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系统性硬皮病患者接受托珠单抗治疗的结果:病例系列和文献复习。

Outcomes of patients with systemic sclerosis treated with tocilizumab: Case series and review of the literature.

机构信息

Department of Medicine E, Meir Hospital, Kfar Saba, Israel; Sackler School of Medicine, University of Tel Aviv, Israel.

Department of Medicine E, Meir Hospital, Kfar Saba, Israel; Sackler School of Medicine, University of Tel Aviv, Israel.

出版信息

Best Pract Res Clin Rheumatol. 2018 Aug;32(4):563-571. doi: 10.1016/j.berh.2019.01.011. Epub 2019 Feb 21.

Abstract

The treatment of systemic sclerosis (SSc) presents a clinical challenge because of the progressive nature of the disease, relatively poor prognosis, and lack of a proven treatment. In the last 10 years, several studies demonstrated the importance of interleukin 6 (IL6) as a pivotal cytokine in the development of fibrosis and angiopathy, especially in SSc. Tocilizumab, an IL6 receptor antibody, has shown promising results for patients with SSc. A total of 16 patients with SSc were treated with tocilizumab; 14 were female and 2 were male, with a median age of 45.5 years and median disease duration of 31.5 months. Ten patients had anti-SCl-70, none had anticentromere, and two had antipolymerase. Tocilizumab treatment was provided as long as the patient's condition improved. Total treatment duration was 30.33 patient-years. Median treatment duration was 18.5 months, and 3 patients were treated for a period of 4 years and longer. Ten patients were treated with tocilizumab to the date of data collection. All were feeling good and maintained the achieved improvement throughout the treatment period. Improvement was recorded in 12 patients (75%). Mean reduction in modified Rodnan skin score was 11 points (p < 0.001), musculoskeletal and joint involvement improved in 75% and 80% of patients, respectively, and improvement in lung function was recorded in 46%. Patients with early SSc responded better to tocilizumab (p = 0.01). This is the largest reported case series of tocilizumab treatment in patients with SSc. The treatment was without significant side-effects and was beneficial for most patients, especially in early disease. The present study reinforces previous findings regarding the efficacy of tocilizumab in treating SSc.

摘要

系统性硬化症 (SSc) 的治疗具有挑战性,因为该疾病具有进行性、预后相对较差,并且缺乏已证实的治疗方法。在过去的 10 年中,有几项研究表明白细胞介素 6 (IL6) 作为纤维化和血管病变发展的关键细胞因子的重要性,尤其是在 SSc 中。IL6 受体抗体托珠单抗已显示出对 SSc 患者有希望的效果。共 16 例 SSc 患者接受了托珠单抗治疗;14 例为女性,2 例为男性,中位年龄为 45.5 岁,中位疾病持续时间为 31.5 个月。10 例患者抗 SCl-70 阳性,无抗着丝粒,2 例抗聚合酶。只要患者病情改善,就提供托珠单抗治疗。总治疗时间为 30.33 患者年。中位治疗持续时间为 18.5 个月,3 例患者治疗时间超过 4 年。截至数据收集日期,10 例患者仍在接受托珠单抗治疗。所有患者感觉良好,并在整个治疗期间保持所获得的改善。12 例患者(75%)记录到改善。改良 Rodnan 皮肤评分平均降低 11 分(p<0.001),肌肉骨骼和关节受累分别在 75%和 80%的患者中改善,肺功能改善在 46%的患者中记录到。早期 SSc 患者对托珠单抗的反应更好(p=0.01)。这是迄今为止报道的托珠单抗治疗 SSc 患者的最大病例系列。该治疗没有明显的副作用,对大多数患者有益,尤其是在早期疾病中。本研究加强了之前关于托珠单抗治疗 SSc 疗效的发现。

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