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利妥昔单抗对乙酰胆碱受体抗体和肌肉特异性激酶抗体阳性的重症肌无力患者的持续疗效。

Sustained response to Rituximab in anti-AChR and anti-MuSK positive Myasthenia Gravis patients.

作者信息

Illa Isabel, Diaz-Manera Jordi, Rojas-Garcia Ricard, Pradas Jesus, Rey Antonio, Blesa Rafael, Juarez Candido, Gallardo Eduard

机构信息

Department of Neurology, Neuromuscular Unit, Hospital Santa Creu i Sant Pau, Universitat Autònoma Barcelona, Barcelona, Spain.

出版信息

J Neuroimmunol. 2008 Sep 15;201-202:90-4. doi: 10.1016/j.jneuroim.2008.04.039. Epub 2008 Jul 23.

Abstract

We report the results of treatment with Rituximab in six severe, non-responder MG patients. We treated three AChR+MG and three MuSK+MG patients, representing 2% and 20% of the respective groups of our series. Patients were assessed according to the Myasthenia Gravis Foundation of America (MGFA) recommendations. Antibody titers to AChR and MuSK, Ig levels, and IgG subclasses, were tested before treatment and during a follow-up of 9-22 months. All patients, one class V and five class IVB, improved dramatically, with no side effects. Antibody titers declined in all patients (p=0.006). The decline was significantly better in MuSK+MG patients at 9 months (p=0.046) and correlated with a more sustained clinical improvement. We did not find any significant changes in IgG4 that could explain the different outcome observed between these two groups.

摘要

我们报告了利妥昔单抗治疗6例重症、无反应性重症肌无力(MG)患者的结果。我们治疗了3例抗乙酰胆碱受体(AChR)阳性MG患者和3例肌肉特异性酪氨酸激酶(MuSK)阳性MG患者,分别占我们系列中各自组别的2%和20%。根据美国重症肌无力基金会(MGFA)的建议对患者进行评估。在治疗前以及9至22个月的随访期间,检测了抗AChR和MuSK的抗体滴度、免疫球蛋白(Ig)水平以及IgG亚类。所有患者,1例V级和5例IVB级,均显著改善,且无副作用。所有患者的抗体滴度均下降(p = 0.006)。在9个月时,MuSK阳性MG患者的抗体滴度下降更为显著(p = 0.046),且与更持久的临床改善相关。我们未发现IgG4有任何显著变化可以解释这两组之间观察到的不同结果。

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