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抗 cN1A 抗体与散发型包涵体肌炎更严重的吞咽困难相关。

Anti-cN1A Antibodies Are Associated with More Severe Dysphagia in Sporadic Inclusion Body Myositis.

机构信息

Fondazione Policlinico Universitario Agostino Gemelli IRCCS, 00168 Roma, Italy.

Department of Neurosciences, Section of Neurology, Università Cattolica del Sacro Cuore, 00168 Roma, Italy.

出版信息

Cells. 2021 May 10;10(5):1146. doi: 10.3390/cells10051146.

Abstract

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.

摘要

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。

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