Current Classification and Management of Inflammatory Myopathies.
Current Classification and Management of Inflammatory Myopathies.
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DOI：
10.3233/jnd-180308
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发表时间：
2018
影响因子：
3.3
通讯作者：
Schmidt J
中科院分区：
文献类型：
--
作者：
Schmidt J
骨骼肌的炎性病症包括多发性肌炎（PM）、皮肌炎（DM）、（免疫介导的）坏死性肌病（NM）、包括抗合成酶综合征（ASS）的肌炎重叠综合征（重叠肌炎，OM）和包涵体肌炎（IBM）。虽然DM发生在儿童和成人中，但所有其他形式的肌炎主要发生在中年人中。除了IBM的缓慢进展的慢性病程外，肌炎患者通常表现为亚急性发作的手臂和腿部无力，通常与疼痛和血清中肌酸激酶明显升高有关。PM、DM和大多数NM和OM患者通常对免疫抑制治疗有反应，而IBM在很大程度上对治疗无效。肌炎的诊断需要仔细和组合评估（1）临床症状，包括虚弱模式和临床试验，如肌肉MRI和肌电图（EMG），（2）与肌炎相关的自身抗体的广泛分析，和（3）骨骼肌活检的详细组织病理学检查。本文综述了肌炎的分类、诊断途径、治疗方案和病理机制。
Inflammatory disorders of the skeletal muscle include polymyositis (PM), dermatomyositis (DM), (immune mediated) necrotizing myopathy (NM), overlap syndrome with myositis (overlap myositis, OM) including anti-synthetase syndrome (ASS), and inclusion body myositis (IBM). Whereas DM occurs in children and adults, all other forms of myositis mostly develop in middle aged individuals. Apart from a slowly progressive, chronic disease course in IBM, patients with myositis typically present with a subacute onset of weakness of arms and legs, often associated with pain and clearly elevated creatine kinase in the serum. PM, DM and most patients with NM and OM usually respond to immunosuppressive therapy, whereas IBM is largely refractory to treatment. The diagnosis of myositis requires careful and combinatorial assessment of (1) clinical symptoms including pattern of weakness and paraclinical tests such as MRI of the muscle and electromyography (EMG), (2) broad analysis of auto-antibodies associated with myositis, and (3) detailed histopathological work-up of a skeletal muscle biopsy. This review provides a comprehensive overview of the current classification, diagnostic pathway, treatment regimen and pathomechanistic understanding of myositis.