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抗TIF1γ阳性特发性炎性肌病患者的临床特征与转归:一项希腊队列研究

Clinical Profile and Outcomes in Anti-TIF1γ Positive Idiopathic Inflammatory Myositis Patients: A Greek Cohort Study.

作者信息

Syrmou Vasiliki, Liaskos Christos, Patrikious Eleni, Alexiou Ioannis, Simopoulou Theodora, Katsiari Christina G, Bogdanos Dimitrios P

机构信息

Department of Rheumatology and Clinical Immunology, Faculty of Medicine, School of Health Sciences, University of Thessaly, University General Hospital of Larissa, Larissa, Greece.

出版信息

Mediterr J Rheumatol. 2025 Jun 30;36(2):200-209. doi: 10.31138/mjr.300525.iao. eCollection 2025 Jun.

Abstract

BACKGROUND

Anti-transcription intermediary factor 1-gamma (anti-TIF1γ) antibodies are closely associated with Inflammatory myositis (IIM) and cancer-associated myositis.

OBJECTIVE

Description of clinical characteristics of anti-TIF1γ(+) IIM patients in a Greek population.

MATERIAL & METHODS: Retrospective analysis with 113 IIM cases between 2001 and 2024 was performed and clinical and laboratory data were collected. Disease manifestations and outcomes were compared between anti-TIF1γ-positive and -negative groups.

RESULTS

Twenty patients (17.7%) were anti-TIF1γ(+), of which 70% were women. The mean age was 64.8 ± 12.5 years vs 59.61 ± 12.81 of anti-TIF1γ(-) patients (p>0.05). Anti-TIF1γ was strongly associated with Dermatomyositis (DM) (95%, p < 0.001) and more severe cutaneous involvement (mean CDASI=27.35 ± 15.01 vs 14 ± 12.25 p =0.0015). Malignancy was significantly more frequent in the anti-TIF1γ(+) group (60% vs. 20.4%, p = 0.001), with an odds ratio of 5.84 (95% CI 2.09-16.31). Logistic regression identified anti-TIF1γ positivity as independent predictor of malignancy. Interstitial Lung Disease was uncommon among anti-TIF1γ(+) cases (15%, p = 0.004), while dysphagia was far more prevalent (55% vs. 22.6%, p = 0.001). Muscle power (MMT-8score) and CPK levels did not differ significantly, and survival was lower in anti-TIF1γ(+) patients (55.7% vs. 82.6% p<0.001), associated with malignancy.

CONCLUSIONS

In our cohort, anti-TIF1γ antibodies define a distinct IIM subset marked by severe skin disease, high malignancy risk, and poorer survival, supporting comprehensive cancer screening and tailored immunosuppressive treatment. This study describes this phenotype in a Greek cohort, aligning with international evidence and highlighting the need for collaborative studies.

摘要

背景

抗转录中介因子1-γ(抗TIF1γ)抗体与炎性肌病(IIM)及癌症相关性肌炎密切相关。

目的

描述希腊人群中抗TIF1γ(+)IIM患者的临床特征。

材料与方法

对2001年至2024年间的113例IIM病例进行回顾性分析,并收集临床和实验室数据。比较抗TIF1γ阳性和阴性组的疾病表现及预后。

结果

20例患者(17.7%)抗TIF1γ(+),其中70%为女性。平均年龄为64.8±12.5岁,而抗TIF1γ(-)患者为59.61±12.81岁(p>0.05)。抗TIF1γ与皮肌炎(DM)密切相关(95%,p<0.001),且皮肤受累更严重(平均CDASI=27.35±15.01 vs 14±12.25,p=0.0015)。抗TIF1γ(+)组恶性肿瘤的发生率显著更高(60% vs. 20.4%,p=0.001),优势比为5.84(95%CI 2.09-16.31)。逻辑回归分析确定抗TIF1γ阳性是恶性肿瘤的独立预测因素。抗TIF1γ(+)病例中间质性肺病不常见(15%,p=0.004),而吞咽困难更为普遍(55% vs. 22.6%,p=0.001)。肌肉力量(MMT-8评分)和肌酸磷酸激酶水平无显著差异,抗TIF1γ(+)患者的生存率较低(55.7% vs.

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b7d3/12312477/4d53327d2847/MJR-36-2-200-g001.jpg

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