Fondazione Policlinico Universitario Agostino Gemelli IRCCS, 00168 Roma, Italy.
Department of Neurosciences, Section of Neurology, Università Cattolica del Sacro Cuore, 00168 Roma, Italy.
Cells. 2021 May 10;10(5):1146. doi: 10.3390/cells10051146.
In recent years, an autoantibody directed against the 5'-citosolic nucleotidase1A (cN1A) was identified in the sera of sporadic inclusion body myositis (s-IBM) patients with widely variable sensitivity (33%-76%) and specificity (87%-100%). We assessed the sensitivity/specificity of anti-cN1A antibodies in an Italian cohort of s-IBM patients, searching for a potential correlation with clinical data. We collected clinical data and sera from 62 consecutive s-IBM patients and 62 other inflammatory myopathies patients. Testing for anti-cN1A antibodies was performed using a commercial ELISA. Anti-cN1A antibodies were detected in 23 s-IBM patients, resulting in a sensitivity of 37.1% with a specificity of 96.8%. Positive and negative predictive values were 92.0% and 60.6%, respectively. We did not find significant difference regarding demographic variables, nor quadriceps or finger flexor weakness. Nevertheless, we found that anti-cN1A-positive patients presented significantly lower scores in IBMFRS item 1 (swallowing, = 0.045) and more frequently reported more severe swallowing problems, expressed as an IBMFRS item 1 score ≤ 2 ( < 0.001). We confirmed the low sensitivity and high specificity of anti-cN1A Ab in s-IBM patients with a high positive predictive value. The presence of anti-CN1A antibodies identified patients with a greater risk of more severe dysphagia.
近年来,在散发性包涵体肌炎(s-IBM)患者的血清中发现了一种针对 5'-胞浆核苷酸酶 1A(cN1A)的自身抗体,其敏感性(33%-76%)和特异性(87%-100%)差异很大。我们评估了抗 cN1A 抗体在意大利 s-IBM 患者队列中的敏感性/特异性,寻找与临床数据的潜在相关性。我们收集了 62 例连续 s-IBM 患者和 62 例其他炎性肌病患者的临床数据和血清。使用商业 ELISA 检测抗 cN1A 抗体。在 23 例 s-IBM 患者中检测到抗 cN1A 抗体,敏感性为 37.1%,特异性为 96.8%。阳性预测值和阴性预测值分别为 92.0%和 60.6%。我们没有发现抗 cN1A 阳性患者在人口统计学变量、股四头肌或手指屈肌无力方面有显著差异。然而,我们发现抗 cN1A 阳性患者在 IBMFRS 项目 1(吞咽, = 0.045)的评分明显较低,并且更频繁地报告更严重的吞咽问题,表现为 IBMFRS 项目 1 评分≤2(<0.001)。我们证实了抗 cN1A 抗体在 s-IBM 患者中的低敏感性和高特异性,具有较高的阳性预测值。抗-CN1A 抗体的存在确定了吞咽困难更严重的患者风险更高。