Department of Neurology, Graduate School of Medical Sciences, Kumamoto University, Kumamoto, Japan; Department of Neurology, International University of Health and Welfare Narita Hospital, Narita, Japan.
Department of Neurology, Graduate School of Medical Sciences, Kumamoto University, Kumamoto, Japan.
J Neurol Sci. 2024 Sep 15;464:123164. doi: 10.1016/j.jns.2024.123164. Epub 2024 Aug 6.
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.
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.
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.
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.
包涵体肌炎(IBM)是一种发生在 45 岁以上患者的进行性肌病,具有异质性和多变的临床特征。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。
回顾了 570 名疑似 IBM 患者的病历和肌肉组织学检查结果。根据是否存在抗细胞质 5'-核苷酸酶 1A(cN1A)自身抗体、性别、发病年龄和疾病持续时间,比较符合 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。发病年龄的平均值为 64.6±9.3 岁,从发病到诊断的平均病程为 5.7±4.7 年。在 353 名患者中,有 196 名(55.5%)抗 cN1A 自身抗体阳性,157 名阴性。逻辑回归显示,抗 cN1A 自身抗体阳性的患者手指弯曲无力的频率更高。多元回归显示,发病年龄较晚的患者病程较短、BMI 较低、血清 CK 水平较低。男性患者手指无力的发病频率更高,而女性患者 BMI 较低。
自身抗体、性别、发病年龄和疾病持续时间可能影响 IBM 的临床表现,强调需要采用精准医学方法,综合考虑这些因素以及疾病的潜在机制。