered on 22 May 2020).

Highlights

  • The structural and functional changes of the skeletal muscles in idiopathic inflammatory myopathies (IIM) caused by inflammation and immune changes can be severely disabling

  • At week 24, compared to deterioration in the control group (CG), we found a significant improvement in the intervention group (IG) in muscle strength, endurance (135%), disability (39%), depression (26%), stability (11%), and basal metabolism (2%) and a stabilization of fitness for physical exercise

  • These patients were allocated into the IG or the CG based on their motivation to undergo the intervention and ability to adhere to the study protocol

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Summary

Introduction

The structural and functional changes of the skeletal muscles in idiopathic inflammatory myopathies (IIM) caused by inflammation and immune changes can be severely disabling. Idiopathic inflammatory myopathies (IIM, briefly myositis) are a heterogeneous group of rare disorders characterized by chronic inflammation of skeletal muscles and multiple organ involvement associated with muscle weakness and low muscle endurance [1]. The widely recognized subsets of IIM are dermatomyositis (DM), polymyositis (PM), and inclusion body myositis (IBM). Other subsets of IIM have been defined such as clinically amyopathic dermatomyositis (CADM), antisynthetase syndrome (ASS), and immunemediated necrotizing myopathy (IMNM) [3]. The new insights into pathogenesis are paving the way for improvements in early diagnosis, classification, and treatment, IIM remain a major medical challenge [4, 5]

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