包涵体肌炎患者的免疫分型：INSPIRE-IBM研究

IF 2.8 4区医学 Q2 CLINICAL NEUROLOGY

Neuromuscular Disorders Pub Date : 2025-09-01 DOI:10.1016/j.nmd.2025.105466

P. Farahat, C. Phillips, I. Hernandez, M. Wencel, T. Mozaffar, A. Villalta

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引用次数: 0

摘要

包涵体肌炎（IBM）是一种骨骼肌疾病，其特征是严重的肌肉炎症和变性，导致功能下降。IBM患者的T细胞倾向于Th1和与疾病持续时间相关的高度分化表型。然而，这种T细胞信号在IBM进展中的调控尚不清楚。INSPIRE-IBM研究是一项多中心纵向调查，招募年龄在40岁或以上的患者，根据ENMC 2011标准进行临床诊断。基线外周血单个核细胞（PBMC）样本的免疫表型证实了KLRG1+ CD8+ TemRA表型的高分化T细胞的升高。尽管调节性T细胞（Tregs）和KLRG1+ TemRAs之间存在轻微的负相关，但我们发现Tregs的一个子集表现出高的TIGIT表达。鉴于TIGIT抑制Th1反应，进一步研究Treg亚群对高度分化T细胞的功能影响，可能会为免疫调节疗法提供信息，以改善IBM结果。

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02PImmunophenotyping of patients with inclusion body myositis: INSPIRE-IBM study

Inclusion Body Myositis (IBM) is a skeletal muscle disorder characterized by severe muscle inflammation and degeneration, leading to functional decline. T cells in IBM patients are skewed towards a Th1 and highly-differentiated phenotype that correlates with disease duration. However, the regulation of this T cell signature with IBM progression remains unclear. The INSPIRE-IBM study is a multicenter longitudinal investigation that enrolled patients ages 40 years or older with clinically defined IBM under the ENMC 2011 criteria. Immunophenotyping of baseline peripheral blood mononuclear cell (PBMC) samples confirmed an elevation of highly-differentiated T cells with a KLRG1+ CD8+ TemRA phenotype. Despite a slight negative correlation between regulatory T cells (Tregs) and KLRG1+ TemRAs, We found a subset of Tregs exhibiting high TIGIT expression. Given that TIGIT suppresses Th1 responses, further studies that define the functional impact of Treg subsets on highly-differentiated T cells may inform immune-modulating therapies to improve IBM outcomes.

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来源期刊

Neuromuscular Disorders 医学-临床神经学

CiteScore

4.60

自引率

3.60%

发文量

543

审稿时长

53 days

期刊介绍： This international, multidisciplinary journal covers all aspects of neuromuscular disorders in childhood and adult life (including the muscular dystrophies, spinal muscular atrophies, hereditary neuropathies, congenital myopathies, myasthenias, myotonic syndromes, metabolic myopathies and inflammatory myopathies). The Editors welcome original articles from all areas of the field: • Clinical aspects, such as new clinical entities, case studies of interest, treatment, management and rehabilitation (including biomechanics, orthotic design and surgery). • Basic scientific studies of relevance to the clinical syndromes, including advances in the fields of molecular biology and genetics. • Studies of animal models relevant to the human diseases. The journal is aimed at a wide range of clinicians, pathologists, associated paramedical professionals and clinical and basic scientists with an interest in the study of neuromuscular disorders.