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利妥昔单抗在儿童和青少年自身免疫性大疱性疾病中的一线和二线治疗:13 例病例系列。

Rituximab in childhood and juvenile autoimmune bullous diseases as first-line and second-line treatment: a case series of 13 patients.

机构信息

Autoimmune Bullous Diseases Research Center, Tehran University of Medical Sciences, Tehran, Iran.

出版信息

J Dermatolog Treat. 2022 Mar;33(2):869-874. doi: 10.1080/09546634.2020.1788702. Epub 2020 Jul 7.

DOI:10.1080/09546634.2020.1788702
PMID:32589481
Abstract

BACKGROUND

Little data are available concerning the outcome of rituximab (RTX) therapy in pediatric patients with autoimmune bullous diseases (AIBDs).

OBJECTIVE

We sought to evaluate safety and efficacy of RTX administration in pediatric patients with AIBDs and to assess first-line RTX therapy in pemphigus patients.

METHODS

AIBD patients consisting of 12 pemphigus patients and a patient with bullous pemphigoid who received RTX before the age of 18 were enrolled. Detailed information regarding patients' outcome after the first RTX cycle was assessed.

RESULTS

The mean age of the patients at RTX infusion was 15 ± 2 years. Six patients in the pemphigus group received RTX as first-line therapy. In pemphigus patients: complete remission (on minimal therapy) was achieved by seven patients, partial remission (on minimal therapy) and complete remission (off therapy) were achieved by three patients and one, respectively. Relapse occurred in nine patients, which were mostly mild. Likewise, the BP patient received RTX with a good clinical response. The observed adverse events were mostly mild infusion reactions and a case of sepsis.

CONCLUSION

Rituximab is safe and effective in childhood/juvenile patients with AIBDs. Furthermore, RTX can be used as first-line treatment in pediatric patients with pemphigus.

摘要

背景

关于儿童自身免疫性大疱性疾病(AIBD)患者接受利妥昔单抗(RTX)治疗的结果,相关数据较少。

目的

我们旨在评估 RTX 治疗儿科 AIBD 患者的安全性和有效性,并评估天疱疮患者的一线 RTX 治疗。

方法

本研究纳入了 12 名天疱疮患者和 1 名大疱性类天疱疮患者,这些患者在 18 岁之前接受了 RTX 治疗。评估了患者在首次 RTX 周期后的详细转归信息。

结果

RTX 输注时患者的平均年龄为 15±2 岁。天疱疮组中有 6 名患者接受 RTX 作为一线治疗。在天疱疮患者中:7 名患者达到完全缓解(接受最小剂量治疗),3 名患者达到部分缓解(接受最小剂量治疗)和完全缓解(停药),1 名患者达到完全缓解(停药)。9 名患者发生复发,大多为轻度。同样,BP 患者接受 RTX 治疗后获得了良好的临床反应。观察到的不良事件主要为轻度输注反应和 1 例脓毒症。

结论

RTX 治疗儿童/青少年 AIBD 患者是安全有效的。此外,RTX 可作为儿科天疱疮患者的一线治疗药物。

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