Dupilumab as a novel therapy for bullous pemphigoid: A multicenter case series
Dupilumab as a novel therapy for bullous pemphigoid: A multicenter case series
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DOI：
10.1016/j.jaad.2020.01.089
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发表时间：
2020-07-01
影响因子：
13.8
通讯作者：
Rosmarin, David
中科院分区：
文献类型：
--
作者：
Abdat, Rana;Waldman, Reid A.;Rosmarin, David
背景资料：大疱性类天疱疮（BP）是一种自身免疫性起泡性疾病，主要发生在老年人，缺乏足够的treatment.Objective：描述我们的经验，使用dupilumab在一系列患者BP.Methods：这是一个病例系列的患者从5个学术中心接受dupilumab BP。患者符合条件，如果他们有一个临床诊断的BP确诊的病变皮肤活检评估以下一个或多个：苏木精和伊红染色，直接免疫荧光，或酶联免疫吸附测定BP 180或BP 230，或两者兼而有之。患者的平均年龄为76.8岁，dupilumab治疗开始前BP的平均持续时间为28.8个月（范围：1-60个月）。92.3%（12/13）的患者实现了疾病清除或满意缓解。满意反应定义为临床医生记录疾病改善和患者希望继续用药，而没有记录疾病清除。53.8%（7/13）的患者实现了BP的完全清除。无不良事件报告。局限性：包括样本量小，缺乏对照组，缺乏标准化的评估工具，缺乏标准化的安全性监测。Dupilumab可能是BP的额外治疗，导致92.3%患者的疾病清除或令人满意的缓解，包括那些先前常规治疗失败的患者。
Background: Bullous pemphigoid (BP) is an autoimmune blistering disorder occurring mostly in the elderly that lacks adequate treatments.Objective: To describe our experience using dupilumab in a series of patients with BP.Methods: This is a case series of patients from 5 academic centers receiving dupilumab for BP. Patients were eligible if they had a clinical diagnosis of BP confirmed by lesional skin biopsy evaluated by one of more of the following: hematoxylin and eosin staining, direct immunofluorescence, or enzyme-linked immunosorbent assay for BP180 or BP230, or both.Results: We identified 13 patients. Patients were an average age of 76.8 years, and the average duration of BP before dupilumab initiation was 28.8 months (range, 1-60 months). Disease clearance or satisfactory response was achieved in 92.3% (12 of 13) of the patients. Satisfactory response was defined as clinician documentation of disease improvement and patient desire to stay on the medication without documentation of disease clearance. Total clearance of the BP was achieved in 53.8% (7 of 13) of patients No adverse events were reported.Limitations: Include small sample size, lack of a control group, lack of a standardized assessment tool, and lack of standardized safety monitoring.Conclusion: Dupilumab may be an additional treatment for BP, leading to disease clearance or satisfactory response in 92.3% of patients, including in those in whom previous conventional therapy had failed.