Muscular Dystrophy Association Care Center, Department of Neuromuscular Medicine, Hospital for Special Care, New Britain, CT, USA.
Muscular Dystrophy Association Care Center, Department of Neuromuscular Medicine, Hospital for Special Care, New Britain, CT, USA.
Neuromuscul Disord. 2018 Aug;28(8):660-664. doi: 10.1016/j.nmd.2018.06.005. Epub 2018 Jun 18.
Sporadic inclusion body myositis (IBM) is the most common acquired myopathy affecting patients over age 50. The discovery of an autoantibody directed against a 43-44 kD protein (anti-cytosolic-5'-nucleotidase 1A or anti-cN1A) has provided support for the hypothesis of an immune-mediated pathogenesis. Previous studies have reported variable test sensitivity and specificity, and inconsistent results on the predictive value. In our cohort of 40 patients with clinico-pathologically or clinically defined IBM, we found the sensitivity of the anti-cN1A antibody test to be 50%. Comparing characteristics for test positive and test negative groups, we found that patients in our cohort testing positive for the anti-cN1A antibody were significantly more likely to be older than age 60 years at symptom onset. We found no positive association between anti-cN1A reactivity and other clinical, laboratory, and muscle histopathologic findings. Based on all clinical studies published to date including the present, the anti-cN1A antibody test shows high diagnostic specificity, moderate sensitivity, and a low predictive value in regards to age of onset, disease severity and other associated clinicopathological findings.
散发性包涵体肌炎(IBM)是最常见的获得性肌病,影响 50 岁以上的患者。针对一种针对 43-44kD 蛋白(抗细胞质 5'-核苷酸酶 1A 或抗 cN1A)的自身抗体的发现,为免疫介导的发病机制假说提供了支持。以前的研究报告了可变的测试敏感性和特异性,以及预测值的不一致结果。在我们的 40 名临床病理或临床定义的 IBM 患者队列中,我们发现抗 cN1A 抗体测试的敏感性为 50%。比较阳性和阴性组的特征,我们发现我们队列中抗 cN1A 抗体检测阳性的患者在症状发作时年龄明显大于 60 岁。我们没有发现抗 cN1A 反应性与其他临床、实验室和肌肉组织病理学发现之间存在阳性关联。基于迄今为止包括本研究在内的所有临床研究,抗 cN1A 抗体测试在发病年龄、疾病严重程度和其他相关临床病理发现方面显示出高诊断特异性、中等敏感性和低预测值。