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伴发和不伴发恶性肿瘤的抗TIF1-γ相关皮肌炎之间的微血管异常

Microvascular abnormalities between anti-TIF1-γ-associated dermatomyositis with and without malignancy.

作者信息

Mumtaz Sehreen, Phillipps Jordan, Sullivan Megan M, Diaz-Menindez Maximiliano, Wang Benjamin, Majithia Vikas, Craver Emily, Berianu Florentina

机构信息

Department of Rheumatology, Mayo Clinic, 4500 San Pablo Rd S, Jacksonville, FL, 32224, USA.

Department of Dermatology, Mayo Clinic, Jacksonville, FL, 32224, USA.

出版信息

BMC Rheumatol. 2025 May 6;9(1):50. doi: 10.1186/s41927-025-00504-z.

Abstract

BACKGROUND

Dermatomyositis (DM) is an immune-mediated myopathy characterized by proximal muscle weakness, inflammation, and cutaneous manifestations. Up to 25% of DM patients have an associated malignancy. Those with cancer-associated DM often face worse prognoses, poorer treatment responses, and reduced survival rates. Interestingly, anti TIF1γ-positive DM patients are notably at increased risk for malignancy, yet the underlying mechanisms and clinical correlation remain poorly understood. Nailfold video capillaroscopy (NVC) is a safe, non-invasive method for assessing vascular abnormalities, previously explored in various DM subsets but not specifically in anti TIF1γ-positive DM patients with malignancy. This study aims to characterize NVC findings in anti-TIF1γ-positive DM and assess their clinical relevance, particularly in malignancy-associated cases.

METHODS

A retrospective review at Mayo Clinic, Jacksonville from January 1st, 2010 to May 16th, 2024 was conducted. 19 cases with anti TIF1γ-positive DM and 18 idiopathic inflammatory myopathy controls were included.

RESULTS

We observed anti TIF1γ-positive DM cases to have significantly increased capillary density loss and higher microhemorrhages (p = 0.057). Cases also had higher frequencies of dilated capillaries, capillary ramifications, and capillary disorganization. Although no statistically significant differences in NVC pattern were identified in cancer vs. non-cancer anti TIF1γ-positive DM, there were greater hemorrhages and ramifications noted in the cancer anti TIF1γ-positive subset.

CONCLUSION

This study investigated NVC differences among anti TIF1γ-positive DM with malignancies versus idiopathic inflammatory myopathy controls. Our findings indicate promising microvascular differences with a potential for predicting cancer development that warrant further exploration in larger studies.

CLINICAL TRIAL NUMBER

Not applicable.

摘要

背景

皮肌炎(DM)是一种免疫介导的肌病,其特征为近端肌无力、炎症和皮肤表现。高达25%的DM患者伴有恶性肿瘤。患有癌症相关性DM的患者通常面临更差的预后、更差的治疗反应和更低的生存率。有趣的是,抗TIF1γ阳性的DM患者发生恶性肿瘤的风险显著增加,但其潜在机制和临床相关性仍知之甚少。甲襞视频毛细血管镜检查(NVC)是一种评估血管异常的安全、非侵入性方法,此前已在各种DM亚组中进行过研究,但未专门针对患有恶性肿瘤的抗TIF1γ阳性DM患者。本研究旨在描述抗TIF1γ阳性DM患者的NVC表现,并评估其临床相关性,特别是在恶性肿瘤相关病例中。

方法

对杰克逊维尔梅奥诊所2010年1月1日至2024年5月16日期间进行了回顾性研究。纳入了19例抗TIF1γ阳性DM患者和18例特发性炎性肌病对照。

结果

我们观察到抗TIF1γ阳性DM患者的毛细血管密度损失显著增加,微出血更多(p = 0.057)。这些病例中扩张毛细血管、毛细血管分支和毛细血管紊乱的频率也更高。虽然在癌症患者与非癌症患者的抗TIF1γ阳性DM中,NVC模式没有统计学上的显著差异,但在癌症抗TIF1γ阳性亚组中观察到更多的出血和分支。

结论

本研究调查了患有恶性肿瘤的抗TIF1γ阳性DM患者与特发性炎性肌病对照之间的NVC差异。我们的研究结果表明,存在有前景的微血管差异,有可能预测癌症的发生,值得在更大规模的研究中进一步探索。

临床试验编号

不适用。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e99c/12057095/e8d8083204bb/41927_2025_504_Fig1_HTML.jpg

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