Longitudinal MuSK antibody levels may correlate with disease severity in MuSK myasthenia gravis

IF 2.5 4区医学 Q3 IMMUNOLOGY

Journal of neuroimmunology Pub Date : 2026-05-01 Epub Date: 2026-02-01 DOI:10.1016/j.jneuroim.2026.578876

Manato Yasuda , Akiyuki Uzawa , Etsuko Ogaya , Hideo Handa , Kentaro Kurumada , Kyosuke Takasaka , Hiroyuki Akamine , Yukiko Ozawa , Satoshi Kuwabara

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引用次数: 0

Abstract

Background

In muscle-specific kinase antibody-positive myasthenia gravis (MuSK-MG), the clinical utility of MuSK antibody levels as a biomarker for disease severity is debated, with conflicting reports on its correlation with clinical status. This study aimed to clarify this association by applying a rigorous statistical model to a longitudinal dataset followed from an immunotherapy-naïve state.

Methods

We conducted a retrospective longitudinal study on 6 patients with MuSK-MG tracked from their treatment-naïve baseline. The primary analysis used 103 data points where MuSK antibody levels and MG activities of daily living (MG-ADL) scores were available. A secondary analysis used the subset of 81 data points where total immunoglobulin G (IgG) levels were also concurrently measured. Generalized linear mixed-effects models (GLMM) were used to assess the intrapatient correlation, adjusting for interpatient variability by including a random intercept for each subject.

Results

First, the GLMM analysis of 103 data points revealed a strong positive intrapatient correlation between MuSK antibody levels and MG-ADL scores (P < 0.001). Second, in the subset analysis (n = 81), MuSK antibody levels remained positively and specifically correlated with MG-ADL scores (P = 0.002), whereas total IgG levels showed no independent correlation (P = 0.61) when included in the same model.

Conclusions

MuSK antibody level, unlike total IgG, is a specific and valuable biomarker for monitoring intrapatient disease activity and therapeutic response. These findings strongly support the utility of serial MuSK antibody monitoring within individual patients.

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麝香纵向抗体水平可能与麝香型重症肌无力的疾病严重程度相关。

背景：在肌肉特异性激酶抗体阳性的重症肌无力（MuSK- mg）中，MuSK抗体水平作为疾病严重程度的生物标志物的临床应用存在争议，关于其与临床状态的相关性的报道相互矛盾。本研究旨在通过应用严格的统计模型来澄清这种关联，以纵向数据集遵循immunotherapy-naïve状态。方法：我们对6例MuSK-MG患者进行了回顾性的纵向研究，追踪他们的treatment-naïve基线。初步分析使用103个数据点，其中MuSK抗体水平和MG日常生活活性（MG- adl）评分可用。第二次分析使用了81个数据点的子集，其中也同时测量了总免疫球蛋白G （IgG）水平。使用广义线性混合效应模型（GLMM）来评估患者内部相关性，通过包括每个受试者的随机截距来调整患者间变异性。结果：首先，103个数据点的GLMM分析显示，MuSK抗体水平与MG-ADL评分之间存在很强的正相关性(P)。结论：MuSK抗体水平与总IgG不同，是监测患者体内疾病活动和治疗反应的特异性和有价值的生物标志物。这些发现有力地支持了对个体患者进行MuSK抗体连续监测的实用性。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

Journal of neuroimmunology 医学-免疫学

CiteScore

6.10

自引率

3.00%

发文量

154

审稿时长

37 days

期刊介绍： The Journal of Neuroimmunology affords a forum for the publication of works applying immunologic methodology to the furtherance of the neurological sciences. Studies on all branches of the neurosciences, particularly fundamental and applied neurobiology, neurology, neuropathology, neurochemistry, neurovirology, neuroendocrinology, neuromuscular research, neuropharmacology and psychology, which involve either immunologic methodology (e.g. immunocytochemistry) or fundamental immunology (e.g. antibody and lymphocyte assays), are considered for publication.