免疫介导的坏死性肌病：我们目前的状况如何？

Immune Mediated Necrotizing Myopathy: Where do we Stand?

作者信息

Mohammed Abdel Gaffar A, Gcelu Ayanda, Moosajee Farzana, Botha Stella, Kalla Asgar Ali

机构信息

Department of Medicine, Rheumatic Diseases Unit, Groote Schuur Hospital, University of Cape Town, Cape Town, South Africa.

出版信息

Curr Rheumatol Rev. 2019;15(1):23-26. doi: 10.2174/1573397114666180406101850.

DOI:10.2174/1573397114666180406101850

PMID:29623846

Abstract

Immune-mediated necrotizing myopathies (IMNMs) are a group of acquired autoimmune muscle disorders which are characterized by proximal muscle weakness, high levels of creatinine kinase, and myopathic findings on electromyogram (EMG). Muscle biopsy in IMNM differentiates it from the other subgroups of Idiopathic Inflammatory Myositis (IIM) by the presence of myofibre necrosis and prominent regeneration without substantial lymphocytic inflammatory infiltrates. Anti-signal recognition particle (SRP) and anti-3hydroxy-3 methylglutarylcoenzyme A reductase (HMGCR) autoantibodies were found in two-thirds of IMNM patients. In terms of treatment, IMNM is more resistant to conventional immunosuppressive treatment, therefore, other modalities of treatment such as Intravenous Immunoglobulin (IVIG) and rituximab are often required.

摘要

免疫介导性坏死性肌病（IMNMs）是一组获得性自身免疫性肌肉疾病，其特征为近端肌无力、肌酸激酶水平升高以及肌电图（EMG）显示肌病表现。IMNM的肌肉活检通过存在肌纤维坏死和显著再生且无大量淋巴细胞炎性浸润，将其与特发性炎性肌病（IIM）的其他亚组区分开来。三分之二的IMNM患者中发现了抗信号识别颗粒（SRP）和抗3-羟基-3-甲基戊二酰辅酶A还原酶（HMGCR）自身抗体。在治疗方面，IMNM对传统免疫抑制治疗更具抗性，因此，通常需要其他治疗方式，如静脉注射免疫球蛋白（IVIG）和利妥昔单抗。