Mahmoud Ines, Jellouli Manel, Boukhris Imen, Charfi Rim, Ben Tekaya Aicha, Saidane Olfa, Ferjani Maryem, Hammi Yousra, Trabelsi Sameh, Khalfallah Narjess, Tekaya Rawdha, Gargah Tahar, Abdelmoula Leila
Department of Rheumatology, Charles Nicolle Hospital, Faculty of Medicine, Manar University, Tunis, Tunisia.
Department of Pediatric Nephrology, Charles Nicolle Hospital, Faculty of Medicine, Manar University, Tunis, Tunisia.
J Pediatr. 2017 Aug;187:213-219.e2. doi: 10.1016/j.jpeds.2017.05.002.
To evaluate the efficacy and safety of rituximab for treating pediatric systemic lupus erythematosus (pSLE).
We performed a systematic review to evaluate the efficacy and safety of rituximab in children with pSLE. Data from studies performed before July 2016 were collected from MEDLINE, the Cochrane Library, Scopus, and the International Rheumatic Disease Abstracts, with no language restrictions. Study eligibility criteria included clinical trials and observational studies with a minimal sample size of 5 patients, regarding treatment with rituximab in patients with refractory pSLE (aged <18 years at the time of diagnosis). Independent extraction of articles was performed by 2 investigators using predefined data fields.
Twelve case series met the criteria for data extraction for the systematic review with a good quality assessment according to an 18-criteria checklist using a modified Delphi method. Among them, 3 studies were multicenter and 3 were prospective. The total number of patients was 272. Studies collected patients with active disease refractory to steroids and immunosuppressant drugs. Refractory lupus nephritis was the most common indication (33%). Acceptable evidence suggested improvements in renal, neuropsychiatric and haematological manifestations, disease activity, complement and anti-double stranded Desoxy-Nucleo-Adenosine, with a steroid-sparing effect. However, there was poor evidence suggesting efficacy on arthralgia, photosensitivity, and mucocutaneous manifestations of SLE in children. An overall acceptable safety profile with few major adverse events was shown.
Rituximab exhibited a satisfactory profile regarding efficacy and safety indicating that this agent is a promising therapy for pSLE and should be further investigated.
评估利妥昔单抗治疗儿童系统性红斑狼疮（pSLE）的疗效和安全性。
我们进行了一项系统评价，以评估利妥昔单抗在pSLE患儿中的疗效和安全性。从MEDLINE、Cochrane图书馆、Scopus和国际风湿病文摘中收集了2016年7月之前进行的研究数据，无语言限制。研究纳入标准包括针对难治性pSLE（诊断时年龄<18岁）患者使用利妥昔单抗治疗的临床试验和观察性研究，最小样本量为5例患者。由2名研究人员使用预定义的数据字段独立提取文章。
根据使用改良德尔菲法的18项标准清单，12个病例系列符合系统评价的数据提取标准，质量评估良好。其中，3项研究为多中心研究，3项为前瞻性研究。患者总数为272例。研究收集了对类固醇和免疫抑制药物难治的活动性疾病患者。难治性狼疮性肾炎是最常见的适应症（33%）。可接受的证据表明，肾脏、神经精神和血液学表现、疾病活动度、补体和抗双链脱氧核糖核酸有所改善，具有类固醇节省效应。然而，证据不足表明对儿童SLE的关节痛、光敏性和皮肤黏膜表现有效。总体安全性良好，主要不良事件较少。
利妥昔单抗在疗效和安全性方面表现出令人满意的特征，表明该药物是一种有前途的pSLE治疗方法，应进一步研究。
