Gornowicz-Porowska Justyna, Seraszek-Jaros Agnieszka, Bowszyc-Dmochowska Monika, Kaczmarek Elżbieta, Pietkiewicz Paweł, Bartkiewicz Paweł, Dmochowski Marian
Autoimmune Blistering Dermatoses Section, Department of Dermatology, Poznan University of Medical Sciences, Poznan, Poland.
Department of Bioinformatics and Computational Biology, Poznan University of Medical Sciences, Poznan, Poland.
Cent Eur J Immunol. 2017;42(1):85-90. doi: 10.5114/ceji.2017.67322. Epub 2017 May 8.
Recent studies postulated the association between bullous pemphigoid (BP) and neurodegenerative disorders (ND). The autoantibodies to BP180 and/or BP230 may be present not only in BP, but also in ND as neuronal isoforms of these proteins are identified in the central nervous system. However, there are only scant data about the precise pathogenetic mechanisms interlinking ND and BP as well as the immunologic profile in these patients. The aim is to analyze the serological immunopathological profiles (anti-BP180 IgG, anti-BP230 IgG) in BP patients with and without ND in order to identify the specific autoantibody(ies) and corresponding antigens responsible for ND development in BP patients. Altogether, 82 ethnic Poles with BP and their medical records were examined (62 BP-ND; 20 BP+ND). Levels of serum anti-BP180/BP230 IgG in BP patients were evaluated with ELISAs. The statistical analyses involved Pearson chi-squared test, Mann-Whitney U-test and ranking of autoantibodies. The prevalence of ND among BP patients was 24.4%. There were no statistically significant differences in autoantigens profiles (anti-BP180/anti-BP230 IgG) between BP+ND and BP-ND groups. There was no relationship between ND development and anti-BP180/anti-BP230 IgG level (p = 0.5933, p = 0.4701, respectively). The autoantibodies levels of BP+ND and BP-ND patients show insignificant differences suggesting that also in ethnic Poles a hypothetical pathogenetic association of BP and ND, but not only an aging-related epidemiological one, appears to be independent of a particular BP antigen. Nevertheless, it cannot be excluded that phenomena of epitopes spreading, immune cross-reaction and conformational changes in BP180/BP230 may underlie BP development in ND patients.
近期研究推测大疱性类天疱疮（BP）与神经退行性疾病（ND）之间存在关联。针对BP180和/或BP230的自身抗体不仅可能存在于BP患者中，在ND患者中也可能存在，因为在中枢神经系统中已鉴定出这些蛋白质的神经元异构体。然而，关于连接ND和BP的精确发病机制以及这些患者的免疫特征的数据却很少。目的是分析有或没有ND的BP患者的血清免疫病理特征（抗BP180 IgG、抗BP230 IgG），以确定导致BP患者发生ND的特定自身抗体和相应抗原。总共检查了82名患有BP的波兰族患者及其病历（62例BP-ND；20例BP+ND）。采用酶联免疫吸附测定法（ELISAs）评估BP患者血清抗BP180/BP230 IgG水平。统计分析采用Pearson卡方检验、Mann-Whitney U检验和自身抗体排名。BP患者中ND的患病率为24.4%。BP+ND组和BP-ND组之间的自身抗原谱（抗BP180/抗BP230 IgG）无统计学显著差异。ND的发生与抗BP180/抗BP230 IgG水平之间无相关性（p分别为0.5933和0.4701）。BP+ND和BP-ND患者的自身抗体水平显示出不显著差异，这表明在波兰族中，BP与ND之间假设的发病机制关联，而非仅与年龄相关的流行病学关联，似乎独立于特定的BP抗原。然而，不能排除BP180/BP230中表位扩展、免疫交叉反应和构象变化现象可能是ND患者发生BP的基础。
