多发性肌炎和皮肌炎概述。

An overview of polymyositis and dermatomyositis.

作者信息

Findlay Andrew R, Goyal Namita A, Mozaffar Tahseen

机构信息

Department of Neurology, University of California, Irvine UC Irvine, MDA ALS and Neuromuscular Center, 200 South Manchester Avenue, Suite 110, Orange, California, 92868, USA.

出版信息

Muscle Nerve. 2015 May;51(5):638-56. doi: 10.1002/mus.24566.

DOI:10.1002/mus.24566

PMID:25641317

Abstract

Polymyositis and dermatomyositis are inflammatory myopathies that differ in their clinical features, histopathology, response to treatment, and prognosis. Although their clinical pictures differ, they both present with symmetrical, proximal muscle weakness. Treatment relies mainly upon empirical use of corticosteroids and immunosuppressive agents. A deeper understanding of the molecular pathways that drive pathogenesis, careful phenotyping, and accurate disease classification will aid clinical research and development of more efficacious treatments. In this review we address the current knowledge of the epidemiology, clinical characteristics, diagnostic evaluation, classification, pathogenesis, treatment, and prognosis of polymyositis and dermatomyositis.

摘要

多发性肌炎和皮肌炎是炎性肌病，它们在临床特征、组织病理学、对治疗的反应及预后方面存在差异。尽管它们的临床表现不同，但均表现为对称性近端肌无力。治疗主要依靠经验性使用皮质类固醇和免疫抑制剂。更深入了解驱动发病机制的分子途径、仔细进行表型分析以及准确的疾病分类将有助于临床研究和开发更有效的治疗方法。在本综述中，我们阐述了关于多发性肌炎和皮肌炎的流行病学、临床特征、诊断评估、分类、发病机制、治疗及预后的现有知识。