ArticleScience translational medicine2013
BPIFB1 is a lung-specific autoantigen associated with interstitial lung disease.
Article in Science translational medicine, 2013. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 58 papers.
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Who cites it
58 citing papers in PubMed, 96 citations in OpenAlex.
- Ultrarapid Measurement of Diagnostic Antibodies by Magnetic Capture of Immune Complexes.Scientific reports · 2017Trial
- Proteome-wide survey of the autoimmune target repertoire in autoimmune polyendocrine syndrome type 1.Scientific reports · 2016Trial
- Autoantibody-Targeted Treatments for Acute Exacerbations of Idiopathic Pulmonary Fibrosis.PloS one · 2015Trial
- Autoantibodies in chronic non-infectious respiratory diseases: a scoping review.Frontiers in immunology · 2026Article
- Active Antigen-Specific Adaptive Immune Responses Are Shared among Patients with Progressive Fibrotic Interstitial Lung Disease.American journal of respiratory and critical care medicine · 2025Article
- Machine learning-based integration identifies plasma cells-related gene signature ST6GAL1 in idiopathic pulmonary fibrosis.BMC pulmonary medicine · 2025Article
- Distinct mural cells and fibroblasts promote pathogenic plasma cell accumulation in idiopathic pulmonary fibrosis.The European respiratory journal · 2025Article
- Lessons From Prospective Longitudinal Follow-up of a French APECED Cohort.The Journal of clinical endocrinology and metabolism · 2025Observational
- Aire Mutations and Autoimmune Diseases.Advances in experimental medicine and biology · 2025Review
- Multifunctional acyltransferase HBO1: a key regulatory factor for cellular functions.Cellular & molecular biology letters · 2024Review
- Phage Immunoprecipitation-Sequencing Reveals CDHR5 Autoantibodies in Select Patients With Interstitial Lung Disease.ACR open rheumatology · 2024Article
- SFTPB in serum extracellular vesicles as a biomarker of progressive pulmonary fibrosis.JCI insight · 2024Article
- Diabetes mellitus and idiopathic pulmonary fibrosis: a Mendelian randomization study.BMC pulmonary medicine · 2024Article
- Proteomics: Potential techniques for discovering the pathogenesis of connective tissue diseases-interstitial lung disease.Frontiers in immunology · 2023Review
- Pulmonary Fibrosis as a Result of Acute Lung Inflammation: Molecular Mechanisms, Relevant In Vivo Models, Prognostic and Therapeutic Approaches.International journal of molecular sciences · 2022Review
- Plasma cell but not CD20-mediated B-cell depletion protects from bleomycin-induced lung fibrosis.The European respiratory journal · 2022Article
- Article
- Autoantibodies are present in the bronchoalveolar lavage but not circulation in patients with fibrotic interstitial lung disease.ERJ open research · 2022Article
- B Cells and Autoantibodies in AIRE Deficiency.Biomedicines · 2021Review
- Comparative Proteomic Analysis to Investigate the Pathogenesis of Oral Adenoid Cystic Carcinoma.ACS omega · 2021Article
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20 authors at 3 institutions in 3 countries.
Funding
Abstract
Interstitial lung disease (ILD) is a complex and heterogeneous disorder that is often associated with autoimmune syndromes. Despite the connection between ILD and autoimmunity, it remains unclear whether ILD can develop from an autoimmune response that specifically targets the lung parenchyma. We examined a severe form of autoimmune disease, autoimmune polyglandular syndrome type 1 (APS1), and established a strong link between an autoimmune response to the lung-specific protein BPIFB1 (bactericidal/permeability-increasing fold-containing B1) and clinical ILD. Screening of a large cohort of APS1 patients revealed autoantibodies to BPIFB1 in 9.6% of APS1 subjects overall and in 100% of APS1 subjects with ILD. Further investigation of ILD outside the APS1 disorder revealed BPIFB1 autoantibodies present in 14.6% of patients with connective tissue disease-associated ILD and in 12.0% of patients with idiopathic ILD. The animal model for APS1, Aire⁻/⁻ mice, harbors autoantibodies to a similar lung antigen (BPIFB9); these autoantibodies are a marker for ILD. We found that a defect in thymic tolerance was responsible for the production of BPIFB9 autoantibodies and the development of ILD. We also found that immunoreactivity targeting BPIFB1 independent of a defect in Aire also led to ILD, consistent with our discovery of BPIFB1 autoantibodies in non-APS1 patients. Overall, our results demonstrate that autoimmunity targeting the lung-specific antigen BPIFB1 may contribute to the pathogenesis of ILD in patients with APS1 and in subsets of patients with non-APS1 ILD, demonstrating the role of lung-specific autoimmunity in the genesis of ILD.
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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.