Division of Dermatology, Rabin Medical Center, Petah Tikva, Israel.
Sackler School of Medicine, Tel Aviv University, Tel Aviv, Israel.
Front Immunol. 2023 Apr 27;14:1157250. doi: 10.3389/fimmu.2023.1157250. eCollection 2023.
Bullous pemphigoid (BP) is the most common autoimmune subepidermal bullous disease. Topical or systemic corticosteroids are often used as the first-line treatment. However, long-term corticosteroid use may lead to significant side effects. Therefore, various adjuvant immunosuppressant therapies are used as steroid-sparing agents, with accumulating reports of biological treatments for severely recalcitrant BP.
To describe the clinical and immunological features of a series of patients with recalcitrant BP treated with immunobiological therapies. To assess the efficacy and safety of their therapies.
Patients receiving biological treatment for BP from two centers were assessed. Here, we described the clinical, immunopathological, and immunofluorescence findings of adult patients with BP and analyzed the clinical response and adverse events associated with various biological therapies.
We identified nine eligible patients treated with rituximab (seven), omalizumab (three), or dupilumab (one). The mean age at diagnosis was 60.4 years, the average BP duration before biologic initiation was 1.9 years, and the average previous treatment failure was 2.11 therapies. The mean follow-up period from the first biological treatment to the last visit was 29.3 months. Satisfactory response, defined as clinical improvement, was achieved in 78% (7) of the patients, and total BP clearance was achieved in 55% (5) of the patients at the last follow-up visit. Additional rituximab courses improved the disease outcomes. No adverse events were reported.
Efficient and safe novel therapies can be considered in recalcitrant steroid-dependent BP non-responsive to conventional immunosuppressant therapies.
大疱性类天疱疮（BP）是最常见的自身免疫性表皮下大疱病。局部或全身皮质类固醇常作为一线治疗。然而，长期使用皮质类固醇可能会导致明显的副作用。因此，各种辅助免疫抑制剂治疗被用作皮质类固醇节约剂，并且有越来越多的关于严重难治性 BP 的生物治疗的报道。
描述一系列接受免疫生物治疗的难治性 BP 患者的临床和免疫学特征。评估他们治疗的疗效和安全性。
评估来自两个中心接受生物治疗 BP 的患者。在这里，我们描述了成人 BP 患者的临床、免疫病理学和免疫荧光检查结果，并分析了各种生物治疗相关的临床反应和不良事件。
我们确定了 9 名符合条件的患者，他们接受了利妥昔单抗（7 名）、奥马珠单抗（3 名）或度普利尤单抗（1 名）治疗。诊断时的平均年龄为 60.4 岁，开始生物治疗前的平均 BP 病程为 1.9 年，平均先前治疗失败为 2.11 种治疗方法。从第一次生物治疗到最后一次就诊的平均随访时间为 29.3 个月。78%（7）的患者达到了满意的反应，定义为临床改善，55%（5）的患者在最后一次随访时达到了 BP 完全清除。额外的利妥昔单抗疗程改善了疾病结局。没有报告不良事件。
在常规免疫抑制剂治疗反应不佳的难治性、皮质类固醇依赖的 BP 中，可以考虑使用高效且安全的新型治疗方法。
