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散发性包涵体肌炎:发病机制探讨

Sporadic inclusion body myositis: pathogenic considerations.

作者信息

Karpati George, O'Ferrall Erin K

机构信息

Department of Neurology, McGill University, Montreal Neurological Institute, Montreal, Quebec, Canada.

出版信息

Ann Neurol. 2009 Jan;65(1):7-11. doi: 10.1002/ana.21622.

Abstract

Sporadic inclusion body myositis is the commonest acquired disease of skeletal muscles after 50 years of age, and as such it has commanded a great deal of attention of investigators over the past 25 years. As a result, a large amount of information has accumulated concerning its clinical profile, myopathology, and immunopathology. In the myopathology and immunopathology, there is general agreement that the characteristic features could be divided into a degenerative and an inflammatory group. However, there has been controversy about the possible role of these changes in the pathogenesis of muscle fiber damage. In particular, there is no agreement whether a cause-and-effect relationship exists between these two groups of changes, and if so, which is the primary one. In this brief overview, we examine the validity of the various controversial observations and critically review the justification for the two major hypotheses for the primary role of inflammation versus degeneration.

摘要

散发性包涵体肌炎是50岁以后最常见的获得性骨骼肌疾病,因此在过去25年里受到了研究者的广泛关注。结果,积累了大量关于其临床特征、肌病理学和免疫病理学的信息。在肌病理学和免疫病理学方面,人们普遍认为其特征性表现可分为退行性和炎症性两组。然而,关于这些变化在肌纤维损伤发病机制中的可能作用一直存在争议。特别是,对于这两组变化之间是否存在因果关系,以及如果存在,哪一组是主要的,尚无定论。在这篇简要综述中,我们检验了各种有争议观察结果的有效性,并批判性地审视了炎症与退行性变哪一个起主要作用的两种主要假说的依据。

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