ArticleJournal of cystic fibrosis : official journal of the European Cystic Fibrosis Society2020
Dissociation of systemic and mucosal autoimmunity in cystic fibrosis.
Article in Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2020. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 15 papers.
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Who cites it
15 citing papers in PubMed, 18 citations in OpenAlex.
- Crosstalk between the microbiome and the mucosal immunoglobulin A system in the lung, in health and disease.Frontiers in cellular and infection microbiology · 2026Review
- Autoantibodies in chronic non-infectious respiratory diseases: a scoping review.Frontiers in immunology · 2026Article
- IgA-producing B cells in lung homeostasis and disease.Frontiers in immunology · 2023Review
- Cystic fibrosis autoantibody signatures associate withFrontiers in immunology · 2023Article
- The S100A7 nuclear interactors in autoimmune diseases: a coevolutionary study in mammals.Immunogenetics · 2022Article
- An Immunoproteomic Survey of the Antibody Landscape: Insights and Opportunities Revealed by Serological Repertoire Profiling.Frontiers in immunology · 2022Review
- Differential Enhancement of Neutrophil Phagocytosis by Anti-Bactericidal/Permeability-Increasing Protein Antibodies.Journal of immunology (Baltimore, Md. : 1950) · 2021Article
- Bactericidal/Permeability-Increasing Protein Preeminently Mediates Clearance ofFrontiers in immunology · 2021Article
- Killing three birds with one BPI: Bactericidal, opsonic, and anti-inflammatory functions.Journal of translational autoimmunity · 2021Review
- IgA autoantibodies directed against self DNA are elevated in cystic fibrosis and associated with more severe lung dysfunction.Autoimmunity · 2020Article
- Low-Avidity Autoantibodies against Bactericidal/Permeability-Increasing Protein Occur in Gram-Negative and Gram-Positive Bacteremia.Infection and immunity · 2020Article
- SARS-CoV-2 (COVID-19) and cystic fibrosis.American journal of physiology. Lung cellular and molecular physiology · 2020Article
- Pre-existing self-reactive IgA antibodies associated with primary graft dysfunction after lung transplantation.Transplant immunology · 2020Article
- Vasculitis in Cystic Fibrosis.Frontiers in pediatrics · 2020Review
- Targeting IgG Autoantibodies for Improved Cytotoxicity of Bactericidal Permeability Increasing Protein in Cystic Fibrosis.Frontiers in pharmacology · 2020Article
Corrections and comments
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Authors and funding
7 authors at 3 institutions in 1 country.
Funding
Abstract
backgroundPseudomonas aeruginosa accounts for ~80% of cystic fibrosis (CF) airway infection. It shows a remarkable correlation with presence of autoantibody to bactericidal/permeability-increasing protein (BPI), which is not understood. In this study, we sought to better understand the characteristics of systemic and mucosal autoimmunity and their relation to humoral immunity to P. aeruginosa.
methodsAntibody titers and isotypes to BPI and P. aeruginosa were characterized in sera and bronchoalveolar lavage (BAL) of adult and pediatric CF patients (n = 131), by ELISA and/or immunoblot.
resultsSerum BPI autoantibodies were common (~43%) in adult while rare (≪5%) in pediatric (≤18 yrs) CF patients. Serum BPI IgG autoantibodies were of high avidity and strongly correlated with anti-P. aeruginosa IgG responses. A parallel relationship was observed with IgA, but not IgG, responses in adult and pediatric CF patient in the BAL. Thus, BAL IgA anti-BPI antibodies were independent of age and correlated with the presence of BPI cleavage in BAL.
conclusionsIgG and IgA autoreactivity to BPI in CF patients was demonstrated in serum and BAL, respectively, and correlated with the isotype of the antibody response to P. aeruginosa. The co-occurrence of anti-BPI and anti-P. aeruginosa IgA in the BAL, but not serum, of pediatric CF patients suggests that BPI tolerance is broken in the P. aeruginosa-infected airway and that serologic IgG autoantibodies are later induced, potentially through a separate pathway. The relationship between P. aeruginosa, BPI cleavage, and IgA autoantibodies in the BAL suggests a role for cryptic epitope generation in the breaking of tolerance.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.