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利妥昔单抗治疗皮肌炎和其他炎性肌病。4 例报告及文献复习。

Rituximab in the treatment of dermatomyositis and other inflammatory myopathies. A report of 4 cases and review of the literature.

机构信息

Autoinmune Diseases Unit, Hospital Clínico San Cecilio, Granada, Spain.

出版信息

Clin Exp Rheumatol. 2009 Nov-Dec;27(6):1009-16.

PMID:20149324
Abstract

OBJECTIVE

Rituximab is an anti-CD20 monoclonal antibody targeting B cells, which has been used with success in a wide variety of autoimmune diseases. The experience with this drug in patients with inflammatory myopathies (IM), nonetheless, is still limited. We review the literature and highlight several aspects in relation to therapy with rituximab in IM.

METHODS

We performed a research in the MEDLINE DATABASE. All cases identified from the literature research and cases diagnosed in our Unit were included in the analysis.

RESULTS

We identified 49 patients with IM treated with rituximab in the review of the literature carried out (31 female; 18 male), including our patients. Dermatomyositis (DM) was the most common disorder for which rituximab treatment was administered (69.4%). The other diseases treated included polymyositis (PM) 16.3%, antisynthetase syndrome (AS) 8.2%, one case with anti-SRP-syndrome and other with juvenile dermatomyositis. The median time to diagnosis was 48 (0.75-480) months. Sixty-five per cent (65.3%) of patients presented with skin manifestations, 89.8% with muscle weakness, 7.3% with arthritis, 16.3% with interstitial lung disease, and 7.3% with cardiomyopathy. Seventy-one (71.4%) of the patients received only one course of rituximab, 18.4% two courses, 4.1% three, 2% four and only 4.1% five. We have observed both among our patients and those reported in the literature a high rate of response to rituximab, 75% of our patients and 72.5% of those described in the literature showed a good response. The median time free of symptoms between two courses was 12 (6-19) months. Rituximab was generally well tolerated by all patients, with no serious adverse events. Most of the adverse events reported were mainly infections, particularly respiratory tract infections.

CONCLUSIONS

It is our belief that rituximab may be an optimal therapeutic choice for inflammatory myopathies. Nevertheless, there is a need for additional studies in order to assess the optimal regimen of treatment in the different subsets, as well as the initial dose, combination of treatments and re-treatment schedule.

摘要

目的

利妥昔单抗是一种靶向 B 细胞的抗 CD20 单克隆抗体,已成功应用于多种自身免疫性疾病。然而,该药在炎性肌病(IM)患者中的应用经验仍然有限。我们复习文献并强调了与 IM 中利妥昔单抗治疗相关的几个方面。

方法

我们在 MEDLINE 数据库中进行了研究。从文献研究中确定的所有病例和在我们单位诊断的病例均纳入分析。

结果

我们在进行的文献复习中发现 49 例接受利妥昔单抗治疗的 IM 患者(31 例女性;18 例男性),包括我们的患者。皮肌炎(DM)是最常见的接受利妥昔单抗治疗的疾病(69.4%)。其他治疗的疾病包括多发性肌炎(PM)16.3%、抗合成酶综合征(AS)8.2%、1 例抗 SRP- 综合征和 1 例青少年皮肌炎。中位诊断时间为 48(0.75-480)个月。65%(65.3%)的患者有皮肤表现,89.8%有肌肉无力,7.3%有关节炎,16.3%有间质性肺病,7.3%有心肌病。71(71.4%)例患者仅接受 1 个疗程利妥昔单抗,18.4%接受 2 个疗程,4.1%接受 3 个疗程,2%接受 4 个疗程,只有 4.1%接受 5 个疗程。我们观察到,无论是在我们的患者还是在文献中报告的患者中,利妥昔单抗的反应率都很高,75%的患者和 72.5%的文献患者表现出良好的反应。两次疗程之间无症状的中位时间为 12(6-19)个月。所有患者均能很好地耐受利妥昔单抗,无严重不良事件。报告的大多数不良事件主要为感染,特别是呼吸道感染。

结论

我们认为利妥昔单抗可能是炎性肌病的一种最佳治疗选择。然而,需要进一步研究以评估不同亚组的最佳治疗方案,以及初始剂量、联合治疗和再治疗方案。

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