Vasculitis Research Unit, Department of Autoimmune Diseases, Hospital Clínic of Barcelona, University of Barcelona, Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), Barcelona, Spain.
Conective Tissue Diseases Unit, Department of Internal Medicine, Hospital Clínico Universitario de Salamanca, Salamanca, Spain.
Autoimmun Rev. 2020 Apr;19(4):102490. doi: 10.1016/j.autrev.2020.102490. Epub 2020 Feb 13.
Immunoglobulin A vasculitis (IgAV) is a systemic small vessel vasculitis for which treatment of severe cases is usually based on glucocorticoids and other conventional immunosuppressive drugs. The role of rituximab for resistant or refractory cases has been explored in isolated case reports and small series.
To perform a literature review of all pediatric and adult patients with IgAV treated with rituximab (RTX) and to assess disease characteristics, RTX efficacy and safety.
We conducted a systematic literature review according to PRISMA guidelines by selecting articles with information on IgAV and RTX up to October 2019. We extracted data on patient characteristics, disease course, RTX efficacy and tolerance. The resulting database was analyzed with statistical software package SPSS v 22.0.
Among the initial 161 articles found, 20 studies including 35 well-characterized IgAV patients treated with RTX were finally analyzed. Distribution by sex was similar, and the median age at diagnosis was 26 (range: 2 months to 70 years). Patients included were equally diagnosed at pediatric age and in the adulthood. Almost 90% of patients had renal involvement before RTX treatment and resistant or refractory disease to glucocorticoids or other immunosuppressive agents, mainly with renal impairment, was the reason for RTX administration in 85.7% of patients. RTX was used because of contraindication to these previous agents in 8.6% of patients, and as first line therapy in 5.7% of them. With regard to RTX response, 94.3% of patients presented clinical improvement of any type and 74.3% achieved sustained remission at the end of follow-up. Among the 13 (37.1%) patients who experienced a disease relapse, 11 (31.4%) were treated with a new RTX dose, with good disease control in all cases. In terms of treatment requirements, glucocorticoids and additional immunosuppressants were significantly lower after RTX administration. No deaths were observed and the rate of minor RTX-associated adverse effects was of 8.6%.
RTX seems to be a safe and useful agent in inducing disease remission and reducing previous immunosuppressive treatment in IgAV pediatric and adult patients resistant or refractory to glucocorticoids or other immunosuppressive drugs, and in those patients in whom these agents are contraindicated. Nevertheless, controlled clinical trials in are still warranted to clarify the role of RTX in IgAV.
免疫球蛋白 A 血管炎（IgAV）是一种系统性小血管血管炎，其严重病例的治疗通常基于糖皮质激素和其他常规免疫抑制剂。利妥昔单抗在耐药或难治性病例中的作用已在孤立病例报告和小系列中进行了探讨。
对所有接受利妥昔单抗（RTX）治疗的 IgAV 儿科和成人患者进行文献复习，评估疾病特征、RTX 疗效和安全性。
我们根据 PRISMA 指南进行了系统的文献综述，选择了截至 2019 年 10 月有关 IgAV 和 RTX 的信息的文章。我们提取了患者特征、疾病过程、RTX 疗效和耐受性的数据。使用统计软件包 SPSS v 22.0 分析所得数据库。
在最初发现的 161 篇文章中，最终分析了 20 项研究，其中包括 35 例经充分特征描述的接受 RTX 治疗的 IgAV 患者。性别分布相似，诊断时的中位年龄为 26 岁（范围：2 个月至 70 岁）。患者在儿科年龄和成年期同样被诊断出患有该疾病。几乎 90%的患者在接受 RTX 治疗前有肾脏受累，对糖皮质激素或其他免疫抑制剂耐药或难治是 85.7%患者接受 RTX 治疗的原因。由于这些先前药物的禁忌，8.6%的患者使用 RTX，5.7%的患者将其作为一线治疗。关于 RTX 反应，94.3%的患者出现任何类型的临床改善，74.3%的患者在随访结束时达到持续缓解。在 13 例（37.1%）疾病复发的患者中，11 例（31.4%）接受了新的 RTX 剂量治疗，所有病例的疾病均得到良好控制。在治疗需求方面，RTX 治疗后糖皮质激素和额外免疫抑制剂的用量显著降低。未观察到死亡，RTX 相关不良事件的发生率为 8.6%。
RTX 似乎是一种安全有效的药物，可诱导儿科和成年 IgAV 患者对糖皮质激素或其他免疫抑制剂耐药或难治性疾病缓解，并减少先前的免疫抑制治疗，对于这些药物有禁忌的患者也是如此。然而，仍需要进行对照临床试验，以明确 RTX 在 IgAV 中的作用。
