Department of Dermatology, The Second Affiliated Hospital, School of Medicine, Xi'an Jiaotong University, Xi'an, China.
Front Immunol. 2021 Aug 24;12:718073. doi: 10.3389/fimmu.2021.718073. eCollection 2021.
Bullous pemphigoid (BP) is the most common autoimmune subepidermal blistering disorder in the elderly. Systemic and topical use of glucocorticoids and immunosuppressants has been shown to be effective in most patients. However, refractory BP patients are challenged to clinicians with severe clinical symptoms, resistance to treatment, and high relapse rate. How to predict and assess the refractory and severity of bullous pemphigoid is the key issue in clinical practice, and the urgent need for precision medicine in refractory patients is driving the search for biomarkers and biologics. Recently, some biomarkers, such as the level of specific autoantibodies and released cytokines, have been proposed as the potential parameters to reflect the disease severity and predict the treatment response and relapse of refractory BP. Moreover, new biologics targeting pathogenic antibodies, complement, Th2 axis, eosinophils, and Th17 axis have shown potent efficacy on refractory BP. Here, we review the literature and give an overview of emerging biomarkers and therapeutic strategies for refractory bullous pemphigoid to improve the prognosis of the patient.
大疱性类天疱疮(BP)是老年人中最常见的自身免疫性表皮下疱病。全身性和局部性使用糖皮质激素和免疫抑制剂已被证明对大多数患者有效。然而,难治性 BP 患者给临床医生带来了严峻的挑战,他们具有严重的临床症状、对治疗的耐药性和高复发率。如何预测和评估大疱性类天疱疮的难治性和严重程度是临床实践中的关键问题,对难治性患者的精准医学的迫切需求推动了对生物标志物和生物制剂的探索。最近,一些生物标志物,如特定自身抗体的水平和释放的细胞因子,已被提出作为反映疾病严重程度、预测难治性 BP 治疗反应和复发的潜在参数。此外,针对致病性抗体、补体、Th2 轴、嗜酸性粒细胞和 Th17 轴的新型生物制剂已显示出对难治性 BP 的强大疗效。在这里,我们回顾了文献,概述了难治性大疱性类天疱疮的新兴生物标志物和治疗策略,以改善患者的预后。