Quantitative muscle MRI in sporadic inclusion body myositis (sIBM): A prospective cohort study.

IF 3.2 4区 医学 Q2 CLINICAL NEUROLOGY
Lara Schlaffke, Robert Rehmann, Martijn Froeling, Anne-Katrin Güttsches, Matthias Vorgerd, Elena Enax-Krumova, Johannes Forsting
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引用次数: 0

Abstract

Background: Sporadic inclusion body myositis (sIBM) is the predominant idiopathic inflammatory myopathy (IIM) in older people. Limitations of classical clinical assessments have been discussed as possible explanations for failed clinical trials, underlining the need for more sensitive outcome measures. Quantitative muscle MRI (qMRI) is a promising candidate for evaluating and monitoring sIBM.

Objective: Longitudinal assessment of qMRI in sIBM patients.

Methods: We evaluated fifteen lower extremity muscles of 12 sIBM patients (5 females, mean age 69.6, BMI 27.8) and 12 healthy age- and gender-matched controls. Seven patients and matched controls underwent a follow-up evaluation after one year. Clinical assessment included testing for muscle strength with Quick Motor Function Measure (QMFM), IBM functional rating scale (IBM-FRS), and gait analysis (6-minute walking distance). 3T-MRI scans of the lower extremities were performed, including a Dixon-based sequence, T2 mapping and Diffusion Tensor Imaging. The qMRI-values fat-fraction (FF), water T2 relaxation time (wT2), fractional anisotropy (FA), mean diffusivity (MD), axial diffusivity (λ1), and radial diffusivity (RD) were analysed.

Results: Compared to healthy controls, significant differences for all qMRI parameters averaged over all muscles were found in sIBM using a MANOVA (p < 0.001). In low-fat muscles (FF < 10%), a significant increase of wT2 and FA with an accompanying decrease of MD, λ1, and RD was observed (p≤0.020). The highest correlation with clinical assessments was found for wT2 values in thigh muscles (r≤-0.634). Significant changes of FF (+3.0%), wT2 (+0.6 ms), MD (-0.04 10-3mm2/s), λ1 (-0.05 10-3mm2/s), and RD (-0.03 10-3mm2/s) were observed in the longitudinal evaluation of sIBM patients (p≤0.001). FA showed no significant change (p = 0.242).

Conclusion: qMRI metrics correlate with clinical findings and can reflect different ongoing pathophysiological mechanisms. While wT2 is an emerging marker of disease activity, the role of diffusion metrics, possibly reflecting changes in fibre size and intracellular deposits, remains subject to further investigations.

散发性包涵体肌炎(sIBM)的定量肌肉磁共振成像:前瞻性队列研究
背景:散发性包涵体肌炎(sIBM)是老年人最主要的特发性炎症性肌病(IIM)。传统临床评估的局限性被认为是临床试验失败的可能原因,这强调了对更敏感的结果测量的需求。定量肌肉磁共振成像(qMRI)是评估和监测sIBM的一个有前途的候选方法:方法:我们对 15 名下肢肌肉萎缩症患者进行了评估:我们评估了 12 名 sIBM 患者(5 名女性,平均年龄 69.6 岁,体重指数 27.8)和 12 名年龄和性别匹配的健康对照者的 15 块下肢肌肉。7 名患者和匹配对照组在一年后接受了随访评估。临床评估包括快速运动功能测量法(QMFM)肌力测试、IBM功能评分量表(IBM-FRS)和步态分析(6分钟步行距离)。对下肢进行了 3T-MRI 扫描,包括基于狄克逊序列、T2 映射和弥散张量成像。对qMRI值脂肪分数(FF)、水T2弛豫时间(wT2)、分数各向异性(FA)、平均扩散率(MD)、轴向扩散率(λ1)和径向扩散率(RD)进行了分析:结论:qMRI 指标与临床发现相关,可反映不同的持续病理生理机制。虽然 wT2 是疾病活动性的新兴标志物,但弥散指标的作用可能反映纤维大小和细胞内沉积物的变化,仍有待进一步研究。
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来源期刊
Journal of neuromuscular diseases
Journal of neuromuscular diseases Medicine-Neurology (clinical)
CiteScore
5.10
自引率
6.10%
发文量
102
期刊介绍: The Journal of Neuromuscular Diseases aims to facilitate progress in understanding the molecular genetics/correlates, pathogenesis, pharmacology, diagnosis and treatment of acquired and genetic neuromuscular diseases (including muscular dystrophy, myasthenia gravis, spinal muscular atrophy, neuropathies, myopathies, myotonias and myositis). The journal publishes research reports, reviews, short communications, letters-to-the-editor, and will consider research that has negative findings. The journal is dedicated to providing an open forum for original research in basic science, translational and clinical research that will improve our fundamental understanding and lead to effective treatments of neuromuscular diseases.
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