神经肌肉接头疾病血清阴性重症肌无力基于聚集乙酰胆碱受体细胞的检测的临床需求。
The clinical need for clustered AChR cell-based assay testing of seronegative MG.
机构信息
Department of Neurology, Yale School of Medicine, New Haven, CT 06511, USA; Department of Immunobiology, Yale School of Medicine, New Haven, CT 06511, USA.
Neuromuscular Division, Department of Neurology, Duke University Medical Center, Durham, NC 27710, USA.
出版信息
J Neuroimmunol. 2022 Jun 15;367:577850. doi: 10.1016/j.jneuroim.2022.577850. Epub 2022 Mar 25.
Abstract
Trial eligibility in myasthenia gravis (MG) remains largely dependent on a positive autoantibody serostatus. This significantly hinders seronegative MG (SNMG) patients from receiving potentially beneficial new treatments. In a subset of SNMG patients, acetylcholine receptor (AChR) autoantibodies are detectable by a clustered AChR cell-based assay (CBA). Of 99 SNMG patients from two academic U.S. centers, 18 (18.2%) tested positive by this assay. Autoantibody positivity was further validated in 17/18 patients. In a complementary experiment, circulating AChR-specific B cells were identified in a CBA-positive SNMG patient. These findings corroborate the clinical need for clustered AChR CBA testing when evaluating SNMG patients.
摘要
在重症肌无力 (MG) 中，试验入选标准在很大程度上仍然依赖于自身抗体的阳性血清学状态。这极大地阻碍了血清阴性重症肌无力 (SNMG) 患者接受潜在有益的新治疗。在 SNMG 患者的亚组中，乙酰胆碱受体 (AChR) 自身抗体可通过聚集的 AChR 基于细胞的测定 (CBA) 检测到。在美国两个学术中心的 99 名 SNMG 患者中，有 18 名（18.2%）通过该检测呈阳性。在 17/18 名患者中进一步验证了抗体阳性。在一项补充实验中，在一名 CBA 阳性的 SNMG 患者中鉴定出了循环 AChR 特异性 B 细胞。这些发现证实了当评估 SNMG 患者时，进行聚集的 AChR CBA 检测具有临床必要性。
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