Parma University Hospital, Parma, Italy.
G. Bosco Hospital and University of Turin, Turin, Italy.
Arthritis Rheumatol. 2018 Jan;70(1):109-114. doi: 10.1002/art.40339. Epub 2017 Dec 1.
Adult-onset IgA vasculitis (Henoch-Schönlein) (IgAV) is a rare systemic vasculitis characterized by IgA1-dominant deposits. The treatment of adult-onset IgAV is controversial and is based on the combination of glucocorticoids and immunosuppressive agents, but many patients have refractory or relapsing disease despite treatment. Rituximab (RTX) is a B cell-depleting antibody of proven efficacy in antineutrophil cytoplasmic antibody-associated vasculitis. We undertook this study to test the efficacy and safety of RTX in a multicenter cohort of patients with adult-onset IgAV.
In this multicenter observational study, we included patients with adult-onset IgAV who had received RTX either for refractory/relapsing disease or because they had contraindications to conventional glucocorticoid/immunosuppressive therapy. We analyzed the rates of remission (defined on the basis of the Birmingham Vasculitis Activity Score [BVAS]) and relapse as well as the variations over time in estimated glomerular filtration rate (GFR), proteinuria, C-reactive protein (CRP) level, BVAS, and prednisone dose.
Twenty-two patients were included; their median duration of follow-up was 24 months (interquartile range 18-48 months). Sixteen patients received RTX as add-on therapy and 6 as monotherapy. Twenty patients (90.9%) achieved remission, and 7 of those 20 patients (35%) had subsequent relapse of disease. There were significant reductions in 24-hour proteinuria (P < 0.0001), CRP level (P = 0.0005), BVAS (P < 0.0001), and prednisone dose (P < 0.0001) from RTX initiation through the last follow-up visit; estimated GFR remained stable. RTX was generally well tolerated. One patient died after 60 months of follow-up.
Our data suggest that RTX is an effective and safe therapeutic option for adult-onset IgAV.
成人发病 IgA 血管炎（亨诺克-舍恩莱因）（IgAV）是一种罕见的系统性血管炎，其特征为 IgA1 为主的沉积物。成人发病 IgAV 的治疗存在争议，基于糖皮质激素和免疫抑制剂的联合治疗，但许多患者尽管接受治疗仍存在难治性或复发性疾病。利妥昔单抗（RTX）是一种已被证明在抗中性粒细胞胞质抗体相关性血管炎中有效的 B 细胞耗竭抗体。我们进行这项研究，旨在测试 RTX 在成人发病 IgAV 的多中心队列患者中的疗效和安全性。
在这项多中心观察性研究中，我们纳入了接受 RTX 治疗的成人发病 IgAV 患者，这些患者要么患有难治性/复发性疾病，要么对常规糖皮质激素/免疫抑制治疗有禁忌证。我们分析了缓解率（基于伯明翰血管炎活动评分 [BVAS] 定义）和复发率，以及估计肾小球滤过率（GFR）、蛋白尿、C 反应蛋白（CRP）水平、BVAS 和泼尼松剂量随时间的变化。
共纳入 22 例患者；中位随访时间为 24 个月（四分位距 18-48 个月）。16 例患者接受 RTX 作为附加治疗，6 例患者接受 RTX 作为单药治疗。20 例患者（90.9%）达到缓解，其中 20 例患者中的 7 例（35%）随后发生疾病复发。从 RTX 起始到最后一次随访，24 小时蛋白尿（P < 0.0001）、CRP 水平（P = 0.0005）、BVAS（P < 0.0001）和泼尼松剂量（P < 0.0001）均显著降低；估计 GFR 保持稳定。RTX 总体上耐受性良好。1 例患者在 60 个月随访后死亡。
我们的数据表明，RTX 是成人发病 IgAV 的一种有效且安全的治疗选择。
