抗黑色素瘤分化相关基因5阳性皮肌炎的不同抗核抗体亚群在临床表现上表现出可变性：一项多中心回顾性队列研究。

IF 3.3 4区医学 Q1 MEDICINE, GENERAL & INTERNAL

Archives of Medical Science Pub Date : 2024-10-26 eCollection Date: 2025-01-01 DOI:10.5114/aoms/192272

Gang Wang, Dong Yan, Leixi Xue, Chenghua Weng, Lei Zhang, Lingxiao Xu, Fenghong Yuan, Ju Li, Min Wu, Zhanyun Da, Hua Wei, Lei Zhou, Songlou Yin, Jian Wu, Yan Lu, Dinglei Su, Lin Liu, Longxin Ma, Xiaoyan Xu, Huijie Liu, Tianli Ren, Yinshan Zang, Zhichun Liu

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引用次数: 0

摘要

抗核抗体（ANA）在抗黑色素瘤分化相关基因5阳性皮肌炎（anti-MDA5+ DM）患者中经常呈阳性。本研究旨在探讨抗mda5 + dm患者队列中ANA与临床特征及预后的关系。材料和方法：我们对南京医科大学肌炎相关间质性肺疾病（ILD）患者队列的病历进行了系统的回顾性研究。对ana阳性组和ana阴性组的各项参数进行比较分析。结果：共纳入246例抗mda5 + DM患者，其中男性28.5%，女性71.5%。中位年龄53.0岁，中位病程2个月，中位随访时间12.0个月。抗mda5 + DM患者基线时ANA阳性率为52.4%。ana阳性组抗ro52抗体阳性率（72.9% vs. 54.7%, p = 0.003）和抗氨基酰trna合成酶（抗ars）抗体阳性率（9.3% vs. 2.6%, p = 0.033）显著高于ana阴性组，ALT水平明显低于ana阴性组，分别为39.0（21.5,79.3）和51.3 (36.5,95.8),p = 0.006。两组患者在总生存率、快速进展性间质性肺病（RPILD）发病率、年龄、病程和临床特征方面均无显著差异。在ana阳性组的亚组分析中，mda5++ +患者的RPILD发生率高于MDA5+组（p = 0.028）。在ana阴性亚组分析中，mda5++ +患者的死亡率高于MDA5+组，预后较差（p = 0.026）。多因素Cox回归分析显示，乳酸脱氢酶（LDH）水平升高和快速进行性肺间质性疾病（RPILD）的存在与ana阴性抗mda5 + DM患者预后不良相关，风险比分别为1.002 （95% CI: 1.001, 1.003, p = 0.020）和13.694 （95% CI: 15.032, 37.267, p < 0.001）。结论：抗mda5 + DM患者常见于ANA，抗mda5抗体滴度高与死亡率和RPILD相关。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

Different antinuclear antibody subgroups of anti-melanoma differentiation-associated gene 5-positive dermatomyositis showed variability in clinical presentation: a multicenter retrospective cohort study.

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Introduction: Antinuclear antibodies (ANA) are frequently positive in patients with anti-melanoma differentiation-associated gene 5-positive dermatomyositis (anti-MDA5+ DM). This study aimed to investigate the association between ANA and clinical characteristics as well as prognosis in a cohort of patients with anti-MDA5+ DM.

Material and methods: We conducted a systematic retrospective study of medical records from a Nanjing Medical University cohort of patients with myositis-associated interstitial lung disease (ILD). Various parameters were compared and analyzed between the ANA-positive group and the ANA-negative group.

Results: A total of 246 patients with anti-MDA5+ DM were enrolled in this study, with 28.5% males and 71.5% females. The median age was 53.0 years, the median disease duration was 2 months, and the median follow-up period was 12.0 months. The ANA positivity rate at baseline was 52.4% in anti-MDA5+ DM patients. The ANA-positive group showed significantly higher positivity rates of anti-Ro52 antibodies (72.9% vs. 54.7%, p = 0.003) and anti-aminoacyl-tRNA synthetase (anti-ARS) antibodies (9.3% vs. 2.6%, p = 0.033) compared to the ANA-negative group, but lower ALT levels: 39.0 (21.5, 79.3) vs. 51.3 (36.5, 95.8), p = 0.006. No significant differences were observed between the two groups in terms of overall survival, rapidly progressive interstitial lung disease (RPILD) incidence, age, disease duration, and clinical characteristics. In a subgroup analysis of the ANA-positive group, MDA5+++ patients had a higher incidence of RPILD compared to the MDA5+ group (p = 0.028). In the ANA-negative subgroup analysis, MDA5+++ patients had a higher mortality rate and worse prognosis compared to the MDA5+ group (p = 0.026). Multivariate Cox regression analysis showed that elevated lactate dehydrogenase (LDH) levels and the presence of rapidly progressive interstitial lung disease (RPILD) were associated with poor prognosis in ANA-negative anti-MDA5+ DM patients, with hazard ratios of 1.002 (95% CI: 1.001, 1.003, p = 0.020) and 13.694 (95% CI: 15.032, 37.267, p < 0.001), respectively.

Conclusions: ANA is frequently found in patients with anti-MDA5+ DM. High titers of anti-MDA5 antibodies are associated with mortality and RPILD.

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来源期刊

Archives of Medical Science 医学-医学：内科

CiteScore

4.90

自引率

7.90%

发文量

139

审稿时长

1.7 months

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