Rituximab therapy for myopathy associated with anti-signal recognition particle antibodies: a case series.
Rituximab therapy for myopathy associated with anti-signal recognition particle antibodies: a case series.
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DOI：
10.1002/acr.20219
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发表时间：
2010-09
影响因子：
4.7
通讯作者：
Christopher-Stine, Lisa
中科院分区：
文献类型：
--
作者：
Valiyil, Ritu;Casciola-Rosen, Livia;Hong, Grace;Mammen, Andrew;Christopher-Stine, Lisa
与抗信号识别颗粒（SRP）相关的肌病是一种严重的坏死性免疫介导的疾病，其特征是快速进行性近端肌无力，血清肌酸激酶（CK）水平显著升高，对传统免疫抑制治疗的反应性差。关于B细胞耗竭疗法治疗抗SRP相关肌病的疗效的报道不一。我们描述了8例与抗SRP相关的肌病和他们的反应与抗CD 20单克隆抗体利妥昔单抗治疗。我们通过免疫沉淀法确定了8名抗SRP抗体检测呈阳性的肌病患者，并作为临床护理的一部分接受了利妥昔单抗治疗。我们回顾了他们的医疗记录，以评估临床、血清学和组织学特征以及对治疗的反应。在5例患者中，血清收集之前和之后利妥昔单抗治疗。通过免疫沉淀法检测自身抗体，并通过光密度法定量，计算抗SRP自身抗体水平的下降百分比。8例标准免疫抑制治疗难治性患者中有6例在利妥昔单抗治疗后2个月就表现出手部肌肉力量改善和/或CK水平下降。3例患者在首次给药后持续反应12至18个月。所有患者均继续使用糖皮质激素，但在利妥昔单抗治疗后剂量大幅减少。利妥昔单抗治疗后血清抗SRP抗体的定量水平也降低。利妥昔单抗的B细胞耗竭疗法对抗SRP相关肌病患者有效。利妥昔单抗治疗后抗SRP抗体水平的显著降低也表明B细胞和抗SRP抗体可能在该肌病的发病机制中起作用。
The myopathy associated with anti-signal recognition particle (SRP) is a severe necrotizing immune-mediated disease characterized by rapidly progressive proximal muscle weakness, markedly elevated serum creatine kinase (CK) levels, and poor responsiveness to traditional immunosuppressive therapies. Reports on the efficacy of B cell depletion therapy for anti-SRP associated myopathy are mixed. We describe eight patients with anti-SRP associated myopathy and their response to treatment with the anti-CD20 monoclonal antibody rituximab. We identified eight patients with myopathy who tested positive for anti-SRP antibodies by immunoprecipitation and were treated with rituximab as part of clinical care. We reviewed their medical records to assess clinical, serologic, and histologic characteristics and response to therapy. In five patients, serum was collected before and after rituximab therapy. Autoantibodies were detected by immunoprecipitation and quantitated by densitometry, and the percent decreases in anti-SRP autoantibody levels were calculated. Six of eight patients who had been refractory to standard immunosuppressive therapy demonstrated improved manual muscle strength and/or decline in CK levels as early as two months after rituximab treatment. Three patients sustained the response for twelve to eighteen months after initial dosing. All patients were continued on adjunctive corticosteroids, but dosages were substantially reduced after rituximab. Quantitative levels of serum anti-SRP antibodies also decreased after rituximab treatment. B cell depletion therapy with rituximab is effective for patients with myopathy associated with anti-SRP. The substantial decrease in anti-SRP antibody levels after rituximab treatment also suggests that B cells and anti-SRP antibodies may play a role in the pathogenesis of this myopathy.
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影响因子：
27.4
作者：
Brouwer, R;Hengstman, GJD;van Venrooij, WJ
通讯作者：
van Venrooij, WJ
影响因子：
27.4
作者：
Hengstman, G. J. D.;ter Laak, H. J.;van Engelen, B. G. M.
通讯作者：
van Engelen, B. G. M.
影响因子：
158.5
作者：
Edwards, JCW;Szczepanski, L;Shaw, T
通讯作者：
Shaw, T
影响因子：
--
作者：
Kao, AH;Lacomis, D;Oddis, CV
通讯作者：
Oddis, CV
影响因子：
158.5
作者：
BOHAN, A;PETER, JB
通讯作者：
PETER, JB