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利妥昔单抗治疗寻常型天疱疮和落叶型天疱疮的临床疗效评价。

Rituximab in practice: Clinical evaluation of patients with pemphigus after rituximab administration.

机构信息

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

Dana-Farber Cancer Institute, Harvard Medical School, Boston, Massachusetts, USA.

出版信息

Dermatol Ther. 2021 Jan;34(1):e14633. doi: 10.1111/dth.14633. Epub 2020 Dec 15.

Abstract

Pemphigus Vulgaris (PV) is a rare autoimmune blistering disease, which mainly causes mucosal and/or cutaneous lesions. In June 2018, FDA approved Rituximab (RTX)-a B-cell depleting agent-for the management of patients with moderate-to-severe pemphigus. Although the majority of patients respond well to this drug, some do not reach complete remission with a single cycle of RTX. In this review, following an overview of RTX and its clinical outcomes, we have focused on the possible outcomes after RTX therapy in patients with PV. The response is defined into four main categories; complete responders, partial responders, nonresponders, and paradoxical reactions, based on three possibilities of reaching the consolidation phase after 3 months, reaching remission until 6 months, and the ability of corticosteroid tapering in 6 months after RTX administration. Concerning the safety of RTX, three categories of infusion reactions, short and long-term side effects are discussed. Additionally, we have suggested approaches for the evaluation of clinical and serological responses at different critical time-points, including 1, 2, 3, and 6 months after RTX administration. Finally, available markers to predict the response to RTX and research gaps in the field of RTX therapy have been summarized.

摘要

寻常型天疱疮(PV)是一种罕见的自身免疫性水疱病,主要导致黏膜和/或皮肤损伤。2018 年 6 月,美国食品药品监督管理局(FDA)批准利妥昔单抗(RTX)-一种 B 细胞耗竭剂-用于治疗中重度天疱疮患者。尽管大多数患者对这种药物反应良好,但有些患者在接受单一周期的 RTX 治疗后未达到完全缓解。在这篇综述中,在概述了 RTX 及其临床结果后,我们重点关注了 PV 患者接受 RTX 治疗后的可能结果。根据 3 个月后达到巩固期、6 个月内达到缓解期以及 6 个月内皮质类固醇减量的能力,将反应分为完全缓解者、部分缓解者、无缓解者和矛盾反应者这四个主要类别。关于 RTX 的安全性,讨论了三种输注反应和短期及长期副作用类别。此外,我们还提出了在 RTX 给药后不同关键时间点(1、2、3 和 6 个月)评估临床和血清学反应的方法。最后,总结了预测 RTX 反应的可用标志物和 RTX 治疗领域的研究空白。

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