Cui Beibei, Liu Hongjiang, Liu Ruiting, Yin Geng, Xie Qibing
Department of Rheumatology and Immunology, West China Hospital, Sichuan University, Chengdu, China.
Health Management Center, General Practice Medical Center, West China Hospital, Sichuan University, No. 37 Guoxue Alley, Chengdu, 610041, China.
Ther Adv Musculoskelet Dis. 2025 Jan 28;17:1759720X251314697. doi: 10.1177/1759720X251314697. eCollection 2025.
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.
To identify distinct clinical subgroups of anti-SRP IMNM patients.
A retrospective study was conducted on anti-SRP IMNM patients treated at West China Hospital of Sichuan University between January 2010 and October 2023.
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.
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.
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.
抗信号识别颗粒免疫介导的坏死性肌病（抗SRP免疫介导坏死性肌病，anti-SRP IMNM）是一种罕见的自身免疫性疾病，其特征为肌肉无力和坏死。确定该患者群体中的临床亚组有助于疾病的管理。
确定抗SRP IMNM患者的不同临床亚组。
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患者进行了一项回顾性研究。
收集临床数据。进行无监督聚类分析，根据患者的临床特征将其分为不同的亚组。进行统计分析以比较所确定聚类之间的临床特征和结局。
该研究共纳入116例患者，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组患者病程较短（中位病程3个月），肌肉无力严重（医学研究委员会（MRC）评分≤3分者占78.38%），肌肉酶水平高，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（中位病程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%），间质性肺疾病（70.97%）和心脏损伤（45.16%）的发生率较高。在第1组中，16.13%的病例为难治性，复发率为38.71%，与其他两组相比显著更高。
本研究突出了抗SRP IMNM患者之间的临床异质性，并确定了具有独特特征的三个不同临床亚组。这些发现为个性化管理提供了见解。
