背景:大疱性类天疱疮(BP),最常见的表皮下自身免疫性起泡疾病,通常由针对半网间体蛋白BP180(XVII型胶原蛋白)和BP230的IgG自身抗体的存在以及皮肤病变的优势来定义。一些研究已经解决了抗BP180IgE在患者和实验模型中的作用,而抗BP230IgE的数据很少。
目的:通过ELISA评估BP血清中抗BP230IgE水平,并将其与疾病严重程度和临床特征相关联。
方法:BP血清进行抗BP230IgEELISA和针对人BP230片段的Western印迹。
结果:我们证明36/154(23%)的BP血清抗BP230IgE阳性。抗BP230IgE水平与临床表型或疾病活动本身无相关性。有趣的是,抗BP230IgE在疾病过程中与个体的疾病活动显著相关.此外,抗BP230IgE水平与总IgE水平呈显著相关。值得注意的是,抗BP230IgG与疾病活动相互关联。通过西方印迹,BP230片段的C端结构域(C2;氨基酸2024-2349和C3;氨基酸2326-2649),为抗BP230IgE检测提供了最好的血清学检测方法。
结论:作为补充工具,建议使用IgE免疫印迹法来获得最佳的血清学诊断,特别是通过BP180或BP230特异性ELISA没有IgG反应性的严重疾病患者。尽管血清抗BP230IgE的检测没有主要的诊断意义,它可能与治疗决策有关,例如,用于抗IgE定向治疗,已成功用于BP案例系列。
BACKGROUND: Bullous pemphigoid (BP), the most common subepidermal autoimmune blistering disease, is classically defined by the presence of IgG autoantibodies directed against the hemidesmosomal proteins BP180 (type XVII collagen) and BP230 and the predominance of skin lesions. Several studies have addressed the role of anti-BP180 IgE in patients and experimental models, while data on anti-BP230 IgE are scarce.
OBJECTIVE: To assess anti-BP230 IgE level by ELISA in BP sera and to correlate it with disease severity and clinical characteristics.
METHODS: BP sera underwent anti-BP230 IgE ELISA and Western blotting against human BP230 fragments.
RESULTS: We demonstrate that 36/154 (23%) of BP sera were positive for anti-BP230 IgE. Anti-BP230 IgE levels had no correlation with clinical phenotype or disease activity per se. Interestingly, anti-BP230 IgE was significantly associated with disease activity within individuals during the course of the disease. Additionally, anti-BP230 IgE and total IgE levels showed a significant correlation. Notably, anti-BP230 IgG correlated interindividually with disease activity. By Western blotting, the C-terminal domain of BP230 fragments (C2; amino acids 2024-2349 and C3; amino acids 2326-2649), provided the best serological assay for anti-BP230 IgE detection.
CONCLUSIONS: As a complementary tool, IgE immunoblotting is recommended to obtain an optimal serological diagnosis, particularly in patients with severe disease without IgG reactivity by BP180- or BP230-specific ELISA. Although the detection of serum anti-BP230 IgE is not of major diagnostic significance, it may be relevant for therapeutic decisions, e.g., for anti-IgE-directed treatment, which has been successfully used in case series of BP.