Department of Neurology, Mokdong Hospital, Ewha Womans University School of Medicine, Seoul, Republic of Korea.
Department of Neurology, Gangnam Severance Hospital, Yonsei University College of Medicine, Seoul, Republic of Korea.
PLoS One. 2023 Apr 14;18(4):e0284409. doi: 10.1371/journal.pone.0284409. eCollection 2023.
To explore the clinical significance of anti-cytosolic 5'-nucleoditase 1A (NT5c1A) antibody seropositivity in inflammatory myopathies, we measured anti-NT5c1A antibodies and analyzed their clinical features. Anti-NT5c1A antibodies were measured in the sera of 103 patients with inflammatory myopathies using an enzyme-linked immunosorbent assay. Positivity for anti-NT5c1A antibody was found in 13 (12.6%) of 103 patients with inflammatory myopathy. Anti-NT5c1A antibody was most frequently identified in patients with inclusion body myositis (IBM) (8/20, 40%), followed by dermatomyositis (2/13, 15.4%), immune-mediated necrotizing myopathy (2/28, 7.1%), and polymyositis (1/42, 2.4%). In eight patients with the anti-NT5c1A antibody-seropositive IBM, the median age at symptom onset was 54 years (interquartile range [IQR]: 48-57 years), and the median disease duration was 34 months (IQR: 24-50 months]. Knee extension weakness was greater than or equal to hip flexion weakness in eight (100%) patients, and finger flexion strength was less than shoulder abduction in three (38%) patients. Dysphagia symptoms were found in three (38%) patients. The median serum CK level was 581 IU/l (IQR: 434-868 IU/L]. Clinically significant differences were not found between anti-NT5c1A antibody-seropositive and seronegative IBM groups with respect to gender, age at symptom onset, age at diagnosis, disease duration, serum CK values, presence of other autoantibodies, dysphagia, and the pattern of muscle impairment. Although anti-NT5c1A antibody is known to be associated with IBM, seropositivity has also been noted in non-IBM inflammatory myopathies, and is insufficient to have clinical significance by itself. These findings have important implications for interpreting anti-NT5c1A antibody test results as the first study in Korea.
为了探究抗胞浆 5'-核苷酸酶 1A(NT5c1A)抗体阳性在炎性肌病中的临床意义,我们检测了抗-NT5c1A 抗体并分析了其临床特征。采用酶联免疫吸附试验检测了 103 例炎性肌病患者的血清抗-NT5c1A 抗体。在 103 例炎性肌病患者中,13 例(12.6%)抗-NT5c1A 抗体阳性。抗-NT5c1A 抗体在包涵体肌炎(IBM)患者中最常被检测到(8/20,40%),其次是皮肌炎(2/13,15.4%)、免疫介导性坏死性肌病(2/28,7.1%)和多发性肌炎(1/42,2.4%)。在 8 例抗-NT5c1A 抗体阳性的 IBM 患者中,症状发作的中位年龄为 54 岁(四分位间距 [IQR]:48-57 岁),中位病程为 34 个月(IQR:24-50 个月)。8 例患者中,膝伸肌无力大于或等于髋屈肌无力,3 例(38%)患者手指屈肌力量小于肩外展力量。3 例(38%)患者出现吞咽困难症状。中位血清肌酸激酶(CK)水平为 581IU/L(IQR:434-868IU/L)。在性别、症状发作年龄、诊断年龄、病程、血清 CK 值、是否存在其他自身抗体、吞咽困难以及肌肉损害模式方面,抗-NT5c1A 抗体阳性和阴性 IBM 组之间无显著差异。虽然抗-NT5c1A 抗体与 IBM 相关,但在非 IBM 炎性肌病中也有阳性报道,因此单凭其阳性不足以具有临床意义。这些发现对解释抗-NT5c1A 抗体检测结果具有重要意义,这是韩国的首次研究。