Disease Score gda Association Type Type Original DB Sentence supporting the association PMID PMID Year
Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis
0.100 Biomarker disease BEFREE All vasculitis relapses in the belimumab group (n = 6) occurred in patients who had PR3-ANCA-associated vasculitis with cyclophosphamide-induced disease remission. 30666823 2019
Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis
0.100 Biomarker disease BEFREE Anti-neutrophil cytoplasmic autoantibodies (ANCA) directed against myeloperoxidase (MPO) and proteinase 3 (PR3) are pathogenic in ANCA-associated vasculitis (AAV). 30818380 2019
Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis
0.100 Biomarker disease BEFREE Exploring Frequencies of Circulating Specific Th17 Cells against Myeloperoxidase and Proteinase 3 in ANCA Associated Vasculitis. 31756913 2019
Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis
0.100 Biomarker disease BEFREE Furthermore, the pathogenesis of ANCA-associated vasculitis has been reviewed with particular emphasis on the role of proteinase 3 (PR3) in fuelling granulomatosis with polyangiitis (GPA) inflammation. 31198793 2019
Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis
0.100 Biomarker disease BEFREE Myeloperoxidase ANCA is more common than proteinase-3 ANCA, and glomerulonephritis is the most common clinical presentation of ANCA-associated vasculitis in SSc. 31776158 2019
Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis
0.100 Biomarker disease BEFREE Proteomic analysis of neutrophils in ANCA-associated vasculitis reveals a dysregulation in proteinase 3-associated proteins such as annexin-A1 involved in apoptotic cell clearance. 31142442 2019
Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis
0.100 AlteredExpression disease BEFREE Moreover, PR3 is a serine protease whose membrane expression can potentiate inflammatory diseases such as ANCA-associated vasculitis and rheumatoid arthritis. 29755460 2018
Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis
0.100 Biomarker disease BEFREE Moreover, patients with proteinase 3 (PR3)-AAV may respond better to rituximab than cyclophosphamide. 28785984 2018
Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis
0.100 Biomarker disease BEFREE This review gives a brief overview on current knowledge about genetic and epigenetic factors, barrier dysfunction and chronic non-resolving inflammation, necro-inflammatory auto-amplification of cellular death and inflammation, altered autoantigen presentation, alternative complement pathway activation, alterations within peripheral and inflamed tissue-residing T- and B-cell populations, ectopic lymphoid tissue neoformation, the characterization of PR3-specific T-cells, properties of ANCA, links between autoimmune disease and infection-triggered pathology, and animal models in AAV. 29686675 2018
Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis
0.100 Biomarker disease BEFREE As dependable immunoassays for PR3-ANCAs and MPO-ANCAs have become broadly available, there is increasing international agreement that high-quality immunoassays are the preferred screening method for the diagnosis of ANCA-associated vasculitis. 28905856 2017
Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis
0.100 Biomarker disease BEFREE To establish a link between DNA methylation, a model epigenetic gene silencing modification, and autoantigen gene expression and disease status in ANCA-associated vasculitis, we measured gene-specific DNA methylation of the autoantigen genes myeloperoxidase (<i>MPO</i>) and proteinase 3 (<i>PRTN3</i>) in leukocytes of patients with ANCA-associated vasculitis observed longitudinally (<i>n</i>=82) and of healthy controls (<i>n</i>=32). 27821628 2017
Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis
0.100 Biomarker disease BEFREE Our comparison of various ANCA detection methods showed (i) large variability between the two IIF methods tested and (ii) a high diagnostic performance of PR3-ANCA and MPO-ANCA by immunoassay to discriminate AAV from disease controls. 27481830 2017
Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis
0.100 Biomarker disease BEFREE Antibodies to myeloperoxidase (MPO) and proteinase 3 (PR3) are vital in the diagnosis and management of ANCA-associated vasculitis. 28549721 2017
Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis
0.100 Biomarker disease BEFREE Identification and phenotyping of circulating autoreactive proteinase 3-specific B cells in patients with PR3-ANCA associated vasculitis and healthy controls. 28870527 2017
Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis
0.100 Biomarker disease BEFREE The association of transient IgM PR3-ANCA with an acute respiratory manifestation of AAV suggests a possible link between an infectious trigger and AAV disease activity. 28076879 2017
Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis
0.100 Biomarker disease BEFREE C3c was found in 78 of 187 renal biopsies (41.7%) divided over 32.3% of proteinase-3 (PR3)-AAV patients and 52.3% of myeloperoxidase (MPO)-AAV patients (P = 0.006), whereas C3d was found positive in 51.1% of PR3-AAV patients and 70.4% of MPO-AAV patients (P = 0.105). 26275893 2017
Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis
0.100 Biomarker disease BEFREE ANCA-associated vasculitis(AAV) covers a group of systemic necrotising vasculitides characterised by inflammation of small vessels, sometimes with granuloma, and associated with autoantibodies to neutrophil cytoplasmic proteases (proteinase-3 or myeloperoxidase). 28406765 2017
Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis
0.100 AlteredExpression disease BEFREE Expression of KDM6B mRNA is elevated in leukocytes from patients with ANCA-associated vasculitis (AAV) and has been suggested to be the reason for higher proteinase 3 (PR3) mRNA expression in these cells due to derepression of PRTN3 gene transcription. 27755585 2016
Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis
0.100 Biomarker disease BEFREE A recent genome-wide association study of patients with ANCA-associated vasculitides confirmed the genetic contribution to the pathogenesis of these conditions, with significant association of PR3-ANCA and human leukocyte antigen-DP and the genes encoding α1-antitrypsin and PR3. 24737903 2014
Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis
0.100 Biomarker disease BEFREE In addition, these studies have also shown that different AAV subtypes such as granulomatosis with polyangiitis (Wegener's, GPA) and microscopic polyangiitis (MPA) are underpinned by distinct genetic risk factors, with GPA being associated with HLA-DP, SERPINA1 (encoding α1-antitrypsin), PRTN3 (encoding proteinase-3, PR3, the main GPA-related autoantigen) and SEMA6A (semaphorin 6A), whereas MPA has been mainly associated with HLA-DQ. 24854378 2014
Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis
0.100 Biomarker disease BEFREE A recent genome-wide association study of patients with ANCA-associated vasculitides confirmed the genetic contribution to the pathogenesis of these conditions, with significant association of PR3-ANCA and human leukocyte antigen-DP and the genes encoding α1-antitrypsin and PR3. 23606701 2013
Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis
0.100 Biomarker disease BEFREE This study confirms that the pathogenesis of ANCA-associated vasculitis has a genetic component, shows genetic distinctions between granulomatosis with polyangiitis and microscopic polyangiitis that are associated with ANCA specificity, and suggests that the response against the autoantigen proteinase 3 is a central pathogenic feature of proteinase 3 ANCA-associated vasculitis. 22808956 2012
Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis
0.100 Biomarker disease BEFREE The neutrophil serine protease proteinase 3 (PR3) is a main autoantigen in anti-neutrophil cytoplasmic antibody-associated vasculitis. 18462208 2008
Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis
0.100 GeneticVariation disease BEFREE The data suggest that G-463A polymorphism does not seem to contribute to either MPO-ANCA- or PR3-ANCA-associated vasculitis formation. 15031357 2004
Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis
0.100 Biomarker disease BEFREE Alpha-1 anti-trypsin is the physiological inhibitor of PR3 and carriage of the defective allele PI*Z was observed as the first genetic risk factor for the development of PR3-ANCA-associated vasculitis. 15675141 2004