Prevalence of pemphigus and pemphigoid autoantibodies in the general population

Abstract

BackgroundMucocutaneous blistering is characteristic of autoimmune bullous dermatoses (AIBD). Blisters are caused by autoantibodies directed against structural components of the skin. Hence, detection of specific autoantibodies has become a hallmark for AIBD diagnosis. Studies on prevalence of AIBD autoantibodies in healthy individuals yielded contradictory results.MethodsTo clarify this, samples from 7063 blood donors were tested for presence of anti-BP180-NC16A, anti-BP230 and anti-Dsg1/3 IgG by indirect immunofluorescence (IF) microscopy using a biochip.ResultsCumulative prevalence of these autoantibodies was 0.9 % (CI: 0.7–1.1 %), with anti-BP180-NC16A IgG being most prevalent. Validation of IF findings using ELISA confirmed presence of autoantibodies in 7/15 (anti-Dsg1), 6/7 (anti-Dsg3), 35/37 (anti-BP180-NC16A) and 2/3 (anti-BP230) cases. Moreover, in 16 samples, anti-BP180-NC16A autoantibody concentrations exceeded the cut-off for the diagnosis of bullous pemphigoid. Interestingly, these anti-BP180-NC16A autoantibodies from healthy individuals formed immune complexes with recombinant antigen and dose-dependently activated neutrophils in vitro. However, fine-epitope mapping within NC16A showed a different binding pattern of anti-BP180-NC16A autoantibodies from healthy individuals compared to bullous pemphigoid patients, while IgG subclasses were identical.ConclusionsCollectively, we here report a low prevalence of AIBD autoantibodies in a large cohort of healthy individuals. Furthermore, functional analysis shows differences between autoantibodies from healthy donors and AIBD patients.Electronic supplementary materialThe online version of this article (doi:10.1186/s13023-015-0278-x) contains supplementary material, which is available to authorized users.

BP180 Autoantibodies Target Different Epitopes in Multiple Sclerosis or Alzheimer’s Disease than in Bullous Pemphigoid

“…Despite the fact that MS, of all neurologic diseases, has the strongest association with BP (Lai et al, 2016), the existence of autoantibodies against BP180 in patients with MS has so far been analyzed by only one study, in which none of the MS sera tested (n ¼ 50) showed positivity in the BP180-NC16A ELISA or in the immunoblotting against the full-length BP180 (Recke et al, 2016). We found that about half of MS sera recognize the full-length BP180 in immunoblotting, but the proportion that showed elevated antieBP180-NC16A ELISA values was only slightly higher than in the control group and similar to that reported in general population (Prussmann et al, 2015;Wieland et al, 2010). The obvious explanation for why our results differed from those described is the sensitivity of the immunoblotting assay, because we used recombinant BP180 while the previous study employed a keratinocyte extracellular matrix.…”

The 2016 Bowman Lecture Conjunctival curses: scarring conjunctivitis 30 years on

“…However, the prevalence of these antibodies has varied in relation to the sensitivity and specificity of the assay used and the control group. The lowest prevalence has been less than 1% in a large cohort of blood donors of unknown ages, 54 other publications have given values of 7.5%, 55 13%, 16% and 26% for ELISA and or immunoblots 56 although higher values have been reported. 57 Results for IIF have generally shown o5% positivity 55,56 although this was 19% (n = 32) in another study.…”

Autoimmunity and Cytokine Imbalance in Inherited Epidermolysis Bullosa

“…In our study population, in addition to antibodies against BP180, BP230 and COL7, antibodies against DSG1 and DSG3 were evaluated. These proteins are members of the cadherin cell adhesion molecule superfamily and are major components of the cell–cell junctions that help resist shearing forces and are found in high concentrations in cells subject to mechanical stress [14]. The pathogenic role of these antibodies has previously been shown in experimental animals and in humans.…”