Amyloid deposits and inflammatory infiltrates in sporadic inclusion body myositis: the inflammatory egg comes before the degenerative chicken.
Amyloid deposits and inflammatory infiltrates in sporadic inclusion body myositis: the inflammatory egg comes before the degenerative chicken.
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DOI：
10.1007/s00401-015-1384-5
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发表时间：
2015-05
影响因子：
12.7
通讯作者：
van Engelen BG
中科院分区：
文献类型：
--
作者：
Benveniste O;Stenzel W;Hilton-Jones D;Sandri M;Boyer O;van Engelen BG
散发性包涵体肌炎（sIBM）是50岁以上患者中最常见的获得性肌病。神经学家和风湿病学家必须认识到这种疾病，通过临床和病理学上的相似性，它可能与其他肌病相似，特别是多发性肌炎。而多发性肌炎响应免疫抑制剂药物治疗，sIBM响应差，如果在所有。关于sIBM主要是炎症性还是退行性肌病存在争议，这种区别对于指导有效的特定疗法的研究至关重要。我们在这里回顾各自的假设的优点和缺点。我们的经验使我们倾向于一种可能的情况，即sIBM可能始于肌肉内的炎症。由趋化因子和细胞因子吸引的白细胞的涌入可诱导纤维损伤和HLA-I过表达。如果蛋白质降解系统过载（可能是由于遗传易感性，特别是HLA-I亚型或衰老），淀粉样蛋白和其他蛋白质沉积可能会出现在肌肉纤维中，从而加强恶性循环中的肌病过程。
Sporadic inclusion body myositis (sIBM) is the most frequently acquired myopathy in patients over 50 years of age. It is imperative that neurologists and rheumatologists recognize this disorder which may, through clinical and pathological similarities, mimic other myopathies, especially polymyositis. Whereas polymyositis responds to immunosuppressant drug therapy, sIBM responds poorly, if at all. Controversy reigns as to whether sIBM is primarily an inflammatory or a degenerative myopathy, the distinction being vitally important in terms of directing research for effective specific therapies. We review here the pros and the cons for the respective hypotheses. A possible scenario, which our experience leads us to favour, is that sIBM may start with inflammation within muscle. The rush of leukocytes attracted by chemokines and cytokines may induce fibre injury and HLA-I overexpression. If the protein degradation systems are overloaded (possibly due to genetic predisposition, particular HLA-I subtypes or ageing), amyloid and other protein deposits may appear within muscle fibres, reinforcing the myopathic process in a vicious circle.
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影响因子：
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作者：
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