Department of Dermatology, University of Lübeck, Lübeck, Germany.
Lübeck Institute of Experimental Dermatology (LIED), University of Lübeck, Lübeck, Germany.
Br J Dermatol. 2018 Oct;179(4):918-924. doi: 10.1111/bjd.16553. Epub 2018 Jul 5.
Bullous pemphigoid (BP) is a subepidermal blistering disease characterized by autoantibodies against the two hemidesmosomal proteins, BP180 (type XVII collagen) and BP230. The multicentre prospective BLISTER (Bullous Pemphigoid Steroids and Tetracyclines) trial randomized 253 patients with BP to compare the benefits and harms between initial treatment with doxycycline or prednisolone.
To analyse distinct autoantibody profiles for the prediction of the disease course in a well-characterized cohort of BP sera.
One hundred and forty-three patients of the BLISTER trial consented to participate in this serological study. Sera taken at baseline were analysed by (i) indirect immunofluorescence, (ii) anti-BP180 NC16A (16th noncollagenous domain) and anti-BP230 enzyme-linked immunosorbent assay and (iii) immunoblotting with various substrates. Results were then linked with clinical parameters including age, Karnofsky score, number of blisters, related adverse events and mortality.
Disease activity correlated with immunoglobulin (Ig)G anti-BP180 levels but not with levels of anti-BP230 IgG and anti-BP180 IgE. High levels of both anti-BP180 IgG and anti-BP230 IgG were associated with a low Karnofsky score. The presence of anti-BP230 IgG was more frequent in older patients. Those with higher total IgE serum levels suffered from fewer adverse events. Higher IgG anti-BP180 levels were associated with an increased 1-year mortality rate.
Analysis of the autoantibody profile is not only of diagnostic relevance but may also be helpful in predicting the course of the disease.
大疱性类天疱疮（BP）是一种表皮下水疱性疾病，其特征为自身抗体针对两种半桥粒蛋白，即 BP180（XVII 型胶原）和 BP230。多中心前瞻性 BLISTER（大疱性类天疱疮类固醇和四环素）试验将 253 例 BP 患者随机分为两组，比较初始使用多西环素或泼尼松龙的疗效和安全性。
分析在经过良好特征描述的 BP 血清队列中，不同的自身抗体谱对疾病过程的预测价值。
BLISTER 试验中有 143 例患者同意参与这项血清学研究。在基线时采集的血清通过（i）间接免疫荧光法，（ii）抗-BP180 NC16A（第 16 个非胶原结构域）和抗-BP230 酶联免疫吸附试验，以及（iii）用各种底物进行免疫印迹法进行分析。结果与临床参数相关联，包括年龄、Karnofsky 评分、水疱数、相关不良事件和死亡率。
疾病活动与 IgG 型抗-BP180 水平相关，但与抗-BP230 IgG 和抗-BP180 IgE 水平无关。高 IgG 型抗-BP180 和抗-BP230 IgG 水平与低 Karnofsky 评分相关。高龄患者更常出现抗-BP230 IgG。总 IgE 血清水平较高的患者不良事件较少。较高的 IgG 型抗-BP180 水平与 1 年死亡率增加相关。
自身抗体谱分析不仅具有诊断意义，而且可能有助于预测疾病的病程。
