{"title":"Impact of sex, age at onset, and anti-cN1A antibodies on sporadic inclusion body myositis","authors":"","doi":"10.1016/j.jns.2024.123164","DOIUrl":null,"url":null,"abstract":"<div><h3>Background</h3><p>Inclusion body myositis (IBM) is a progressive myopathy occurring in patients over 45 years of age, with heterogeneous and variable clinical features. This study aimed to determine the influence of autoantibodies, gender, and age of onset on the clinical features of IBM.</p></div><div><h3>Methods</h3><p>Medical records and muscle histology findings of 570 participants with suspected IBM were reviewed. Various characteristics of patients who met the 2011 ENMC IBM diagnostic criteria were compared based on the presence of anti-cytosolic 5′-nucleotidase 1 A (cN1A) autoantibodies, gender, age of onset, and disease duration.</p></div><div><h3>Results</h3><p>Of the 353 patients who met the criteria, 41.6% were female. The mean age at onset was 64.6 ± 9.3 years, and the mean duration from onset to diagnosis was 5.7 ± 4.7 years. 196 of the 353 patients (55.5%) were positive for anti-cN1A autoantibodies and 157 were negative. Logistic regression showed that patients with anti-cN1A autoantibodies had a higher frequency of finger flexion weakness. Multiple regression showed that patients with later age of onset had shorter disease duration, lower BMI, and lower serum CK levels. Male patients had a higher frequency of onset with finger weakness and female patients had a lower BMI.</p></div><div><h3>Conclusion</h3><p>Autoantibodies, gender, age of onset, and disease duration may influence the clinical presentation of IBM, highlighting the need for a precision medicine approach that considers these factors along with the underlying mechanisms of the disease.</p></div>","PeriodicalId":17417,"journal":{"name":"Journal of the Neurological Sciences","volume":null,"pages":null},"PeriodicalIF":3.6000,"publicationDate":"2024-08-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":null,"platform":"Semanticscholar","paperid":null,"PeriodicalName":"Journal of the Neurological Sciences","FirstCategoryId":"3","ListUrlMain":"https://www.sciencedirect.com/science/article/pii/S0022510X24002995","RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q1","JCRName":"CLINICAL NEUROLOGY","Score":null,"Total":0}
引用次数: 0
Abstract
Background
Inclusion body myositis (IBM) is a progressive myopathy occurring in patients over 45 years of age, with heterogeneous and variable clinical features. This study aimed to determine the influence of autoantibodies, gender, and age of onset on the clinical features of IBM.
Methods
Medical records and muscle histology findings of 570 participants with suspected IBM were reviewed. Various characteristics of patients who met the 2011 ENMC IBM diagnostic criteria were compared based on the presence of anti-cytosolic 5′-nucleotidase 1 A (cN1A) autoantibodies, gender, age of onset, and disease duration.
Results
Of the 353 patients who met the criteria, 41.6% were female. The mean age at onset was 64.6 ± 9.3 years, and the mean duration from onset to diagnosis was 5.7 ± 4.7 years. 196 of the 353 patients (55.5%) were positive for anti-cN1A autoantibodies and 157 were negative. Logistic regression showed that patients with anti-cN1A autoantibodies had a higher frequency of finger flexion weakness. Multiple regression showed that patients with later age of onset had shorter disease duration, lower BMI, and lower serum CK levels. Male patients had a higher frequency of onset with finger weakness and female patients had a lower BMI.
Conclusion
Autoantibodies, gender, age of onset, and disease duration may influence the clinical presentation of IBM, highlighting the need for a precision medicine approach that considers these factors along with the underlying mechanisms of the disease.
背景:包涵体肌炎(IBM)是一种进展性肌病,多发于45岁以上的患者,临床特征多样且各不相同。本研究旨在确定自身抗体、性别和发病年龄对 IBM 临床特征的影响:方法:研究人员回顾了 570 名疑似 IBM 患者的病历和肌肉组织学检查结果。根据抗胞嘧啶-5'-核苷酸酶 1 A(cN1A)自身抗体的存在情况、性别、发病年龄和病程,比较了符合 2011 年 ENMC IBM 诊断标准的患者的各种特征:在符合标准的 353 名患者中,41.6% 为女性。平均发病年龄为(64.6 ± 9.3)岁,从发病到确诊的平均病程为(5.7 ± 4.7)年。353名患者中有196人(55.5%)抗N1A自身抗体呈阳性,157人呈阴性。逻辑回归显示,抗N1A自身抗体阳性患者出现手指屈曲无力的频率较高。多元回归显示,发病年龄较晚的患者病程较短、体重指数(BMI)较低、血清 CK 水平较低。男性患者出现手指无力的频率较高,女性患者的体重指数较低:结论:自身抗体、性别、发病年龄和病程可能会影响 IBM 的临床表现,因此需要采用精准医疗的方法,在考虑这些因素的同时也要考虑疾病的潜在机制。
期刊介绍:
The Journal of the Neurological Sciences provides a medium for the prompt publication of original articles in neurology and neuroscience from around the world. JNS places special emphasis on articles that: 1) provide guidance to clinicians around the world (Best Practices, Global Neurology); 2) report cutting-edge science related to neurology (Basic and Translational Sciences); 3) educate readers about relevant and practical clinical outcomes in neurology (Outcomes Research); and 4) summarize or editorialize the current state of the literature (Reviews, Commentaries, and Editorials).
JNS accepts most types of manuscripts for consideration including original research papers, short communications, reviews, book reviews, letters to the Editor, opinions and editorials. Topics considered will be from neurology-related fields that are of interest to practicing physicians around the world. Examples include neuromuscular diseases, demyelination, atrophies, dementia, neoplasms, infections, epilepsies, disturbances of consciousness, stroke and cerebral circulation, growth and development, plasticity and intermediary metabolism.