16PComparative evaluation of respiratory assessments in inclusion body myositis from INSPIRE-IBM study

IF 2.8 4区医学 Q2 CLINICAL NEUROLOGY

Neuromuscular Disorders Pub Date : 2025-09-01 DOI:10.1016/j.nmd.2025.105480

I. Hernandez , M. Wencel , N. Goyal , O. Carbunar , M. Freimer , M. Dimachkie , C. Quinn , T. Lloyd , P. Mohassel , C. Weihl , A. Shaibani , L. Wang , N. Chahin , A. Amato , M. Wicklund , S. Dixon , P. Shieh , L. Herbelin , R. Barohn , T. Mozaffar

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Abstract

Inclusion body myositis (IBM) is the most common acquired muscle disease in individuals over 40, characterized by progressive, asymmetric muscle weakness that impairs mobility and daily functioning. Respiratory failure is a frequent complication. Previous studies in small IBM cohorts have shown that seropositivity for NT5c1A antibodies is associated with significantly reduced pulmonary function, particularly in maximum inspiratory pressure (MIP) and forced vital capacity (FVC), suggesting more severe respiratory involvement. However, larger-scale studies are needed to confirm this relationship. The ongoing INSPIRE-IBM study is a multicenter, prospective observational study involving 150 IBM patients across 13 sites. We analyzed pulmonary function data collected at baseline, 12 months, and 18 months, including sitting and supine FVC, MIP, and maximum expiratory pressure (MEP) to examine correlations with NT5c1A seropositivity. Additionally, correlations with NIH PROMIS (Sleep) were collected. Data analysis is currently underway and will be presented at the upcoming conference. Based on earlier findings, we anticipate that NT5c1A-seropositive participants will again demonstrate lower pulmonary function test results, providing greater evidence of respiratory involvement in this subgroup.

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16p来自INSPIRE-IBM研究的包涵体肌炎呼吸评估的比较评价

包体体肌炎（IBM）是40岁以上人群中最常见的获得性肌肉疾病，其特征是进行性、不对称肌肉无力，损害活动能力和日常功能。呼吸衰竭是常见的并发症。先前在小型IBM队列中的研究表明，NT5c1A抗体的血清阳性与肺功能显着降低相关，特别是在最大吸气压（MIP）和用力肺活量（FVC）方面，表明更严重的呼吸受累。然而，需要更大规模的研究来证实这种关系。正在进行的INSPIRE-IBM研究是一项多中心、前瞻性观察性研究，涉及13个地点的150名IBM患者。我们分析了基线、12个月和18个月收集的肺功能数据，包括坐位和仰卧位FVC、MIP和最大呼气压（MEP），以检查与NT5c1A血清阳性的相关性。此外，还收集了与NIH PROMIS （Sleep）的相关性。数据分析目前正在进行中，并将在即将召开的会议上提出。基于早期的发现，我们预计nt5c1a血清阳性的参与者将再次表现出较低的肺功能测试结果，为该亚组的呼吸受累提供更大的证据。

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

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.