Center for Blistering Diseases, Department of Medicine, New England Baptist Hospital, Boston, MA 02120, USA.
Clin Exp Immunol. 2010 Nov;162(2):224-36. doi: 10.1111/j.1365-2249.2010.04239.x.
In this report,we present 15 patients with histological and immunopathologically proven pemphigus vulgaris (PV). After a mean of 80 months since the onset of disease, when evaluated serologically, they had antibodies typical of PV and pemphigoid (Pg). Similarly, 18 patients with bullous pemphigoid (BP) and mucous membrane pemphigoid (MMP) were diagnosed on the basis of histology and immunopathology.After a mean of 60 months since the onset of disease, when their sera were evaluated they were found to have Pg and PV autoantibodies. In both groups of patients the diseases were characterized by a chronic course, which included several relapses and recurrences and were non-responsive to conventional therapy. The major histocompatibility complex class II (MHC II) genes were studied in both groups of patients and phenotypes associated typically with them were observed. Hence, in 33 patients, two different pathogenic autoantibodies were detected simultaneously. The authors provide a computer model to show that each MHC II gene has relevant epitopes that recognize the antigens associated with both diseases. Using the databases in these computer models, the authors present the hypothesis that these two autoantibodies are produced simultaneously due to the phenomena of epitope spreading.
在本报告中,我们介绍了 15 例组织学和免疫病理学证实的寻常型天疱疮(PV)患者。这些患者在发病后平均 80 个月时进行血清学评估,发现他们具有典型的 PV 和大疱性类天疱疮(Pg)抗体。同样,18 例大疱性类天疱疮(BP)和黏膜类天疱疮(MMP)患者也基于组织学和免疫病理学进行了诊断。这些患者在发病后平均 60 个月时进行血清学评估,发现他们具有 Pg 和 PV 自身抗体。在这两组患者中,疾病均表现为慢性病程,包括多次复发和缓解,且对常规治疗无反应。研究了两组患者的主要组织相容性复合体 II 类(MHC II)基因,并观察到与它们相关的典型表型。因此,在 33 例患者中同时检测到两种不同的致病性自身抗体。作者提供了一个计算机模型,表明每个 MHC II 基因都有相关的表位,可以识别与两种疾病相关的抗原。使用这些计算机模型中的数据库,作者提出了一个假说,即这两种自身抗体是由于表位扩展现象而同时产生的。