Inclusion body myositis: evolving concepts.
Inclusion body myositis: evolving concepts.
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DOI：
10.1097/wco.0000000000001095
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发表时间：
2022-10-01
影响因子：
4.8
通讯作者：
中科院分区：
文献类型：
--
作者：
讨论我们对包涵体肌炎的流行病学、诊断、生物标志物、病理学、发病机制、结果测量和治疗的理解的最新进展。最近的流行病学数据证实，50岁以上人群的患病率相对较高，且预期寿命较低。与癌症和其他系统性疾病的关联已得到更好的定义。磁共振成像和超声在诊断以及跟踪疾病进展中的作用已被阐明。新的血液和成像生物标志物在诊断和治疗试验中作为结果测量显示出巨大的前景。加深对疾病发病机制的了解将带来更好的治疗干预措施，但也强调了采用敏感且反应灵敏的结果测量来准确量化变化的重要性。我们对包涵体肌炎的理解出现了令人兴奋的新进展，这应该会导致改善管理和治疗选择。
To discuss recent developments in our understanding of epidemiology, diagnostics, biomarkers, pathology, pathogenesis, outcome measures and therapeutics in Inclusion Body Myositis. 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 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. There are exciting new developments in our understanding of inclusion body myositis which should lead to improved management and therapeutic options.