Inclusion Body Myositis
Inclusion Body Myositis
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DOI：
10.1212/wnl.0000000000200188
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发表时间：
2022-02
期刊：
影响因子：
9.9
通讯作者：
A. Mammen
中科院分区：
文献类型：
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作者：
A. Mammen
包涵体肌炎(IBM)是一种进展缓慢的肌肉疾病，通常影响50岁的患者1而其他类型的肌炎的特征是对免疫抑制治疗有反应的对称性近端无力，2 IBM患者通常有不对称性远端无力，尽管使用类固醇或其他免疫调节治疗仍会进展。来自IBM患者的肌肉活检不仅包括浸润性炎症细胞，还包括在其他形式的肌炎中没有观察到的边缘空泡和细胞内蛋白质包涵体。鉴于肌肉活检中炎性和退行性改变并存，以及对治疗的不良反应，长期以来一直存在关于肌肉退行性变或自身免疫是推动IBM的病理过程的争论。
Inclusion body myositis (IBM) is a slowly progressive muscle disease that usually affects patients >50 years of age.1 Whereas other types of myositis are characterized by symmetric proximal weakness that responds to immunosuppressive therapy,2 patients with IBM often have asymmetric distal weakness that progresses despite treatment with steroids or other immunomodulatory therapies. Muscle biopsies from patients with IBM include not only infiltrating inflammatory cells but also rimmed vacuoles and intracellular protein inclusions not observed in other forms of myositis. Given the coexistence of inflammatory and degenerative features on muscle biopsy, along with the poor response to treatment, there has been a longstanding debate about whether myodegeneration or autoimmunity is the driving pathologic process in IBM.