Department of Neurology, University of Kansas Medical Center, 3901 Rainbow Blvd, Mail Stop 2012, Kansas City, KS 66160, United States.
J Neuroimmunol. 2011 Feb;231(1-2):32-42. doi: 10.1016/j.jneuroim.2010.10.013. Epub 2010 Nov 18.
The idiopathic inflammatory myopathies (IIM) encompass a heterogeneous group of rare disorders that present with acute, subacute, or chronic muscle weakness. Besides overlapping clinical manifestations, polymyositis, dermatomyositis and autoimmune necrotizing myopathy may be associated with cancer or collagen vascular disease, and respond generally well to immunosuppressive therapy. However, these 3 IIM are divergent from the histopathological and pathogenetic standpoints. On the other hand, inclusion body myositis (IBM), the most common IIM in the elderly, is clinically, histopathologically and pathogenetically distinct. IBM is also refractory to all currently available therapies. In this manuscript, we depict advances in our knowledge of the IIM, with emphasis on clinical presentation, associated conditions, laboratory features, electrophysiology, muscle histopathology, pathogenesis, and therapy.
特发性炎性肌病（IIM）包括一组罕见疾病，其特征为急性、亚急性或慢性肌肉无力。除了重叠的临床表现外，多发性肌炎、皮肌炎和自身免疫性坏死性肌病可能与癌症或胶原血管疾病有关，且通常对免疫抑制治疗有反应。然而，这 3 种 IIM 在组织病理学和发病机制方面存在差异。另一方面，包涵体肌炎（IBM）是老年人最常见的 IIM，在临床表现、组织病理学和发病机制方面均具有独特性。IBM 对目前所有可用的治疗方法均有抵抗性。在本手稿中，我们描述了对 IIM 的认识进展，重点介绍了临床表现、相关疾病、实验室特征、电生理学、肌肉组织病理学、发病机制和治疗。
