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散发性包涵体肌炎(s-IBM)发病机制与治疗的新见解

[New insights in pathogenesis and therapy of sporadic inclusion body myositis (s-IBM)].

作者信息

Walter M C, Lochmüller H, Schlotter B, Reilich P, Müller-Felber W, Pongratz D

机构信息

Friedrich-Baur-Institut, Medizinische Klink, Klinikum der Ludwig-Maximilians-Universität, Ziemssenstr. 1a, 80336 München.

出版信息

Nervenarzt. 2001 Feb;72(2):117-21. doi: 10.1007/s001150050723.

Abstract

Sporadic inclusion body myositis (s-IBM) is a chronic progressive inflammatory myopathy which occurs preferentially in older patients. Histologic hallmarks are rimmed vacuoles and eosinophilic cytoplasmatic inclusions. The etiology is still unknown, but different pathogenetic mechanisms such as slow virus infection, autoimmunopathogenesis, myonuclear alterations, and mitochondrial defects have been implicated. A relation to neurodegenerative disorders and prion diseases has also been suggested. There is a poor response if any to immunosuppressive therapy. Stabilization of disease progression was shown only by intravenous immunoglobulin (IVIG) therapy. Future findings in the field of s-IBM pathogenesis may result in better therapeutic options.

摘要

散发性包涵体肌炎(s-IBM)是一种慢性进行性炎性肌病,好发于老年患者。组织学特征为镶边空泡和嗜酸性胞质包涵体。病因仍不明,但已涉及不同的发病机制,如慢病毒感染、自身免疫发病机制、肌核改变和线粒体缺陷。也有人提出与神经退行性疾病和朊病毒病有关。免疫抑制治疗即便有反应也很差。仅静脉注射免疫球蛋白(IVIG)治疗显示可稳定疾病进展。s-IBM发病机制领域的未来研究结果可能会带来更好的治疗选择。

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