Distinct phenotype and prognosis of immune-mediated necrotizing myopathy based on clinical-serological-pathological classification.

IF 4.7 2区医学 Q1 RHEUMATOLOGY

Rheumatology Pub Date : 2025-04-01 DOI:10.1093/rheumatology/keae361

Hongxia Yang, Lining Zhang, Xiaolan Tian, Wenli Li, Qingyan Liu, Qinglin Peng, Wei Jiang, Guochun Wang, Xin Lu

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Abstract

Objective: The aim of the study was to investigate the characteristics and prognosis of patients with immune-mediated necrotizing myopathy (IMNM) based on clinical, serological and pathological classification.

Methods: A total of 138 patients with IMNM who met the 2018 European Neuromuscular Center criteria for IMNM including 62 anti-SRP, 32 anti-HMGCR-positive and 44 myositis-specific antibody-negative were involved in the study. All patients were followed up and evaluated remission and relapse. Clustering analysis based on clinical, serological and pathological parameters was used to define subgroups.

Results: Clustering analysis classified IMNM into three clusters. Cluster 1 patients (n = 35) had the highest creatine kinase (CK) levels, the shortest disease course, severe muscle weakness and more inflammation infiltration in muscle biopsy. Cluster 2 patients (n = 79) had the lowest CK level and moderate inflammation infiltrate. Cluster 3 patients (n = 24) had the youngest age of onset, the longest disease course and the least frequency of inflammatory infiltration. Patients in cluster 3 had the longest time-to-remission [median survival time: 61 (18.3, 103.7) vs 20.5 (16.2, 24.9) and 27 (19.6, 34.3) months] and shorter relapse-free time than those in cluster 1 and 2 [median remission time 95% CI 34 (19.9, 48.0) vs 73 (49.0, 68.7) and 73 (48.4, 97.6) months]. Patients with age of onset >55 years, more regeneration of muscle fibres, more CD4+ T infiltration and membrane attack complex deposition had more favourable outcomes regarding time to achieving remission.

Conclusions: Stratification combining clinical, serological and pathological features could distinguish phenotypes and prognosis of IMNM. The pathological characteristics may impact the long-term prognosis of patients with IMNM.

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基于临床-血清学-病理学分类的免疫介导坏死性肌病的不同表型和预后。

研究目的该研究旨在根据临床、血清学和病理学分类，探讨免疫介导的坏死性肌病（IMNM）患者的特征和预后：共有138名符合2018年欧洲神经肌肉中心IMNM标准的IMNM患者参与研究，其中包括62名抗SRP、32名抗HMGCR阳性和44名肌炎特异性抗体阴性患者。对所有患者进行了随访，并对缓解和复发情况进行了评估。根据临床、血清学和病理学参数进行聚类分析，以确定亚组：聚类分析将IMNM分为三组。第一组患者（35 人）的 CK 水平最高，病程最短，重症肌无力，肌肉活检中炎症浸润较多。第 2 组患者（79 人）的肌酸激酶水平最低，炎症浸润程度中等。第 3 组患者（人数 = 24）发病年龄最小，病程最长，炎症浸润最少。与第 1 组和第 2 组患者相比，第 3 组患者的缓解时间最长（中位生存时间：61[18.3, 103.7] vs 20.5[16.2, 24.9] 和 27[19.6, 34.3] 个月），无复发时间最短（中位缓解时间 95%CI: 34[19.9, 48.0] vs 73[49.0, 68.7] 和 73[48.4, 97.6] 个月）。发病年龄大于55岁、肌纤维再生较多、CD4+T浸润较多、MAC沉积较多的患者在获得缓解的时间方面更有利：结论：结合临床、血清学和病理学特征进行分层可区分IMNM的表型和预后。病理特征可能会影响 IMNM 患者的长期预后。

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

Rheumatology 医学-风湿病学

CiteScore

9.40

自引率

7.30%

发文量

1091

审稿时长

2 months

期刊介绍： Rheumatology strives to support research and discovery by publishing the highest quality original scientific papers with a focus on basic, clinical and translational research. The journal’s subject areas cover a wide range of paediatric and adult rheumatological conditions from an international perspective. It is an official journal of the British Society for Rheumatology, published by Oxford University Press. Rheumatology publishes original articles, reviews, editorials, guidelines, concise reports, meta-analyses, original case reports, clinical vignettes, letters and matters arising from published material. The journal takes pride in serving the global rheumatology community, with a focus on high societal impact in the form of podcasts, videos and extended social media presence, and utilizing metrics such as Altmetric. Keep up to date by following the journal on Twitter @RheumJnl.