ArticleProceedings of the National Academy of Sciences of the United States of America2009
Pulmonary autoimmunity as a feature of autoimmune polyendocrine syndrome type 1 and identification of KCNRG as a bronchial autoantigen.
Article in Proceedings of the National Academy of Sciences of the United States of America, 2009. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 44 papers.
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Who cites it
44 citing papers in PubMed, 105 citations in OpenAlex.
- Proteome-wide survey of the autoimmune target repertoire in autoimmune polyendocrine syndrome type 1.Scientific reports · 2016Trial
- Natural and disease-specific autoantibodies in chronic obstructive pulmonary disease.Clinical and experimental immunology · 2015Trial
- Aire Mutations and Autoimmune Diseases.Advances in experimental medicine and biology · 2025Review
- Bronchiectasis in a patient with Autoimmune Polyendocrinopathy-Candidiasis-Ectodermal Dystrophy: a case report.BMC pulmonary medicine · 2024Article
- Divergent autoantibody and cytokine levels in COVID-19 sepsis patients influence survival.Journal of medical virology · 2024Article
- Dominant-negative heterozygous mutations in AIRE confer diverse autoimmune phenotypes.iScience · 2023Article
- Retinopathy as an initial sign of hereditary immunological diseases: report of six families and challenges in eye clinic.Frontiers in immunology · 2023Article
- Case report: Virus-induced hemophagocytic lymphohistiocytosis in a patient with APECED.Frontiers in pediatrics · 2023Article
- Autoantibodies are present in the bronchoalveolar lavage but not circulation in patients with fibrotic interstitial lung disease.ERJ open research · 2022Article
- Infections in the monogenic autoimmune syndrome APECED.Current opinion in immunology · 2021Review
- B Cells and Autoantibodies in AIRE Deficiency.Biomedicines · 2021Review
- Fatal autoimmune pneumonitis requiring bilobectomy and omental flap repair in a patient with autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy (APECED).Respiratory medicine case reports · 2021Article
- Autoimmune Polyendocrinopathy-Candidiasis-Ectodermal Dystrophy.Frontiers in pediatrics · 2021Review
- Autoimmune Addison's Disease as Part of the Autoimmune Polyglandular Syndrome Type 1: Historical Overview and Current Evidence.Frontiers in immunology · 2021Review
- Patients With APECED Have Increased Early Mortality Due to Endocrine Causes, Malignancies and infections.The Journal of clinical endocrinology and metabolism · 2020Article
- Article
- An AIREless Breath: Pneumonitis Caused by Impaired Central Immune Tolerance.Frontiers in immunology · 2020Review
- Comment on 'AIRE-deficient patients harbor unique high-affinity disease-ameliorating autoantibodies'.eLife · 2019Article
- Lymphocyte-driven regional immunopathology in pneumonitis caused by impaired central immune tolerance.Science translational medicine · 2019Article
- Petrified pinna and pericarditis in autoimmune polyendocrine syndrome.BMJ case reports · 2019Article
Corrections and comments
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Authors and funding
24 authors at 11 institutions in 7 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Patients with autoimmune polyendocrine syndrome type 1 (APS-1) suffer from multiple organ-specific autoimmunity with autoantibodies against target tissue-specific autoantigens. Endocrine and nonendocrine organs such as skin, hair follicles, and liver are targeted by the immune system. Despite sporadic observations of pulmonary symptoms among APS-1 patients, an autoimmune mechanism for pulmonary involvement has not been elucidated. We report here on a subset of APS-1 patients with respiratory symptoms. Eight patients with pulmonary involvement were identified. Severe airway obstruction was found in 4 patients, leading to death in 2. Immunoscreening of a cDNA library using serum samples from a patient with APS-1 and obstructive respiratory symptoms identified a putative potassium channel regulator (KCNRG) as a pulmonary autoantigen. Reactivity to recombinant KCNRG was assessed in 110 APS-1 patients by using immunoprecipitation. Autoantibodies to KCNRG were present in 7 of the 8 patients with respiratory symptoms, but in only 1 of 102 APS-1 patients without respiratory symptoms. Expression of KCNRG messenger RNA and protein was found to be predominantly restricted to the epithelial cells of terminal bronchioles. Autoantibodies to KCNRG, a protein mainly expressed in bronchial epithelium, are strongly associated with pulmonary involvement in APS-1. These findings may facilitate the recognition, diagnosis, characterization, and understanding of the pulmonary manifestations of APS-1.
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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.