Benveniste Olivier, Stenzel Werner, Hilton-Jones David, Sandri Marco, Boyer Olivier, van Engelen Baziel G M
Département de Médecine Interne et Immunologie Clinique, Assistance Publique-Hôpitaux de Paris, GH Pitié-Salpêtrière, Université Pierre et Marie Curie, Inserm, U974, DHU I2B, Paris, France,
Acta Neuropathol. 2015 May;129(5):611-24. doi: 10.1007/s00401-015-1384-5. Epub 2015 Jan 13.
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.
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。神经科医生和风湿科医生必须认识到这种疾病，它可能通过临床和病理上的相似性，模仿其他肌病，尤其是多发性肌炎。多发性肌炎对免疫抑制药物治疗有反应，而sIBM即使有反应也很差。关于sIBM主要是炎症性肌病还是退行性肌病存在争议，这一区分对于指导有效的特异性治疗研究至关重要。我们在此回顾各自假说的利弊。根据我们的经验，一种可能的情况是，sIBM可能始于肌肉内的炎症。趋化因子和细胞因子吸引的白细胞激增可能导致肌纤维损伤和HLA-I过度表达。如果蛋白质降解系统过载（可能由于遗传易感性、特定的HLA-I亚型或衰老），淀粉样蛋白和其他蛋白质沉积物可能出现在肌纤维内，以恶性循环的方式加强肌病进程。
