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
Clinical characteristics of anti-SRP antibody positive immune-mediated necrotizing myopathy with anti-TRIM21/Ro52 antibody positive
Received date: 2023-02-02
Online published: 2023-11-17
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
徐莉, 高华杰, 杨梦歌, 李悦, 季苏琼 . 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[J]. 诊断学理论与实践, 2023 , 22(03) : 247 -254 . DOI: 10.16150/j.1671-2870.2023.03.07
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.
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