Identification of clinical subgroups in anti-SRP positive immune-mediated necrotizing myopathy patients using cluster analysis.

IF 3.4 2区 医学 Q2 RHEUMATOLOGY
Therapeutic Advances in Musculoskeletal Disease Pub Date : 2025-01-28 eCollection Date: 2025-01-01 DOI:10.1177/1759720X251314697
Beibei Cui, Hongjiang Liu, Ruiting Liu, Geng Yin, Qibing Xie
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引用次数: 0

Abstract

Background: Anti-signal recognition particle immune-mediated necrotizing myopathy (anti-SRP IMNM) is a rare autoimmune disorder characterized by muscle weakness and necrosis. Identifying clinical subgroups within this patient population could facilitate the management of the disease.

Objectives: To identify distinct clinical subgroups of anti-SRP IMNM patients.

Design: A retrospective study was conducted on anti-SRP IMNM patients treated at West China Hospital of Sichuan University between January 2010 and October 2023.

Methods: Clinical data were collected. Unsupervised cluster analysis was conducted to classify patients into distinct subgroups based on their clinical features. Statistical analyses were performed to compare the clinical characteristics and outcomes among the identified clusters.

Results: A total of 116 patients were included in the study, and 3 distinct clinical subgroups were identified: Cluster 3 (acute), Cluster 2 (subacute), and Cluster 1 (poor prognosis). Patients in Cluster 3 exhibited a short disease course (median 3 months), severe muscle weakness (78.38% with Medical Research Council (MRC) score ⩽3), high muscle enzyme levels, and a good response to treatment. Cluster 2 patients were younger (mean age 45.83 years), had a longer disease course (median 6.5 months), milder muscle damage, and lower autoantibody titers. Cluster 1 patients were older (mean age 58.10 years), predominantly male (70.97%), and had higher incidences of interstitial lung disease (70.97%) and cardiac injury (45.16%). In Cluster 1, 16.13% of cases were refractory, and the relapse rate was 38.71%, which was significantly higher compared to the other two clusters.

Conclusion: This study highlights the clinical heterogeneity among anti-SRP IMNM patients and identifies three distinct clinical subgroups with unique characteristics. These findings provide insights for personalized management.

背景:抗信号识别颗粒免疫介导的坏死性肌病(anti-SRP IMNM)是一种罕见的自身免疫性疾病,以肌肉无力和坏死为特征。确定这一患者群体中的临床亚组有助于疾病的治疗:目的:确定抗SRP IMNM患者的不同临床亚群:设计:对 2010 年 1 月至 2023 年 10 月期间在四川大学华西医院接受治疗的抗 SRP IMNM 患者进行回顾性研究:方法:收集临床数据。采用无监督聚类分析,根据临床特征将患者分为不同的亚组。结果:共有 116 名患者被纳入聚类分析:研究共纳入了 116 名患者,并确定了 3 个不同的临床亚组:结果:研究共纳入了 116 名患者,并确定了 3 个不同的临床亚组:第 3 组(急性)、第 2 组(亚急性)和第 1 组(预后不良)。第 3 组患者病程短(中位数为 3 个月),重症肌无力(78.38% 医学研究委员会(MRC)评分⩽3),肌酶水平高,对治疗反应良好。第 2 组患者较年轻(平均年龄 45.83 岁),病程较长(中位数 6.5 个月),肌肉损伤较轻,自身抗体滴度较低。第 1 组患者年龄较大(平均年龄 58.10 岁),以男性为主(70.97%),间质性肺病(70.97%)和心脏损伤(45.16%)的发病率较高。在第一组中,16.13%的病例为难治性,复发率为 38.71%,明显高于其他两组:本研究强调了抗SRP IMNM患者的临床异质性,并确定了三个具有独特特征的临床亚组。这些发现为个性化管理提供了启示。
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来源期刊
CiteScore
6.80
自引率
4.80%
发文量
132
审稿时长
18 weeks
期刊介绍: Therapeutic Advances in Musculoskeletal Disease delivers the highest quality peer-reviewed articles, reviews, and scholarly comment on pioneering efforts and innovative studies across all areas of musculoskeletal disease.
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