Department of Neuropathology, Charité - Universitätsmedizin Berlin, Berlin, Germany.
Neuropathol Appl Neurobiol. 2012 Dec;38(7):632-46. doi: 10.1111/j.1365-2990.2012.01302.x.
Immune-mediated necrotizing myopathies (IMNMs) are now well recognized among the so-called idiopathic inflammatory myopathies (IIMs), which also comprise dermatomyositis (DM), polymyositis (PM), sporadic inclusion body myositis (sIBM) and non-specific myositis. All of these conditions are defined on the basis of distinct clinical symptoms, in combination with results derived from muscle biopsy and additional data, such as measurement of the serum creatine kinase (CK) level as well as myositis-associated and myositis-specific autoantibodies, electromyography (EMG) and modern imaging techniques. Importantly, diagnosis of one of the above mentioned myositis forms implies a specific clinical syndrome or a distinct disease. However, there is considerable clinical heterogeneity, and overlap requiring further diagnostic precision. Classification and subclassification of IIMs are highly debated and the subjects of intense research, especially as clinical trials with anti-inflammatory agents should follow universally defined and accepted criteria. This review focuses on the description of the spectrum of immune-mediated necrotizing myopathies with an emphasis on their myopathological features.
免疫介导的坏死性肌病（IMNM）现在已被认为是所谓的特发性炎性肌病（IIM）中的一种，后者还包括皮肌炎（DM）、多发性肌炎（PM）、散发性包涵体肌炎（sIBM）和非特异性肌炎。所有这些疾病都是基于不同的临床症状来定义的，同时结合肌肉活检和其他数据的结果，如血清肌酸激酶（CK）水平以及肌炎相关和肌炎特异性自身抗体、肌电图（EMG）和现代成像技术的测量结果。重要的是，上述任何一种肌炎形式的诊断都意味着存在特定的临床综合征或特定的疾病。然而，仍存在相当大的临床异质性和重叠，需要进一步提高诊断的准确性。IIM 的分类和亚型划分存在很大争议，也是研究的热点，特别是在临床试验中，应该采用普遍定义和接受的标准。这篇综述重点介绍了免疫介导的坏死性肌病的谱，并强调了其肌病理特征。
