Therapeutic management of immune-mediated necrotizing myositis.
Therapeutic management of immune-mediated necrotizing myositis.
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免疫介导的坏死性肌炎的治疗管理。
DOI：
10.1007/s40674-021-00174-1
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发表时间：
2021-06
影响因子：
1.2
通讯作者：
Tiniakou E
中科院分区：
文献类型：
--
作者：
Weeding E;Tiniakou E
特发性炎性肌病是一组异质性自身免疫性疾病，其特征在于骨骼肌炎症导致慢性肌无力。免疫介导的坏死性肌病（IMNM）是炎性肌病的一个独特亚组，其典型特征是肌纤维坏死，肌肉活检时伴有极轻微的炎性浸润，肌酸激酶水平高度升高，以及罕见的肌外受累。本文综述了目前推荐的IMNM治疗策略，包括根据临床表型和自身抗体状态对疾病活动监测和推荐的一线免疫调节剂的讨论。根据自身抗体阳性，IMNM可分为三种亚型：抗3-羟基-3-甲基戊二酰辅酶A还原酶（HMGCR）IMNM、抗信号识别颗粒（SRP）IMNM和抗体阴性IMNM。自身抗体状态与IMNM的临床表型、预后和推荐的免疫抑制剂的选择有相当大的相关性。抗HMGCR IMNM患者倾向于对静脉注射免疫球蛋白（IVIG）反应良好，IVIG单药治疗可能足以治疗某些患者。在抗SRP IMNM中，早期利妥昔单抗通常是有利的。更一般地，通常指示迅速开始侵袭性免疫抑制，因为抗SRP和抗HMGCR IMNM两者都可能导致使人衰弱的虚弱，并且在疾病过程的早期发生肌肉萎缩和不可逆的脂肪替代。IMNM患者经常需要联合治疗以实现疾病控制，并且当逐渐减少免疫抑制时复发率较高。年轻的发病年龄是一个不良的预后因素。IMNM可能严重致残，通常需要积极的免疫抑制。对于任何给定的患者，治疗策略应根据其表现特征和自身抗体状态的严重程度来确定。虽然我们治疗IMNM的能力确实有所提高，但仍然需要更多的前瞻性试验来提供最佳治疗策略。
The idiopathic inflammatory myopathies are a heterogeneous group of autoimmune disorders characterized by skeletal muscle inflammation leading to chronic muscle weakness. Immune-mediated necrotizing myopathy (IMNM) is a distinct subgroup of inflammatory myopathy typically characterized by myofiber necrosis with minimal inflammatory infiltrates on muscle biopsy, highly elevated creatine kinase levels, and infrequent extra-muscular involvement. This review provides an overview of currently recommended treatment strategies for IMNM, including discussion of disease activity monitoring and recommended first-line immunomodulatory agents depending on clinical phenotype and autoantibody status. IMNM can be divided into three subtypes based on autoantibody positivity: anti-3-hydroxy-3-methylglutaryl-CoA reductase (HMGCR) IMNM, anti-signal recognition particle (SRP) IMNM, and antibody negative IMNM. Autoantibody status in IMNM has considerable correlation with clinical phenotype, prognosis, and recommended choice of immunosuppressive agent. Patients with anti-HMGCR IMNM tend to respond well to intravenous immunoglobulin (IVIG), and IVIG monotherapy may be sufficient treatment for certain patients. In anti-SRP IMNM, early rituximab is commonly favored. More generally, prompt initiation of aggressive immunosuppression is often indicated, as both anti-SRP and anti-HMGCR IMNM can potentially cause debilitating weakness, and muscle atrophy and irreversible fatty replacement happen early in the disease course. Patients with IMNM frequently require combination therapy to achieve disease control, and have a high rate of relapse when tapering immunosuppression. Young age of onset is a poor prognostic factor. IMNM can be severely disabling and often requires aggressive immunosuppression. For any given patient, the treatment strategy should be informed by the severity of their presenting features and autoantibody status. While our ability to treat IMNM has certainly improved, there remains a need for more prospective trials to inform optimal treatment strategies.
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