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

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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 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.

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Most cited references 15

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Bullous pemphigoid: etiology, pathogenesis, and inducing factors: facts and controversies.

Ada Lo Schiavo, Eleonora Ruocco, Gabriella Brancaccio … (2013)

The term pemphigoids includes a group of autoimmune bullous diseases characterized by subepidermal blistering. Bullous pemphigoid (BP) is not only the most common disorder within the pemphigoid group, but also represents the most frequent autoimmune blistering disease in general. The onset and course of BP depend on a variable interaction between predisposing and inducing factors. HLA genes are the most significant genetic predisposition factor to autoimmunity mechanisms. Many studies show an association between HLA-DQβ1*0301 and distinct clinical pemphigoid variants. Imbalance between autoreactive T helper (Th) and T regulatory cells, toll-like receptor activation, and Th17/IL-17 pathway are the three possible autoimmunity triggers underlying BP. The pathomechanism of BP hinges on an autoantibody response toward structural components of the hemidesmosome (BP180 and BP230). The binding of autoantibodies leads to complement activation, recruitment of inflammatory cells, and release of proteolytic enzymes. The inflammatory cascade also may be directly triggered by activation of Th17 cells with no intervention of autoantibodies. The intervention of inducing factors in BP can be identified in no more than 15% of patients. Facilitating factors in genetically predisposed individuals are various (drug intake, physical agents, and viral infections). Drugs may act as triggers by either modifying the immune response or altering the antigenic properties of the epidermal basement membrane. Cases of induction of BP by physical agents (eg, radiation therapy, ultraviolet radiation, thermal or electrical burns, surgical procedures, transplants) are rare, but well-documented events. A contributing role in inducing BP has been suggested for infections, in particular human herpes virus (HHV) infections (cytomegalovirus, Epstein-Barr virus, and HHV-6), but also hepatitis B and C viruses, Helicobacter pylori, and Toxoplasma gondii. Unlike pemphigus, no dietary triggers have been suspected of being involved in the induction of BP. In all patients who have a diagnosis of BP, an environmental agent as a potential cause should always be considered, because the prompt discontinuation of it might result in rapid improvement or even cure of the disease.

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Bullous pemphigoid and internal diseases - A case-control study.

Hana Jedličková, Milos Hlubinka, Tomas Pavlik … (2010)

To study associations of bullous pemphigoid (BP) with internal diseases, we conducted a retrospective case control study assessing the frequency of selected diseases - diabetes mellitus, neurological diseases, malignant tumors, benign prostate hyperplasia, hypertension and ischemic heart disease in patients with BP. 89 patients with BP, whose data were retrieved from the register of the Centre of bullous diseases from the period of 1991-2006, were matched with 89 controls of the same age and gender, recruited from patients treated for other skin diseases. The frequency of internal diseases at the time of the onset of BP was evaluated by unconditional logistic regression adjusted for age and gender and maximum likelihood test for contingency tables. Neurological disease was found in 42.7% of the patients and in 19.1% of controls. This difference was statistically significant (p value = 0.001). Moreover, regression analysis has shown that patients with neurological disease in the age group >or= 80 years have significantly higher risk of pemphigoid than patients without neurological disease (odds ratio 10.55; 95% confidence interval 2.68 to 41.49). Most frequent were cerebral stroke in men and dementia in women. For other diseases and other age groups, no statistically significant influence was found.

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Distribution of collagen XVII in the human brain.

Kari Majamaa, Allan Seppänen, Tiina Suuronen … (2007)

We have recently discovered collagen XVII to be present in neurons of the human central nervous system. Collagen XVII has previously been primarily studied in the field of dermatopathology since it is abundantly expressed in the skin, which, like the nervous system, is ectodermal in origin. A link between dermatopathological and neurological entities has been implied due to clinical case studies revealing an association between bullous pemphigoid and age-related neurodegenerative disorders. The objective of this study was to assess the distribution of collagen XVII in the human brain in relation to normal ageing. Post-mortem brain tissue was obtained from 11 neurologically unimpaired subjects who had died from cardiovascular causes at the age of 17 to 78 years. Collagen XVII expression in various neuroanatomical regions, representing essentially the entire human brain, was then assessed using immunohistochemistry. We found collagen XVII to be expressed widely in the brain and to be located primarily in the soma and proximal axons of neurons. In contrast, glial cells did not express collagen XVII. The expression varied strikingly between different neuroanatomical regions, being most notable in motor nuclei and Betz cells followed by pyramidal neurons. There was no correlation between collagen XVII expression and variables such as gender, age at death, post-mortem delay and fixation time whereas a mode of death leading to notable neuronal ischemia depleted the protein expression. Many neurodegenerative disorders display a specific pattern of neuroanatomical involvement, thus the regionally variable expression of collagen XVII offers new prospects for research.

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Author and article information

Journal

Journal ID (nlm-ta): Cent Eur J Immunol

Journal ID (iso-abbrev): Cent Eur J Immunol

Journal ID (publisher-id): CEJI

Title: Central-European Journal of Immunology

Publisher: Polish Society of Experimental and Clinical Immunology

ISSN (Print): 1426-3912

ISSN (Electronic): 1644-4124

Publication date (Electronic): 08 May 2017

Publication date (Print): 2017

Volume: 42

Issue: 1

Pages: 85-90

Affiliations

[1 ]Autoimmune Blistering Dermatoses Section, Department of Dermatology, Poznan University of Medical Sciences, Poznan, Poland

[2 ]Department of Bioinformatics and Computational Biology, Poznan University of Medical Sciences, Poznan, Poland

[3 ]Cutaneous Histopathology and Immunopathology Section, Department of Dermatology, Poznan University of Medical Sciences, Poznan, Poland

Author notes

Correspondence: Justyna Gornowicz-Porowska, Autoimmune Blistering Dermatoses Section, Department of Dermatology, Poznan University of Medical Sciences, Przybyszewskiego 49, 60-355 Poznan, Poland. e-mail: justynagornowicz1@ 123456poczta.onet.pl

Article

Publisher ID: 29850

DOI: 10.5114/ceji.2017.67322

PMC ID: 5470618

SO-VID: 9a84c027-1932-4434-a3fc-31181030611b

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This is an Open Access article distributed under the terms of the Creative Commons Attribution-NonCommercial-ShareAlike 4.0 International (CC BY-NC-SA 4.0) License, allowing third parties to copy and redistribute the material in any medium or format and to remix, transform, and build upon the material, provided the original work is properly cited and states its license.

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

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Most cited references 15

Bullous pemphigoid: etiology, pathogenesis, and inducing factors: facts and controversies.

Bullous pemphigoid and internal diseases - A case-control study.

Distribution of collagen XVII in the human brain.

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