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重症肌无力患者中肌酸激酶水平升高、肌炎特异性抗体和肌炎相关抗体、肌炎及其他神经肌肉疾病的患病率——来自东欧一家三级中心的经验

Prevalence of Elevated CK Levels, Myositis-Specific and Myositis-Associated Antibodies, Myositis, and Other Neuromuscular Diseases in Myasthenia Gravis Patients-Experience from an Eastern European Tertiary Center.

作者信息

Kozák Márk, Kovács Edina, Nagy-Vince Melinda, Tóth Attila, Boczán Judit

机构信息

Department of Neurology, Faculty of Medicine, University of Debrecen, 22 Móricz Zsigmond krt., 4032 Debrecen, Hungary.

Department of Developmental Neurology, Szent Margit Budapest Hospital, 132 Bécsi út, 1032 Budapest, Hungary.

出版信息

J Clin Med. 2025 Apr 3;14(7):2449. doi: 10.3390/jcm14072449.

Abstract

Myasthenia gravis (MG) and idiopathic inflammatory myopathy (IIM) are autoimmune diseases that affect the musculoskeletal system. The association of the two diseases is rare. Their management is different, so it is important to recognize the concomitant presentation. In this cross-sectional study, we study the presence of CK elevation, myositis-specific and myositis-associated antibodies (MSA/MAA), and vitamin D levels in a cohort of 101 MG patients. Electromyography, limb magnetic resonance imaging (MRI), and, in some cases, muscle biopsy were performed when IIM was suspected. We reviewed the patients' medical records to access the results of these tests if they had been performed previously. CK elevation was detected in 10 patients (9.9%). We identified one case of anti-Jo-1 antibody-positive polymyositis and two cases of possible myositis. MSA/MAA antibodies were not found in the patients with high CK levels, except for the one with anti-Jo-1-positive IIM. One patient with elevated CK levels had an overlapping muscular dystrophy. MSA/MAA antibodies were detected in 19 patients (18.8%). A total of 37% had high-titer antibodies and concomitant systemic autoimmune diseases, while 63% had low-titer antibodies, most of whom had no systemic autoimmune disease. Low serum vitamin D levels were found in 67.3% of patients. Comparison of myasthenia gravis composite (MGC) scores between patients with low and normal vitamin D levels did not show a statistically significant difference. Our results may raise awareness among neuromuscular specialists caring for MG patients of the possibility of associated myositis or other neuromuscular diseases and the need to assess vitamin D levels. Although deficiency was frequent, its impact on MG severity remains unclear, necessitating further investigation into its immunological relevance.

摘要

重症肌无力(MG)和特发性炎性肌病(IIM)是影响肌肉骨骼系统的自身免疫性疾病。这两种疾病的关联较为罕见。它们的治疗方法不同,因此识别两者并存的表现很重要。在这项横断面研究中,我们研究了101例MG患者队列中的肌酸激酶(CK)升高情况、肌炎特异性抗体和肌炎相关抗体(MSA/MAA)以及维生素D水平。当怀疑有IIM时,进行了肌电图、肢体磁共振成像(MRI)检查,在某些情况下还进行了肌肉活检。如果患者之前已经进行过这些检查,我们会查阅其病历以获取检查结果。10例患者(9.9%)检测到CK升高。我们确定了1例抗Jo-1抗体阳性的多发性肌炎病例和2例可能的肌炎病例。除了1例抗Jo-1阳性的IIM患者外,CK水平高的患者未发现MSA/MAA抗体。1例CK水平升高的患者患有重叠性肌营养不良。19例患者(18.8%)检测到MSA/MAA抗体。共有37%的患者有高滴度抗体并伴有系统性自身免疫性疾病,而63%的患者有低滴度抗体,其中大多数没有系统性自身免疫性疾病。67.3%的患者血清维生素D水平较低。维生素D水平低和正常的患者之间的重症肌无力综合(MGC)评分比较未显示出统计学上的显著差异。我们的结果可能会提高诊治MG患者的神经肌肉专科医生对相关肌炎或其他神经肌肉疾病可能性的认识,以及评估维生素D水平的必要性。尽管维生素D缺乏很常见,但其对MG严重程度的影响仍不清楚,有必要进一步研究其免疫相关性。

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