TY - T1的表征与TS-HDS相关的临床和Paraclinical特征自身抗体血清阳性JF -神经学乔-神经病学SP - S71 LP - S71做10.1212/01. wnl.0000903568.79179.7c六世- 99 - 23补充2非首页盟-穆罕默德Rezk盟有约Mirman AU -莎拉Berini盟Pitcha Chompoopong AU -克里斯托弗·克莱因盟-约翰·r·米尔斯AU - Divyanshu Dubey Y1 - 2022/12/05 UR - //www.ez-admanager.com/content/99/23_Supplement_2/S71.1.abstract N2 -客观评估神经病变表型和临床结果与trisulfated肝素二糖(TS-HDS)自身抗体。背景TS-HDS自身抗体已被报道为免疫介导的神经病变的生物标志物。然而,研究评估的临床协会自身抗体是有限的。设计/方法综述了电子医疗记录识别TS-HDS自身抗体血清反应阳性的病人和描述他们的临床和电诊法的发现。结果七十七TS-HDS-IgM血清反应阳性的病人(效价范围9000 - 350000)被确定(50雌性;发病的年龄中位数是48年(9 - 83年)。11个病人还积极FGFR3-IgG(效价范围4000 - 19000)。70%(54/77)有临床/ paraclinical神经病变的证据(54/77,70%的TS-HDS-IgM孤独;10/11,91%的TS-HDS-IgM共存FGFR3-IgG)。管理医生特征的免疫介导的神经病变30%(23/77)和54%(6/11)的TS-HDS-IgM只和TS-HDS-IgM共存FGFR3-IgG血清反应阳性的患者,分别。小纤维神经病在58%(45/77)和63% (7/11)TS-HDS-IgM,分别和两个抗体血清反应阳性的患者。Length-dependent神经病变是最常见的神经病变表型在TS-HDS IgM(43/54, 80%)和双血清反应阳性的病例(7/11,63%)。 Forty-one (53%) patients received immunotherapy, predominantly: IVIG (n = 37), IV solumedrol (n = 7), oral prednisone (n = 14), and mycophenolate mofetil (n = 12). Among these, 43% (15/35) with TS-HDS-IgM seropositivity alone had improvement in inflammatory neuropathy cause and treatment (INCAT) disability score or modified Rankin Scale (mRS), while 33% (2/6) of patients with dual seropositivity had INCAT and mRS improvement. TS-HDS-IgM titers had low discriminative ability to identify immunotherapy response with an AUC of 0.621.Conclusions Neuropathy associations and clinical phenotypes amongst TS-HDS-IgM seropositive cases are variable. Furthermore, only a minority of cases are immunotherapy responsive, limiting the value of this biomarker in identifying immune-mediated neuropathies. ER -