Basharat Pari, Christopher-Stine Lisa
Division of Rheumatology, Department of Medicine, The University of Western Ontario, 268 Grosvenor Street, London, ON, Canada, N6A 4V2.
Johns Hopkins Myositis Center, The Johns Hopkins University School of Medicine, 5200 Eastern Avenue, Mason F. Lord Center Tower; Suite 4500, Baltimore, MD, 21224, USA.
Curr Rheumatol Rep. 2015 Dec;17(12):72. doi: 10.1007/s11926-015-0548-6.
The idiopathic inflammatory myopathies (IIMs) comprise a group of autoimmune disorders that target skeletal muscle. They are characterized by typical laboratory and clinical features including muscle weakness, elevated muscle enzymes, characteristic histopathology of muscle biopsies, as well as electromyography abnormalities. The IIMs are divided into polymyositis, dermatomyositis, inclusion body myositis, nonspecific myositis, and immune-mediated necrotizing myopathy (IMNM). IMNM is distinguished by the absence of primary inflammation on muscle biopsy. IMNM may be associated with myositis-specific autoantibodies (i.e., anti-SRP and anti-HMGCR) and malignancy, in association with viral infections (HIV or hepatitis C), or in relation to other connective tissue diseases (i.e., scleroderma). Typical clinical findings such as severe muscle weakness, highly elevated creatine kinase (CK) levels, as well as resistance to conventional immunosuppressive therapy are associated with this subtype of IIM. This review provides an overview of this disease entity and focuses on its diagnosis and treatment.
特发性炎性肌病（IIM）是一组针对骨骼肌的自身免疫性疾病。其特征在于典型的实验室和临床特征，包括肌肉无力、肌肉酶升高、肌肉活检的特征性组织病理学以及肌电图异常。IIM分为多发性肌炎、皮肌炎、包涵体肌炎、非特异性肌炎和免疫介导的坏死性肌病（IMNM）。IMNM的特点是肌肉活检无原发性炎症。IMNM可能与肌炎特异性自身抗体（即抗SRP和抗HMGCR）及恶性肿瘤有关，与病毒感染（HIV或丙型肝炎）有关，或与其他结缔组织病（即硬皮病）有关。严重肌肉无力、肌酸激酶（CK）水平高度升高以及对传统免疫抑制治疗耐药等典型临床发现与这种IIM亚型相关。本综述概述了这一疾病实体，并重点关注其诊断和治疗。
