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
{"title":"16p来自INSPIRE-IBM研究的包涵体肌炎呼吸评估的比较评价","authors":"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","doi":"10.1016/j.nmd.2025.105480","DOIUrl":null,"url":null,"abstract":"<div><div>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.</div></div>","PeriodicalId":19135,"journal":{"name":"Neuromuscular Disorders","volume":"53 ","pages":"Article 105480"},"PeriodicalIF":2.8000,"publicationDate":"2025-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"16PComparative evaluation of respiratory assessments in inclusion body myositis from INSPIRE-IBM study\",\"authors\":\"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\",\"doi\":\"10.1016/j.nmd.2025.105480\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<div><div>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.</div></div>\",\"PeriodicalId\":19135,\"journal\":{\"name\":\"Neuromuscular Disorders\",\"volume\":\"53 \",\"pages\":\"Article 105480\"},\"PeriodicalIF\":2.8000,\"publicationDate\":\"2025-09-01\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Neuromuscular Disorders\",\"FirstCategoryId\":\"3\",\"ListUrlMain\":\"https://www.sciencedirect.com/science/article/pii/S096089662500207X\",\"RegionNum\":4,\"RegionCategory\":\"医学\",\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"Q2\",\"JCRName\":\"CLINICAL NEUROLOGY\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Neuromuscular Disorders","FirstCategoryId":"3","ListUrlMain":"https://www.sciencedirect.com/science/article/pii/S096089662500207X","RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q2","JCRName":"CLINICAL NEUROLOGY","Score":null,"Total":0}
16PComparative evaluation of respiratory assessments in inclusion body myositis from INSPIRE-IBM study
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.
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.