中国实用儿科杂志

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炎症性肌病病理特征

常杏芝   

  1. 作者单位:北京大学第一医院儿科,北京 100034
  • 出版日期:2014-10-06 发布日期:2014-10-08

Pathologic features in inflammatory myopathy.

CHANG Xing-zhi.   

  1. Department of Pediatrics,Peking University First Hospital,Beijing 100034,China
  • Online:2014-10-06 Published:2014-10-08

摘要:

特征性病理改变有助于提高炎症性肌病诊断和分类的确切性。炎症性肌病共同病理改变是肌纤维坏死、再生和炎症细胞浸润。特发性免疫性炎症性肌病为炎症性肌病最常见者,其临床类型及病理特征为:(1)皮肌炎,束周肌纤维萎缩伴束周小血管周围炎症细胞浸润;(2)多发性肌炎,肌内膜炎症细胞浸润,可侵入非坏死肌纤维;(3)免疫介导的坏死性肌病,显著肌纤维坏死,缺乏或散在少量单核细胞浸润;(4)散发性包涵体肌炎,肌内膜单核细胞浸润,肌纤维内镶边空泡和直径16~21 nm的管丝状包涵体。

关键词: 炎症性肌病, 免疫性, 病理, 分类

Abstract:

Abstract: Myopathology can be used to accurately specify the diagnosis and the classification of the inflammatory myopathy. In general,pathological changes of inflammatory myopathy are muscle fibre necrosis,regeneration,and focal invasion by inflammatory cells.The distinctive pathological features of most common idiopathic autoimmune inflammatory myopathies are:(1)dermatomyositis,perifascicular atrophy with CD4+ lymphocyte around the perimysial blood vessels.(2)Polymyositis,endomysial lymphocyte infiltration,CD8+ lymphocyte invasion of non-necrotic fibres with MCH-I expression.(3)Immune-mediated necrotizing myopathies, prominent necrotic fibres, with sparse or slightly myophagocytosis.(4)Sporadic inclusion body myositis,endomysial mononuclear inflammatory infiltration with invasion of non-necrotic muscle fibres, rimmed vacuoles,ultrastructural tabulofilaments of 16~21 nm.

Key words: inflammatory myopathy, immunology, pathology, classification

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