诊断学理论与实践 ›› 2023, Vol. 22 ›› Issue (03): 247-254.doi: 10.16150/j.1671-2870.2023.03.07
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:XU Li, GAO Huajie, YANG Mengge, LI Yue, JI Suqiong()
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。
中图分类号:
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.
XU Li, GAO Huajie, YANG Mengge, LI Yue, JI Suqiong. Clinical characteristics of anti-SRP antibody positive immune-mediated necrotizing myopathy with anti-TRIM21/Ro52 antibody positive[J]. Journal of Diagnostics Concepts & Practice, 2023, 22(03): 247-254.
表1
2组患者的实验室指标及临床特征比较 [n(%)]
Items | anti-TRIM21/Ro52 positive group (n=23) | anti-TRIM21/Ro52 negative group (n=34) | P value |
---|---|---|---|
Sex | |||
Male | 10(43.5%) | 10(29.4%) | 0.718 |
Female | 13(56.5%) | 24(70.6%) | |
Age at myositis diagnosis (years) | 44.9±10.1 | 44.3±18.4 | 0.868 |
Disease duration (months) | 6 | 5.5 | 0.345 |
Clinical manifestation | |||
Upper promixal limbs weakness (≤3,MRC) | 7(30%) | 16(47%) | 0.275 |
Upper distal limbs weakness (≤3,MRC) | 2(9%) | 10(29%) | 0.097 |
Lower promixal limbs weakness(≤3,MRC) | 8(35%) | 19(56%) | 0.177 |
Lower distal limbs weakness (≤3,MRC) | 7(30%) | 11(32%) | >0.999 |
Atrophy | 10(43.5%) | 9(26.4%) | 0.253 |
Dyspnea | 1(4.3%) | 2(5.9%) | >0.999 |
Dysphagia | 5(21.7%) | 7(20.6%) | >0.999 |
Myalgia | 12(52.1%) | 15(44.1%) | 0.597 |
Rash | 2(8.7%) | 2(5.9%) | >0.999 |
Thyroid dysfunction | 10(43.5%) | 15(44.1%) | 0.563 |
Tumor marker abnormality | 8(34.8%) | 13(38.2%) | 0.752 |
Laboratory examinations | |||
CK (U/L) | 2629 | 1795 | 0.49 |
LDH (U/L) | 541 | 534 | 0.79 |
ALT (U/L) | 97 | 83 | 0.626 |
AST (U/L) | 83 | 88 | 0.771 |
Cr (umol/L) | 35 | 43±15 | 0.141 |
Myoglobin (ng/mL) | 605±460 | 284±307 | 0.071 |
CK-MB (ng/mL) | 98±90 | 54.4 | 0.877 |
cTnI (pg/mL) | 31±41 | 28.4 | 0.339 |
WBC (×109/L) | 11.685 | 6.98 | 0.044 |
Neutro (×109/L) | 8.18 | 3.93 | 0.034 |
Lym (×109/L) | 2.18 | 2.11 | 0.864 |
ASS antibody | 3 (13%) | 2 (5.9%) | 0.384 |
ANA (titer 1∶1000) | 6 (42.9%) | 4 (26.7%) | 0.008 |
ANA (titer 1∶3200) | 4 (28.6%) | 5 (33.3%) | |
ILD | 5 (21.7%) | 1 (2.9%) | 0.034 |
Cardiac dysfunction | 5 (21.7%) | 10(29.4%) | 0.542 |
Treatment | |||
Symptomatic treatment | 1 (4.3%) | 5(14.7%) | |
Glucocorticoid monotherapy | 10(43.5%) | 14(41.2%) | |
Glucocorticoid and Immunosuppressant | 12(52.2%) | 15(44.1%) | |
Outcome | |||
Basically normal | 4 (17.4%) | 4 (11.8%) | 0.076 |
Marked improvement | 18 (78.3%) | 24(70.6%) | |
No improvement | 0 | 5(14.7%) | |
Relapse | 1(4.3%) | 1(2.9%) |
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