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包涵体肌炎:不断演进的概念。

Inclusion body myositis: evolving concepts.

机构信息

Departments of Pathology & Laboratory Medicine.

Department of Neurology.

出版信息

Curr Opin Neurol. 2022 Oct 1;35(5):604-610. doi: 10.1097/WCO.0000000000001095.

Abstract

PURPOSE OF REVIEW

To discuss recent developments in our understanding of epidemiology, diagnostics, biomarkers, pathology, pathogenesis, outcome measures, and therapeutics in inclusion body myositis (IBM).

RECENT FINDINGS

Recent epidemiology data confirms a relatively higher prevalence in the population aged above 50 years and the reduced life expectancy. Association with cancer and other systemic disorders is better defined. The role of magnetic resonance imaging (MRI) and ultrasound in diagnosis as well as in following disease progression has been elucidated. There are new blood and imaging biomarkers that show tremendous promise for diagnosis and as outcome measures in therapeutic trials. Improved understanding of the pathogenesis of the disease will lead to better therapeutic interventions, but also highlights the importance to have sensitive and responsive outcome measures that accurately quantitate change.

SUMMARY

There are exciting new developments in our understanding of IBM which should lead to improved management and therapeutic options.

摘要

目的综述

讨论包涵体肌炎(IBM)在流行病学、诊断、生物标志物、病理学、发病机制、疗效评估和治疗学方面的最新研究进展。

最新发现

最近的流行病学数据证实,50 岁以上人群的 IBM 患病率相对较高,且预期寿命降低。与癌症和其他系统疾病的相关性已得到更好的定义。磁共振成像(MRI)和超声在诊断和监测疾病进展中的作用已被阐明。新的血液和影像学生物标志物在诊断和作为治疗试验的疗效评估方面显示出巨大的潜力。对疾病发病机制的深入了解将导致更好的治疗干预措施,但也强调了需要敏感且响应迅速的疗效评估,以准确量化变化。

总结

在 IBM 的认识方面有令人兴奋的新进展,这将导致治疗管理和选择的改善。

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Inclusion body myositis: evolving concepts.包涵体肌炎:不断演进的概念。
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