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Idiopathic inflammatory myopathies.

机译:特发性炎症性肌病。

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摘要

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.
机译:特发性炎症性肌病(IIM)包括出现急性,亚急性或慢性肌肉无效的异质稀有疾病。 除了重叠的临床表现,多核癌,皮肤病和自身免疫病性肌病外面可能与癌症或胶原血管疾病有关,并对免疫抑制治疗一致反应。 然而,从组织病理学和致病性观点来看,这3个IIM是不同的。 另一方面,包含体肌炎(IBM),老年人最常见的IIM,是临床,组织病理学和致病性截然不同的。 IBM对所有目前可用的疗法也是难以解决的。 在这一稿件中,我们描绘了我们对IIM的了解的进步,重点是临床介绍,相关病症,实验室特征,电生理学,肌肉组织病理学,发病机制和治疗。

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