摘要：

特发性炎性肌病是一组具有临床异质性的自身免疫性疾病，主要表现为肌肉炎症性改变、血清肌酸激酶水平升高、间质性肺病及其他多器官损害。皮肌炎、多发性肌炎和包涵体肌炎是特发性炎性肌病的经典类型。随着病理学“坏死性肌病”概念的提出，逐渐将其视为独立疾病实体。2004 年，免疫性坏死性肌病成为特发性炎性肌病的亚型，其临床表现、免疫学标志物、影像学和肌肉病理学均有别于其他类型特发性炎性肌病，但这一分类尚未被大家所熟知。本文将围绕特发性炎性肌病诊断发展历程进行综述，重点介绍免疫性坏死性肌病这一新亚型。

关键词: 肌炎, 综述

Abstract:

The idiopathic inflammatory myopathy (IIM) is a group of clinically heterogeneous, autoimmune-mediated disorders characterized by inflammation of muscle, elevated creatine kinase (CK), interstitial lung disease and other organ systems involvement. Dermatomyositis (DM), polymyositis (PM) and inclusion body myositis (IBM) are three major subtypes of IIM. "Necrotic myopathy" is firstly described as a pathological concept and later considered as an independent disease entity. Necrotizing autoimmune myopathy (NAM) is a newly recognized subtype of IIM since the year of 2004. NAM tends to show a distinct disease profile in clinical presentation, immune markers, muscle imaging as well as muscle pathology which have not been fully described. Therefore in this article, we will review the advances in the classification and diagnosis of IIM and focus on the characteristics of NAM.

Key words: Myositis, Review