ReviewFrontiers in immunology2021
Pathogenicity of Proteinase 3-Anti-Neutrophil Cytoplasmic Antibody in Granulomatosis With Polyangiitis: Implications as Biomarker and Future Therapies.
Review in Frontiers in immunology, 2021. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 20 papers, 1 of them a synthesis that pooled it.
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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
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Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
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Who cites it
20 citing papers in PubMed, 1 synthesis or guideline pooled it, 28 citations in OpenAlex.
- Alpha-1 antitrypsin deficiency and granulomatosis with polyangiitis: a systematic review and meta-analysis.European respiratory review : an official journal of the European Respiratory Society · 2025Pooled it
- [From sudden hearing loss to an inflammatory systemic disease].Innere Medizin (Heidelberg, Germany) · 2026Article
- A Case Report of Granulomatosis with Polyangiitis Presenting with Chronic Conjunctivitis.Journal of education & teaching in emergency medicine · 2026Article
- Targeting FcRn for immunomodulation: a promising therapy in autoimmune inflammatory rheumatic diseases.Inflammation research : official journal of the European Histamine Research Society ... [et al.] · 2026Review
- Potential impact of two functional variants in theCaspian journal of internal medicine · 2026Article
- A Red Eye in an Elderly Patient: A Case Report Highlighting the Importance of Urgent Diagnosis.Cureus · 2025Article
- Antineutrophil cytoplasmic antibody related vasculitis with a unique imaging presentation of organizing pneumonia. The key role of lung ultrasound.Multidisciplinary respiratory medicine · 2025Article
- Pulmonary and Renal Predictors of Mortality in ANCA-Associated Vasculitis: A Regional Experience from Türkiye.Biomedicines · 2025Article
- Anti-myeloperoxidase IgM B cells in anti-neutrophil cytoplasmic antibody-associated vasculitis.Nature communications · 2025Article
- A1AT dysregulation of metabolically stressed hepatocytes by Kupffer cells drives MASH and fibrosis.Experimental & molecular medicine · 2025Article
- Minimally Invasive Surgical Approach in Granulomatosis with Polyangiitis Complicated with Intramural Descending Aorta Hematoma Followed by Aortic Wall Rupture.Diagnostics (Basel, Switzerland) · 2025Article
- Unexpected Airway Obstruction in a Patient With Undiagnosed Granulomatosis With Polyangiitis.Cureus · 2024Article
- An Autopsy Case of Renal-Limited Granulomatosis With Polyangiitis Presenting With Acute Renal Failure and Initial Delirium.Journal of clinical medicine research · 2024Article
- Clinical Validity of Anti-Proteinase 3 Antibodies in Patients with Inflammatory Bowel Disease: A Short Meta-Analysis.Diagnostics (Basel, Switzerland) · 2023Review
- Defensin Interactions in Relation to Monoclonal and Disease-Related Proteinase 3 Antibodies Binding at the Catalytic Site.Antibodies (Basel, Switzerland) · 2023Article
- Proteomic changes to immune and inflammatory processes underlie lung preservation usingFrontiers in cardiovascular medicine · 2023Article
- GPR97 triggers inflammatory processes in human neutrophils via a macromolecular complex upstream of PAR2 activation.Nature communications · 2022Article
- Article
- Increased frequency of IgD-CD27Arthritis research & therapy · 2022Article
- Granulomatous Inflammation in ANCA-Associated Vasculitis.International journal of molecular sciences · 2021Review
Corrections and comments
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Authors and funding
7 authors at 5 institutions in 2 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Granulomatosis with polyangiitis (GPA) is a rare but serious necrotizing auto-immune vasculitis. GPA is mostly associated with the presence of Anti-Neutrophil Cytoplasmic Antibody (ANCA) targeting proteinase 3 (PR3-ANCA), a serine protease contained in neutrophil granules but also exposed at the membrane. PR3-ANCAs have a proven fundamental role in GPA: they bind neutrophils allowing their auto-immune activation responsible for vasculitis lesions. PR3-ANCAs bind neutrophil surface on the one hand by their Fab binding PR3 and on the other by their Fc binding Fc gamma receptors. Despite current therapies, GPA is still a serious disease with an important mortality and a high risk of relapse. Furthermore, although PR3-ANCAs are a consistent biomarker for GPA diagnosis, relapse management currently based on their level is inconsistent. Indeed, PR3-ANCA level is not correlated with disease activity in 25% of patients suggesting that not all PR3-ANCAs are pathogenic. Therefore, the development of new biomarkers to evaluate disease activity and predict relapse and new therapies is necessary. Understanding factors influencing PR3-ANCA pathogenicity,
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