Pemphigus relapse: Mechanisms, risk factors, and agents associated with disease recurrence.

Gaurav N Pathak, Priya Agarwal, Sydney M Wolfe, Kush H Patel, Jimmy Dhillon, Babar K Rao
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Abstract

Pemphigus represents a spectrum of potentially life-threatening autoimmune-mediated skin blistering conditions caused by antibody production against desmoglein 1 and 3 (anti-DSG 1 and 3) in keratinocytes. Greater than 50% of pemphigus patients experience relapse, which complicates long-term medical management, including risks associated with re-treatment and complications such as infection and dehydration. This review aims to elucidate mechanisms, risk factors, and medications associated with pemphigus relapse. Mechanisms of relapse include the persistence of auto-reactive B-cell populations post-treatment and CD20- B-cell populations that reactivate after B-cell depletion therapy. Risk factors for relapse include high body surface area (BSA) of pemphigus involvement, high body mass index, high severity according to the Pemphigus Disease Area Index (PDAI) at onset, treatment delay, and high anti-DSG1 and DSG3 titers post-treatment. Targeted B-cell localization is associated with better clinical outcomes, including less frequent relapses. Rituximab is currently the gold standard of treatment for moderate-severe pemphigus and has relapse rates of 11%-44% in selected studies, with a mean time to relapse of 5.8 months to 36 months following treatment. Relapse rates across lymphoma dosing (375 mg/m2) versus rheumatoid arthritis dosing (1 g dosing weekly) was inconsistent; however, more frequent dosing, earlier treatment, and higher cumulative dosing were associated with lower relapse rates. Alternative agents that have clinical efficacy include corticosteroid monotherapy, mycophenolate mofetil, azathioprine, and intravenous immunoglobulin. Future studies should include head-to-head comparators over long follow-up periods to identify the best treatment agents associated with the least relapse risk.

天疱疮复发:与疾病复发相关的机制、风险因素和药物。
丘疹性荨麻疹(Pemphigus)是一种由自身免疫介导的皮肤水疱病,由角质形成细胞中产生的抗去疱疹素 1 和 3(anti-DSG 1 和 3)抗体引起,具有潜在的生命危险。50%以上的丘疹性荨麻疹患者会复发,这使得长期医疗管理变得复杂,包括与再治疗相关的风险以及感染和脱水等并发症。本综述旨在阐明与丘疹性荨麻疹复发相关的机制、风险因素和药物。复发机制包括治疗后自身反应性B细胞群的持续存在,以及B细胞去势疗法后重新激活的CD20-B细胞群。复发的风险因素包括天疱疮累及体表面积(BSA)大、体重指数高、发病时天疱疮病区指数(PDAI)严重程度高、治疗延迟以及治疗后抗DSG1和DSG3滴度高。靶向 B 细胞定位与更好的临床疗效相关,包括更少复发。利妥昔单抗是目前治疗中度-重度丘疹性荨麻疹的金标准,在部分研究中,其复发率为11%-44%,平均复发时间为治疗后5.8个月至36个月。淋巴瘤用药(375 毫克/平方米)与类风湿性关节炎用药(每周 1 克)的复发率并不一致;不过,用药次数越多、治疗时间越早、累积用药量越大,复发率越低。具有临床疗效的替代药物包括皮质类固醇单药治疗、霉酚酸酯、硫唑嘌呤和静脉注射免疫球蛋白。未来的研究应包括长期随访的头对头比较研究,以确定复发风险最小的最佳治疗药物。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
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