Immune-Mediated Necrotizing Myopathy (IMNM): A Story of Antibodies

IF 3 Q3 IMMUNOLOGY

Antibodies Pub Date : 2024-02-07 DOI:10.3390/antib13010012

Sarah Julien, Inès Challier, Marine Malleter, F. Jouen, L. Drouot, Olivier Boyer

引用次数: 0

Abstract

Immune-mediated necrotizing myopathy (IMNM) is a rare and severe disease that corresponds to a specific entity of idiopathic inflammatory myopathy. Patients with IMNM suffer from proximal muscle weakness, and present high levels of creatine kinase and necrotic myofibers. Anti-Signal Recognition Particle (SRP) and anti-3-hydroxy-3-methylglutaryl-coenzyme A reductase autoantibodies (HMGCR) have recently been identified in two thirds of patients with IMNM and are used as a hallmark of the disease. In this review, we provide a detailed description of these antibodies and the tests used to detect them in the serum of patients. Based on in vitro studies and mouse models of IMNM, we discuss the role of autoantibodies in the pathogenesis of the disease. Finally, in the light of the latest knowledge, we conclude with a review of recent therapeutic approaches in IMNM.

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免疫相关坏死性肌病 (IMNM)：抗体的故事

免疫介导坏死性肌病（IMNM）是一种罕见的严重疾病，属于特发性炎症性肌病的一种特殊类型。IMNM患者近端肌肉无力，肌酸激酶和坏死肌纤维水平较高。最近在三分之二的 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，并将其作为该病的标志。在这篇综述中，我们详细介绍了这些抗体以及用于检测患者血清中这些抗体的检测方法。根据 IMNM 的体外研究和小鼠模型，我们讨论了自身抗体在该病发病机制中的作用。最后，根据最新的知识，我们回顾了 IMNM 的最新治疗方法。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

Antibodies IMMUNOLOGY-

CiteScore

7.10

自引率

6.40%

发文量

审稿时长

11 weeks

期刊介绍： Antibodies (ISSN 2073-4468), an international, peer-reviewed open access journal which provides an advanced forum for studies related to antibodies and antigens. It publishes reviews, research articles, communications and short notes. Our aim is to encourage scientists to publish their experimental and theoretical results in as much detail as possible. There is no restriction on the length of the papers. Full experimental and/or methodical details must be provided. Electronic files or software regarding the full details of the calculation and experimental procedure - if unable to be published in a normal way - can be deposited as supplementary material. This journal covers all topics related to antibodies and antigens, topics of interest include (but are not limited to): antibody-producing cells (including B cells), antibody structure and function, antibody-antigen interactions, Fc receptors, antibody manufacturing antibody engineering, antibody therapy, immunoassays, antibody diagnosis, tissue antigens, exogenous antigens, endogenous antigens, autoantigens, monoclonal antibodies, natural antibodies, humoral immune responses, immunoregulatory molecules.