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New approaches to the assessment and treatment of the idiopathic inflammatory myopathies

机译:评估和治疗特发性炎症性肌病的新方法

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The rarity and heterogeneity of the idiopathic inflammatory myopathies (IIM), and the few validated assessment tools available, have limited information to guide the management of patients with polymyositis, dermatomyositis or inclusion body myositis. In light of the need for such tools, the International Myositis Assessment and Clinical Studies Group (IMACS) was formed as a multidisciplinary consortium of rheumatologists, neurologists, dermatologists, physiatrists and other myositis experts to develop consensus and standards for the conduct and reporting of myositis studies, and to facilitate myositis research. IMACS has developed consensus core set measures of disease activity, disease damage and patient-reported outcomes, and compiled a preliminary definition of improvement. The IMACS tools assist in the evaluation of the extent of disease activity and damage, although other approaches - including key clinical features, laboratory tests, muscle T1 and short τ inversion recovery MRI and immunological markers - are also helpful. Clinical remission is a realistic objective for most patients and should be pursued aggressively to optimise outcomes. Physical therapy and rehabilitation should be applied early and consistently to achieve optimal strength and function. Treatments that have been developed for other immune-mediated diseases are also being used and tested in the IIM, and some have shown anecdotal evidence of benefit. Recent advances in understanding the pathogenesis of myositis, development of assessments and treatments for other diseases that can be applied to myositis, and international collaborations and consensus standards for evaluating the IIM, all promise improvements in the assessment and treatment of myositis in the future.
机译:特发性炎症性肌病(IIM)的稀有性和异质性,以及少数可用的经过验证的评估工具,对于指导治疗多发性肌炎,皮肌炎或包涵体肌炎的患者的信息有限。鉴于对此类工具的需求,国际性肌炎评估和临床研究小组(IMACS)成立了风湿病学家,神经病学家,皮肤病学家,生理学家和其他肌炎专家组成的多学科联合体,旨在为进行和报告肌炎制定共识和标准研究,并促进肌炎研究。 IMACS已开发出针对疾病活动,疾病损害和患者报告的结局的共识性核心指标,并编制了改善的初步定义。 IMACS工具可帮助评估疾病活动和损害的程度,尽管其他方法(包括关键临床特征,实验室检查,肌肉T1和短τ倒置恢复MRI和免疫学标记)也有帮助。对于大多数患者来说,临床缓解是一个现实的目标,应该积极追求以优化结果。物理治疗和康复应及早应用,以保持最佳强度和功能。 IIM也正在使用和测试针对其他免疫介导疾病开发的治疗方法,其中一些方法已经显示出有益的传闻证据。在了解肌炎的发病机理方面的最新进展,可应用于肌炎的其他疾病的评估和治疗方法的发展以及评估IIM的国际合作和共识标准,都有望在未来的肌炎评估和治疗中得到改善。

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