诊断学理论与实践 ›› 2023, Vol. 22 ›› Issue (03): 247-254.doi: 10.16150/j.1671-2870.2023.03.07

• 论著 • 上一篇    下一篇

合并抗TRIM21/Ro52抗体阳性的抗SRP阳性坏死性肌病患者临床特点分析

徐莉, 高华杰, 杨梦歌, 李悦, 季苏琼()   

  1. 华中科技大学同济医学院附属同济医院神经内科,湖北 武汉 430000
  • 收稿日期:2023-02-02 出版日期:2023-06-25 发布日期:2023-11-17
  • 通讯作者: 季苏琼 E-mail:jisuqiong@163.com

Clinical characteristics of anti-SRP antibody positive immune-mediated necrotizing myopathy with anti-TRIM21/Ro52 antibody positive

XU Li, GAO Huajie, YANG Mengge, LI Yue, JI Suqiong()   

  1. Department of Neurology, Tongji Hospital of Tongji Medical College, Huazhong University of Science and Technology, Hubei Wuhan 430000, China
  • Received:2023-02-02 Online:2023-06-25 Published:2023-11-17

摘要:

目的:探讨合并抗TRIM21/Ro52抗体的抗信号识别颗粒(signal recognition particle, SRP)抗体阳性免疫介导的坏死性肌病(immune-mediated necrotizing myopathy,IMNM)患者的临床特征。方法:回顾收集2010年至2021年华中科技大学同济医学院附属同济医院收治的抗SRP抗体阳性IMNM患者(57例)的临床资料。将患者按血清肌炎相关性抗体(myositis-associated autoantibody, MAA)抗TRIM21/Ro52抗体分为抗TRIM21/Ro52抗体阳性组(以下简称抗体阳性组)和抗TRIM21/Ro52抗体阴性组(以下简称抗体阴性组),对2组患者的临床特点、实验室检查、转归预后进行对比分析。结果:本研究抗TRIM21/Ro52抗体阳性(抗体阳性组)患者为23例,抗体阴性患者(抗体阴性组)为34例。抗体阳性组的间质性肺病(interstitial lung disease, ILD)发生比例高于抗体阴性组(21.7%比2.9%,P=0.034),抗体阳性组的抗核抗体(antinuclear antibodies,ANA)高滴度表达(滴度≥1∶1000)比例(69.5%比 32.3%,P=0.008)、血清中性粒细胞计数(8.18 × 109/L比3.93 × 109/L; P=0.034)和白细胞计数(11.685×109/L比6.98×109/L,P=0.044)显著高于抗体阴性组,且上述4项指标均与抗TRIM21/Ro52抗体表达具有一定正相关(r值分别为0.312、0.351、0.290、0.274,P值分别为0.019、0.008、0.035、0.043)。57例抗SRP (+) IMNM患者在接受了83(62~96)个月的随访,51例(89.4%)患者接受了糖皮质激素治疗(或)联合免疫抑制剂治疗,其中22例(95.6%,22/23)抗TRIM21/Ro52抗体阳性患者接受免疫治疗后病情得到好转,1例(4.3%,1/23)患者失访;29例(85.3%,29/34)抗TRIM21/Ro52抗体阴性而接受免疫治疗的患者中,24例(70.6%,24/34)患者病情得到好转,其余5例(14.7%,5/34)患者临床症状未得到显著改善,1例(2.9%)患者失访。结论:抗TRIM21/Ro52抗体阳性的抗SRP抗体阳性IMNM患者更易合并ILD,血清炎性指标水平显著升高,更容易出现高滴度ANA表达。抗TRIM21/Ro52抗体IMNM患者对激素联合免疫抑制剂治疗反应良好。

关键词: 坏死性肌病, 抗SRP抗体, 抗TRIM21/Ro52抗体, 临床特点, 预后

Abstract:

Objective: To explore the clinical characteristics of anti-SRP antibody positive immune-mediated necrotizing myopathy (IMNM) with anti-TRIM21/Ro52 antibody positive. Methods: The data of 57 patients with anti-SRP antibody positive (+) IMNM admitted to the Neurology Department at Tongji Hospital between 2010 and 2021 were retrospectively analyzed. Patients were divided into anti-TRIM21/Ro52 antibody-positive group (positive group) and anti-TRIM21/Ro52 antibody-negative group (negative group) according to serum myositis-associated autoantibody (MAA) anti-TRIM21/Ro52 antibody. The clinical characteristics, laboratory test results, outcomes and prognosis of the two groups were compared and analyzed. Results: Patients with anti-SRP antibody(+) IMNM were enrolled, including anti-TRIM21/Ro52 positive patients (n=23, positive group) and anti-TRIM21/Ro52 negative patients (n=34, negative group). Higher prevalence of interstitial lung disease (ILD) (21.7% vs 2.9%; P=0.034) and high titer antinuclear antibodies (69.5% vs 32.3%; P=0.008) were observed in positive group than those in negative group, as well as the serum neutrophil counts(8.18×109/L vs 3.93×109/L, P=0.034)and white blood cell counts(11.685×109/L vs 6.98×109/L, P=0.044). All above indicators were positively correlated with the expression of anti-TRIM21/Ro52 antibody(r=0.312, 0.351, 0.290, 0.274, P=0.019, 0.008, 0.035, 0.043). Fifty-seven patients of anti-SRP antibody (+) IMNM were followed up for 83 (62-96) months. Fifty-one (89.4%) patients received glucocorticoid therapy (or) combination with immunotherapy, and 22 (95.6%) patients in positive group achieved remission, and one patient (4.3%) was lost to follow-up. Among 29 (85.3%) patients of negative group who received immunotherapy, 24 patients (70.6%) were improved, while the remaining 5 patients (14.7%) were not improved, 1 patient (2.9%) was lost to follow-up. Conclusions: Anti-SRP (+) IMNM patients with anti-TRIM21/Ro52 antibody are more likely to be complicated with ILD and have higher inflammatory indicators and ANA titer levels. IMNM patients with anti-TRIM21/Ro52 antibody positive respond well to corticosteroids and immunosuppressive therapy.

Key words: Immune-mediated necrotizing myopathy, Anti-SRP antibody, Anti-TRIM21/Ro52 antibody, Clinical characteristics, Prognosis

中图分类号: