Idiopathic Inflammatory Myopathies
Idiopathic Inflammatory Myopathies
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DOI：
10.1016/j.ncl.2014.04.007
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发表时间：
2014-08-01
影响因子：
2.4
通讯作者：
Amato, Anthony A.
中科院分区：
文献类型：
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作者：
Dimachkie, Mazen M.;Barohn, Richard J.;Amato, Anthony A.
特发性炎症性肌病(IIM)是一种罕见的异质性自身免疫性疾病，除了包涵体肌炎的远端和不对称性肌肉无力外，还表现为明显的近端和对称性肌肉无力。尽管有许多相似之处，但从组织病理学和病理学的角度来看，IIM是相当不同的，也显示出一些临床和治疗反应的差异。这一领域见证了我们对这些罕见疾病的病理生理学和治疗的理解方面的重大进展。这篇综述着重于皮肌炎、多发性肌炎和坏死性肌病，并考察了目前和有前景的治疗方法。
The idiopathic inflammatory myopathies (IIM) consist of rare heterogeneous autoimmune disorders that present with marked proximal and symmetric muscle weakness, except for distal and asymmetric weakness in inclusion body myositis. Despite many similarities, the IIM are fairly heterogeneous from the histopathologic and pathogenetic standpoints, and also show some clinical and treatment-response differences. The field has witnessed significant advances in our understanding of the pathophysiology and treatment of these rare disorders. This review focuses on dermatomyositis, polymyositis, and necrotizing myopathy, and examines current and promising therapies.