Multicenter prospective study of the humoral autoimmune response in bullous pemphigoid
Multicenter prospective study of the humoral autoimmune response in bullous pemphigoid
复制标题
DOI：
10.1016/j.clim.2008.04.012
复制
发表时间：
2008-09-01
影响因子：
8.6
通讯作者：
Hertl, Michael
中科院分区：
文献类型：
--
作者：
Di Zenzo, Giovanni;Thoma-Uszynski, Sybille;Hertl, Michael
大疱性类天疱疮 (BP) 是一种自身免疫性大疱性疾病，与针对半桥粒成分 BP180 和 BP230 的自身抗体相关。在这项研究中，不同的实验室首次分析了同一组 49 名前瞻性招募的 BP 患者的自身抗体谱。结果表明：1) 疾病严重程度和活动性与针对 BP180-NC16A 结构域的 IgG 水平相关，而且与针对 BP180 COOH 末端表位的 IgG 水平相关，2) BP180 胞外域的不同表位 BP180-NC16A 被 96% 的 BP 血清识别； 3)联合使用BP180和BP230 ELISA导致在所有BP血清中检测到IgG自身抗体。这些结果证明了基于各种 BP180 和 BP230 片段的组合 ELISA 在建立 BP 诊断中的有用性，并支持 BP180 是 BR (C) 2008 Elsevier Inc. 的主要自身抗原的概念。保留所有权利。
Bullous pemphigoid (BP) is an autoimmune bullous disease, associated with autoantibodies directed against the hemidesmosomal components BP180 and BP230. In this study for the first time different laboratories have analyzed the autoantibody profile in the same group of 49 prospectively recruited BP patients.The results show that: 1) disease severity and activity correlated with levels of IgG against the BP180-NC16A domain, but also against a COOH-terminal epitope of BP180, 2) distinct epitopes of the BP180 ectodomain other than BP180-NC16A were recognized by 96% of the BP sera; and 3) the combined use of BP180 and BP230 ELISA led to the detection of IgG autoantibodies in all the BP sera. These results demonstrate the usefulness of the combined ELISAs based on various BP180 and BP230 fragments in establishing the diagnosis of BP and support the concept that BP180 is the major autoantigen of BR (C) 2008 Elsevier Inc. All rights reserved.