PDF(Pubmed)
Abstract：
Background and Objectives: Rituximab (RTX) has been the predominant treatment for autoimmune bullous diseases (AIBDs). The objective of this research was to assess the advantages and safety characteristics of RTX treatment in individuals with AIBD. This assessment focused on clinical remission and a reduction in glucocorticosteroid usage, its effect on the titers of autoantibodies targeting desmoglein-1 (DSG-1) and desmoglein-3 (DSG-3), and adverse occurrences during a 12-month follow-up period in a dermatology department within a Central European university context. Materials and Methods: Our case series involved eleven patients, including eight patients with pemphigus vulgaris, two with pemphigus foliaceus, and one with epidermolysis bullosa acquisita. They received a 1 g dose of rituximab, repeated over a two-week interval. Results: The reduction in a prednisone-equivalent dosage after 2, 6, and 12 months following the second RTX infusion was 65.05%, 73.99%, and 76.93%, in that order. The titers of antibodies against DSG-1 exhibited reductions of 43.29%, 75.86%, and 54.02% at 2, 6, and 12 months, respectively. By contrast, the antibody concentrations targeting DSG-3 displayed a decrease of 27.88%, 14.48%, and 5.09% at the corresponding time points. Over the course of the 12-month monitoring period, 18.18% of patients experienced disease relapse, while the remaining individuals achieved either complete or partial remission with minimal or no therapy. Adverse effects were noted in 36.36% of the patient population; they were mild, and no serious adverse effects were reported. Conclusions: RTX represents an efficacious and well-tolerated therapeutic option for the management of AIBD and merits consideration in cases of refractory AIBD. However, further research is imperative to delineate the most optimal dosage, dosing frequency, and total quantity of maintenance infusions required. Additionally, there is a compelling need for studies that explore the impact of RTX on individuals with AIBD who do not exhibit a significant reduction in anti-desmoglein autoantibody levels.
摘要：
背景与目的：利妥昔单抗（RTX）已成为自身免疫性大疱性疾病（AIBDs）的主要治疗药物。这项研究的目的是评估RTX治疗AIBD患者的优势和安全性特征。这项评估的重点是临床缓解和糖皮质激素使用的减少，其对靶向桥粒蛋白-1（DSG-1）和桥粒蛋白-3（DSG-3）的自身抗体滴度的影响，以及在中欧大学背景下的皮肤科进行的12个月随访期间的不良事件。材料与方法：我们的病例系列涉及11例患者，包括8名寻常型天疱疮患者,两个患有天疱疮，还有一个患有大疱性表皮松解症。他们接受了1克剂量的利妥昔单抗，在两周的间隔内重复。结果：第二次RTX输注后2、6和12个月泼尼松等效剂量的减少为65.05%，73.99%,76.93%，按这个顺序。抗DSG-1抗体的滴度降低了43.29%,75.86%,在2个月、6个月和12个月时为54.02%，分别。相比之下,靶向DSG-3的抗体浓度下降了27.88%，14.48%,和相应时间点的5.09%。在12个月的监测期间，18.18%的患者出现疾病复发,而其余个体在最小或不接受治疗的情况下实现了完全或部分缓解。36.36％的患者出现不良反应；它们是轻度的，未报告严重不良反应.结论：RTX是治疗AIBD的有效且耐受性良好的治疗选择，在难治性AIBD的情况下值得考虑。然而,进一步的研究是必要的，以划定最佳剂量，给药频率,以及所需的维持输液总量。此外,迫切需要进行研究,探索RTX对AIBD患者的影响,这些患者的抗桥粒蛋白自身抗体水平没有显著降低.
