Objectives/hypothesis: Chronic Rhinosinusitis (CRS) is accompanied by evidence of a vigorous adaptive immune response, and emerging studies demonstrate that some nasal polyps manifest a polyclonal autoantibody response. We previously found that antibodies against BP180, a component of the hemidesmosome complex and the dominant epitope in autoimmune bullous pemphigoid, were found at elevated levels in nasal polyp tissue. Given the critical role of hemidesmosomes in maintaining epithelial integrity, we sought to investigate the distribution of BP180 in nasal tissue and evaluate for evidence of systemic autoimmunity against this antigen in CRS.

Study Design: Case-control experimental study.

Methods: The expression and distribution of BP180 in cultured nasal epithelial cells and normal nasal tissue were confirmed using real-time polymerase chain reaction (PCR), Western immunoblotting, immunofluorescence and immunohistochemistry. Sera were collected from three groups: control, CRSsNP, and CRSwNP. A commercially available ELISA was utilized to compare anti-BP180 autoantibody levels in sera.

Results: BP180 is expressed in nasal epithelium, but is not confined to the basement membrane as it is in human skin. In cultured nasal epithelial cells, confocal immunofluorescence showed a punctate distribution of BP180 along the basal surface, consistent with its distribution in epithelial keratinocytes. There are significantly higher levels of circulating nonpathologic anti-BP180 autoantibodies in CRS patients compared with normal controls (P <0.05).

Conclusions: BP180 is more widely expressed in nasal epithelium versus skin, although it appears to play a similar role in the formation of hemidesmosomes along the basement membrane. Further investigations are ongoing to characterize the pathogenicity of the anti-epithelial antibody response in CRS.

Download full-text PDF

Source
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3813294PMC
http://dx.doi.org/10.1002/lary.24016DOI Listing

Publication Analysis

Top Keywords

distribution bp180
12
anti-bp180 autoantibodies
8
chronic rhinosinusitis
8
nasal tissue
8
cultured nasal
8
nasal epithelial
8
epithelial cells
8
nasal
7
bp180
5
role anti-bp180
4

Similar Publications

Clinical characteristics of bullous pemphigoid patients of different ages and the possible mechanism.

J Dermatol

January 2025

Department of Dermatology, State Key Laboratory of Complex Severe and Rare Diseases, Peking Union Medical College Hospital, Chinese Academy of Medical Science and Peking Union Medical College, National Clinical Research Center for Dermatologic and Immunologic Diseases, Beijing, China.

Bullous pemphigoid (BP) is an acquired autoimmune bullous disease that often occurs in elderly patients. Some BP patients with early age of disease onset were observed to have difficulty in receiving applicable disease control. It remains challenging for clinicians to choose the appropriate treatment for these patients.

View Article and Find Full Text PDF

COVID-19 Vaccine: A Potential Risk Factor for Accelerating the Onset of Bullous Pemphigoid.

Vaccines (Basel)

September 2024

Molecular and Cell Biology Laboratory, Istituto Dermopatico dell'Immacolata (IDI)-IRCCS, 00167 Rome, Italy.

Article Synopsis
  • Bullous pemphigoid (BP) is a prevalent autoimmune disease characterized by the presence of autoantibodies against hemidesmosomal components, specifically BP180 and BP230.
  • Despite studies indicating no direct link between COVID-19 vaccines and BP, there have been over 90 reported cases of vaccine-associated BP since the start of mass vaccinations.
  • An investigation involving 64 BP patients revealed a significant proportion developed the condition shortly after vaccination, suggesting that the vaccine might act as an accelerating factor for BP in genetically susceptible individuals.
View Article and Find Full Text PDF

Bullous pemphigoid (BP) is the most common autoimmune bullous disease: it most commonly affects individuals over 70 years old and impacts severely on their quality of life. BP represents a paradigm for an organ-specific autoimmune disease and is characterized by circulating IgG autoantibodies to hemidesmosomal components: BP180 and BP230. While the crucial role of these autoantibodies in triggering BP inflammatory cascade is fully acknowledged, many ancillary etiological mechanisms need to be elucidated yet.

View Article and Find Full Text PDF

Isolation and analysis of the exosomal membrane proteins in bullous pemphigoid.

Expert Rev Clin Immunol

December 2024

Department of Dermatology, State Key Laboratory of Complex Severe and Rare Diseases, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, National Clinical Research Center for Dermatologic and Immunologic Diseases, Beijing, China.

Background: Bullous pemphigoid (BP) is a severe autoimmune sub-epidermal bullous disease. Exosomes are small extracellular vesicles secreted by most cell types. The exosomal membrane proteins are implicated in various biological and pathological pathways.

View Article and Find Full Text PDF

Bullous pemphigoid (BP) is an autoimmune blistering disease that targets the haemidesmosomal proteins, mainly BP180. Extracellular vesicles (EVs) have been demonstrated to carry tissue-specific autoantigens in the setting of autoimmune diseases and transplant organ rejection; this phenomenon was demonstrated to have pathogenic implications in autoimmune diseases and to correlate with transplant rejection severity. The purpose of this study was to identify the presence of BP targeted autoantigens in blister fluid derived EVs.

View Article and Find Full Text PDF

Want AI Summaries of new PubMed Abstracts delivered to your In-box?

Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!