Analysis of the autoimmune response against BP180 and BP230 in ethnic Poles with neurodegenerative disorders and bullous pemphigoid

Abstract 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 leve

Reviewing putative industrial triggering in pemphigus: cluster of pemphigus in the area near the wastewater treatment plant

A range of pemphigus is relatively rare potentially fatal group of autoimmune blistering dermatoses. Usually, there is no apparent triggering, while in some predisposed patients there are alleged environmental/industrial inducing factors. In a short time period (4 years), we diagnosed 3 novel cases of pemphigus (1 pemphigus vulgaris, 1 pemphigus foliaceus and 1 shift from pemphigus foliaceus into pemphigus vulgaris) at a clinical and laboratory level (ELISA, immunofluorescence studies). We discuss a possible common inducing mechanism as these patients inhabit one estate of the Poznan suburbia (Kozieglowy, population < 12,000), Greater Poland district, Poland, and review literature data on alleged pemphigus triggers. To the best of our knowledge, this is the first report exploring the putative association between pemphigus diseases and wastewater treatment plant waterborne or volatile by-products in the vicinity of such a facility

Neurodegenerative disorders, bullous pemphigoid and psoriasis: a comparative study in ethnic Poles indicates that Parkinson’s disease is more relevant to bullous pemphigoid

Introduction: Bullous pemphigoid (BP) is an autoimmune blistering dermatosis of the elderly with autoimmunity to hemidesmosomal proteins, BP180 and BP230, which are expressed also in neuronal tissue. Aim : The aim here was to retrospectively compare the prevalence of neurodegenerative disorders (ND), particularly Parkinson’s disease (PD), unspecified conditions manifesting as dementia and stroke, in two groups of ethnic Poles, with BP and with psoriasis (Ps), in order to obtain data whether BP is more prone to coexist with ND than Ps in the elderly. Psoriasis was chosen in this comparative study as it was considered to be a paradigm of cutaneous disease with systemic manifestations. Material and methods : The available medical records of 96 BP patients and 149 Ps patients over 70 years of age were analyzed for the presence of ND. Results : There were no statistically significant differences in prevalence of ND without specifying the type and ND types analyzed between BP and Ps groups, except for a higher prevalence of PD in the BP group. Conclusions :Thus, regarding population aging and increasing incidence and prevalence of BP corresponding with that phenomenon in various ethnicities, it appears justified to expand studies of a possible immunopathogenic relationship, appearing to be PD-related, between BP and ND

Accuracy of molecular diagnostics in pemphigus and bullous pemphigoid: comparison of commercial and modified mosaic indirect immunofluorescence tests as well as enzyme-linked immunosorbent assays

Introduction : Pemphigus and bullous pemphigoid (BP) are identified by autoantibodies (abs) against desmoglein 1, 3 (DSG1/3) and BP180/BP230, respectively. A novel mosaic to indirect immunofluorescence (IIF) using purified BP180 recombinant proteins spotted on slide and transfected cells expressing BP230, DSG1, DSG3 is available. The commercial (IgG detection) and modified (IgG4 detection) mosaic for indirect immunofluorescence (IIFc – IIF commercial, IIFm – IIF modified) and IgG ELISAs were evaluated in pemphigus and bullous pemphigoid (BP) molecular diagnostics. Aim : To compare diagnostic accuracy of commercial (IgG detection) and modified (IgG4 detection) mosaic IIF assay and to examine the diagnostic value of ELISAs in relation to mosaic IIF in routine laboratory diagnostics of pemphigus and BP. Material and methods : Sera from 37 BP and 19 pemphigus patients were studied. Associations between tests were assessed using Fisher’s exact test. Results: There are associations between the positive/negative samples detected by IIFc with desmoglein1 (DSG1)/desmoglein3 (DSG3)/BP230 transfected cells and ELISAs and no association between anti-BP180 IgG detection by IIFc and ELISA. IIFm with DSG1 and DSG3 showed both 100% sensitivity and 100% and 78% specificity, respectively, and 100% and 83% positive predictive value in relation to IIFc. IIFm with BP230 had 87% specificity, 55% sensitivity, whereas IIFm with BP180 had a 100% sensitivity and 13% specificity in relation to IIFc. Conclusions : The IIFc with DSG1/DSG3/BP230 transfected cells, excluding BP180 spots, is an alternative method to ELISA in pemphigus/BP diagnostics. IgG4 antibodies, both pathogenically and diagnostically important, are inconsistently detectable with IIFm

A comparative study of expression of Fc receptors in relation to the autoantibody-mediated immune response and neutrophil elastase expression in autoimmune blistering dermatoses

Here we investigated the cutaneous CD32A and CD89 expression in relation to the neutrophil elastase (NE) expression and serum level of anti-desmoglein 1 and 3 (DSG1/DSG3) IgG in pemphigus, anti-BP180/BP230 IgG in bullous pemphigoid (BP), anti-gliadin nonapeptides (npG), tissue (tTG), and epidermal transglutaminases (eTG) IgA in dermatitis herpetiformis (DH). The examined material consisted of skin/mucosal tissues and sera. In total, 87 patients were studied. Immunohistochemistry on paraffin-embedded sections with quantitative digital morphometry was used to measure the intensity of CD32A/CD89/NE expressions. Levels of anti-DSG1/DSG3 IgG, anti-BP180/BP230 IgG, and anti-npG/tTG/eTG IgA were evaluated with ELISAs. CD32A was abundantly expressed in cutaneous lesions in pemphigus and BP. We found no statistically significant correlation between the CD32A/CD89 and NE expression intensities in pemphigus, BP, and DH. There was a significant correlation between CD89 expression and anti-npG IgA in DH. Our results revealed a lack of correlation between CD32A expressions and anti-DSG1/DSG3 IgG levels in pemphigus, anti-BP180/BP230 IgG in BP as well as CD89 expression and anti-tTG/eTG IgA in DH. CD89 seems to be linked with gluten intolerance in DH rather than with proteolytic destruction of dermal-epidermal junction. CD32A appears to play an important role in mediating skin injury in pemphigus and BP, but probably independently from specific autoantibodies

Conceptual replication study of fifteen JDM effects: Insights from the Polish sample

We conducted pre-registered replications of 15 effects in the field of judgment and decision making (JDM). We aimed to test the generalizability of different classical and modern JDM effects, including, among others: less-is-better, anchoring, and framing to different languages, cultures, or current situations (COVID-19 pandemic). Replicated studies were selected and conducted by undergraduate psychology students enrolled in a decision-making course. Two hundred and two adult volunteers completed an online battery of replicated studies. With a classical significance criterion (p &lt; .05), seven effects were successfully replicated (47%), five partially replicated (33%), and three did not replicate (20%). Even though research materials differed from the originals in several ways, the replication rate in our project is slightly above earlier reported findings in similar replication projects