{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,22]],"date-time":"2026-10-22T03:45:48Z","timestamp":1792640748043,"version":"4.5.0"},"reference-count":152,"publisher":"MDPI AG","issue":"13","license":[{"start":{"date-parts":[[2025,6,24]],"date-time":"2025-06-24T00:00:00Z","timestamp":1750723200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["IJMS"],"abstract":"<jats:p>Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) constitutes a group of rare diseases characterized by autoimmune-associated inflammation and vessel damage. Based on the clinical manifestations and involvement of immune components, three disease syndromes are distinguished: granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA). In this review, we present the current data on the epidemiology, the clinical manifestations of each syndrome, and the most up-to-date classification criteria. The role of the underlying genetic and epigenetic abnormalities, as well as their interplay, is described. The immunological diversification of AAV is also described, with a focus on the immune cell dysfunctions detected in patients. In conclusion, we emphasize the urgent need to unravel the sophisticated mechanisms of this disease, which would enable the development of new, effective therapeutic strategies.<\/jats:p>","DOI":"10.3390\/ijms26136065","type":"journal-article","created":{"date-parts":[[2025,6,24]],"date-time":"2025-06-24T09:24:18Z","timestamp":1750757058000},"page":"6065","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":12,"title":["Immunopathology of Antineutrophil Cytoplasmic Antibody (ANCA)-Associated Vasculitis"],"prefix":"10.3390","volume":"26","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-6086-0024","authenticated-orcid":false,"given":"Katarzyna Aleksandra","family":"Lisowska","sequence":"first","affiliation":[{"name":"Department of Pathophysiology, Medical University of Gda\u0144sk, 80-210 Gda\u0144sk, Poland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4392-5594","authenticated-orcid":false,"given":"Anna","family":"Wardowska","sequence":"additional","affiliation":[{"name":"Department of Pathophysiology, Medical University of Gda\u0144sk, 80-210 Gda\u0144sk, Poland"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2025,6,24]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"226","DOI":"10.1186\/s13075-018-1708-7","article-title":"The Frequency of ANCA-Associated Vasculitis in a National Database of Hospitalized Patients in China","volume":"20","author":"Li","year":"2018","journal-title":"Arthritis Res. Ther."},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"2338","DOI":"10.1002\/art.40313","article-title":"The Epidemiology of Antineutrophil Cytoplasmic Autoantibody\u2013Associated Vasculitis in Olmsted County, Minnesota: A Twenty-Year US Population\u2013Based Study","volume":"69","author":"Berti","year":"2017","journal-title":"Arthritis Rheumatol."},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"2147","DOI":"10.1007\/s00296-021-04993-2","article-title":"Prevalence of large vessel vasculitis in ANCA-associated vasculitis: A retrospective cohort study","volume":"41","author":"Coattrenec","year":"2021","journal-title":"Rheumatol. Int."},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"1682","DOI":"10.1093\/rheumatology\/kead467","article-title":"Large vessel involvement in antineutrophil cytoplasmic antibody-associated vasculitis","volume":"63","author":"Kaymakci","year":"2024","journal-title":"Rheumatology"},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"481","DOI":"10.1007\/s11926-012-0286-y","article-title":"Pathogenesis of ANCA-Associated Vasculitis","volume":"14","author":"Peikert","year":"2012","journal-title":"Curr. Rheumatol. Rep."},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"577","DOI":"10.3389\/fimmu.2014.00577","article-title":"Genetic Susceptibility to ANCA-Associated Vasculitis: State of the Art","volume":"5","author":"Bonatti","year":"2014","journal-title":"Front. Immunol."},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"1417","DOI":"10.1002\/art.24790","article-title":"Antineutrophil Cytoplasmic Antibody\u2013Associated Vasculitides: Could Geographic Patterns Be Explained by Ambient Ultraviolet Radiation?","volume":"61","author":"Gatenby","year":"2009","journal-title":"Arthritis Rheum."},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"870","DOI":"10.1001\/jamainternmed.2020.0675","article-title":"Association of Cigarette Smoking With Antineutrophil Cytoplasmic Antibody\u2013Associated Vasculitis","volume":"180","author":"McDermott","year":"2020","journal-title":"JAMA Intern. Med."},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"814","DOI":"10.1002\/art.10830","article-title":"Are Environmental Factors Important in Primary Systemic Vasculitis?: A Case\u2013Control Study","volume":"48","author":"Lane","year":"2003","journal-title":"Arthritis Rheum."},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"34","DOI":"10.1186\/s13148-019-0632-2","article-title":"The Emerging Role of Epigenetics in Human Autoimmune Disorders","volume":"11","author":"Mazzone","year":"2019","journal-title":"Clin. Epigenetics"},{"key":"ref_11","unstructured":"(2025, June 11). Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis. Available online: https:\/\/www.orpha.net\/en\/disease\/detail\/156152."},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"e002949","DOI":"10.1136\/rmdopen-2022-002949","article-title":"Stable Incidence but Increase in Prevalence of ANCA-Associated Vasculitis in Southern Sweden: A 23-Year Study","volume":"9","author":"Rathmann","year":"2023","journal-title":"RMD Open"},{"key":"ref_13","doi-asserted-by":"crossref","first-page":"1656","DOI":"10.1093\/rheumatology\/kew232","article-title":"Incidence of ANCA-Associated Vasculitis in a UK Mixed Ethnicity Population","volume":"55","author":"Pearce","year":"2016","journal-title":"Rheumatology"},{"key":"ref_14","doi-asserted-by":"crossref","unstructured":"Redondo-Rodriguez, R., Mena-V\u00e1zquez, N., Cabezas-Lucena, A.M., Manrique-Arija, S., Mucientes, A., and Fern\u00e1ndez-Nebro, A. (2022). Systematic Review and Metaanalysis of Worldwide Incidence and Prevalence of Antineutrophil Cytoplasmic Antibody (ANCA) Associated Vasculitis. J. Clin. Med., 11.","DOI":"10.3390\/jcm11092573"},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"i14","DOI":"10.1093\/ndt\/gfv022","article-title":"Classification, Epidemiology and Clinical Subgrouping of Antineutrophil Cytoplasmic Antibody (ANCA)-Associated Vasculitis","volume":"30","author":"Watts","year":"2015","journal-title":"Nephrol. Dial. Transplant."},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"351","DOI":"10.1111\/j.1445-5994.2011.02705.x","article-title":"Anti-neutrophil Cytoplasmic Antibody-associated Systemic Vasculitis: Nature or Nurture?","volume":"42","author":"Gatenby","year":"2012","journal-title":"Intern. Med. J."},{"key":"ref_17","doi-asserted-by":"crossref","first-page":"2553","DOI":"10.1007\/s10067-019-04538-w","article-title":"Clinical Characteristics of Polish Patients with ANCA-Associated Vasculitides\u2014Retrospective Analysis of POLVAS Registry","volume":"38","author":"Sznajd","year":"2019","journal-title":"Clin. Rheumatol."},{"key":"ref_18","doi-asserted-by":"crossref","unstructured":"Almaani, S., Fussner, L.A., Brodsky, S., Meara, A.S., and Jayne, D. (2021). ANCA-Associated Vasculitis: An Update. J. Clin. Med., 10.","DOI":"10.3390\/jcm10071446"},{"key":"ref_19","doi-asserted-by":"crossref","first-page":"445","DOI":"10.1016\/j.berh.2009.03.001","article-title":"The diagnosis of vasculitis","volume":"23","author":"Jayne","year":"2009","journal-title":"Best Pract. Res. Clin. Rheumatol."},{"key":"ref_20","doi-asserted-by":"crossref","first-page":"e6083","DOI":"10.1097\/MD.0000000000006083","article-title":"Clinical characteristics and outcome of Spanish patients with ANCA-associated vasculitides: Impact of the vasculitis type, ANCA specificity, and treatment on mortality and morbidity","volume":"96","author":"Fraile","year":"2017","journal-title":"Medicine"},{"key":"ref_21","doi-asserted-by":"crossref","first-page":"361","DOI":"10.1016\/j.jaip.2024.10.013","article-title":"Characteristics of Severe Asthma Clinic Patients with Eosinophilic Granulomatosis with Polyangiitis","volume":"13","author":"Puan","year":"2025","journal-title":"J. Allergy Clin. Immunol. Pract."},{"key":"ref_22","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1002\/art.37715","article-title":"2012 Revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides","volume":"65","author":"Jennette","year":"2013","journal-title":"Arthritis Rheum."},{"key":"ref_23","doi-asserted-by":"crossref","first-page":"357","DOI":"10.1007\/s12016-015-8495-6","article-title":"Epigenetics and Vasculitis: A Comprehensive Review","volume":"50","author":"Renauer","year":"2016","journal-title":"Clin. Rev. Allergy Immunol."},{"key":"ref_24","doi-asserted-by":"crossref","first-page":"1046","DOI":"10.1016\/j.autrev.2016.07.031","article-title":"The Role of Monocytes in ANCA-Associated Vasculitides","volume":"15","author":"Brunini","year":"2016","journal-title":"Autoimmun. Rev."},{"key":"ref_25","doi-asserted-by":"crossref","first-page":"315","DOI":"10.1136\/annrheumdis-2021-221795","article-title":"2022 American College of Rheumatology\/European Alliance of Associations for Rheumatology Classification Criteria for Granulomatosis with Polyangiitis","volume":"81","author":"Robson","year":"2022","journal-title":"Ann. Rheum. Dis."},{"key":"ref_26","doi-asserted-by":"crossref","first-page":"321","DOI":"10.1136\/annrheumdis-2021-221796","article-title":"2022 American College of Rheumatology\/European Alliance of Associations for Rheumatology Classification Criteria for Microscopic Polyangiitis","volume":"81","author":"Suppiah","year":"2022","journal-title":"Ann. Rheum. Dis."},{"key":"ref_27","doi-asserted-by":"crossref","first-page":"309","DOI":"10.1136\/annrheumdis-2021-221794","article-title":"2022 American College of Rheumatology\/European Alliance of Associations for Rheumatology Classification Criteria for Eosinophilic Granulomatosis with Polyangiitis","volume":"81","author":"Grayson","year":"2022","journal-title":"Ann. Rheum. Dis."},{"key":"ref_28","doi-asserted-by":"crossref","first-page":"124","DOI":"10.1053\/j.ajkd.2019.04.031","article-title":"ANCA-Associated Vasculitis: Core Curriculum 2020","volume":"75","author":"Geetha","year":"2020","journal-title":"Am. J. Kidney Dis."},{"key":"ref_29","doi-asserted-by":"crossref","first-page":"211","DOI":"10.1007\/s12016-012-8320-4","article-title":"Antineutrophil Cytoplasmic Autoantibodies: How Are They Detected and What Is Their Use for Diagnosis, Classification and Follow-Up?","volume":"43","author":"Damoiseaux","year":"2012","journal-title":"Clin. Rev. Allerg. Immunol."},{"key":"ref_30","doi-asserted-by":"crossref","first-page":"472","DOI":"10.5858\/2000-124-472a-AAOAP","article-title":"Advanced Atlas of Autoantibody Patterns","volume":"124","author":"Chejfec","year":"2000","journal-title":"Arch. Pathol. Lab. Med."},{"key":"ref_31","doi-asserted-by":"crossref","first-page":"93","DOI":"10.1080\/08916930400022673","article-title":"Antineutrophil Cytoplasmic Antibodies (ANCA)","volume":"38","author":"Radice","year":"2005","journal-title":"Autoimmunity"},{"key":"ref_32","first-page":"8209737","article-title":"Increased Frequencies of Switched Memory B Cells and Plasmablasts in Peripheral Blood from Patients with ANCA-Associated Vasculitis","volume":"2020","author":"Smargianaki","year":"2020","journal-title":"J. Immunol. Res."},{"key":"ref_33","doi-asserted-by":"crossref","first-page":"66","DOI":"10.1007\/s10238-024-01318-y","article-title":"Clinical Features and Prognosis of ANCA-Associated Vasculitis Patients Who Were Double-Seropositive for Myeloperoxidase-ANCA and Proteinase 3-ANCA","volume":"24","author":"Gong","year":"2024","journal-title":"Clin. Exp. Med."},{"key":"ref_34","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1097\/BOR.0b013e328011cb80","article-title":"Controversies in Small Vessel Vasculitis\u2014Comparing the Rheumatology and Nephrology Views","volume":"19","author":"Falk","year":"2007","journal-title":"Curr. Opin. Rheumatol."},{"key":"ref_35","first-page":"101600","article-title":"Antineutrophil Cytoplasmic Antibody-Negative Granulomatosis with Polyangiitis Localized to the Lungs","volume":"36","author":"Ohteru","year":"2022","journal-title":"Respir. Med. Case Rep."},{"key":"ref_36","doi-asserted-by":"crossref","first-page":"1392","DOI":"10.1093\/ndt\/gfh830","article-title":"ANCA-Negative Pauci-Immune Renal Vasculitis: Histology and Outcome","volume":"20","author":"Eisenberger","year":"2005","journal-title":"Nephrol. Dial. Transplant."},{"key":"ref_37","doi-asserted-by":"crossref","first-page":"e314","DOI":"10.1016\/S2665-9913(24)00035-3","article-title":"Proteinase 3-Specific Antineutrophil Cytoplasmic Antibody-Associated Vasculitis","volume":"6","author":"Falde","year":"2024","journal-title":"Lancet Rheumatol."},{"key":"ref_38","doi-asserted-by":"crossref","first-page":"e34450","DOI":"10.1097\/MD.0000000000034450","article-title":"ANCA-Negative Eosinophilic Granulomatosis with Polyangiitis Complicated by Peripheral Nerve Damage: A Case Report","volume":"102","author":"Chen","year":"2023","journal-title":"Medicine"},{"key":"ref_39","doi-asserted-by":"crossref","first-page":"bcr0120125502","DOI":"10.1136\/bcr.01.2012.5502","article-title":"Familial Granulomatosis with Polyangiitis: Three Cases of This Rare Disorder in One Indoasian Family Carrying an Identical HLA DPB1 Allele","volume":"2012","author":"Tanna","year":"2012","journal-title":"BMJ Case Rep."},{"key":"ref_40","doi-asserted-by":"crossref","first-page":"991","DOI":"10.2169\/internalmedicine.3744-19","article-title":"Familial Eosinophilic Granulomatosis with Polyangiitis in a Sister and Brother","volume":"59","author":"Ueki","year":"2020","journal-title":"Intern. Med."},{"key":"ref_41","doi-asserted-by":"crossref","first-page":"181","DOI":"10.1186\/s12890-018-0733-2","article-title":"A Rare Case Report of Polyangiitis Overlap Syndrome: Granulomatosis with Polyangiitis and Eosinophilic Granulomatosis with Polyangiitis","volume":"18","author":"Quan","year":"2018","journal-title":"BMC Pulm. Med."},{"key":"ref_42","doi-asserted-by":"crossref","first-page":"71","DOI":"10.1038\/s41572-020-0204-y","article-title":"ANCA-Associated Vasculitis","volume":"6","author":"Kitching","year":"2020","journal-title":"Nat. Rev. Dis. Primers"},{"key":"ref_43","doi-asserted-by":"crossref","first-page":"302","DOI":"10.1002\/art.23157","article-title":"Risks and Relative Risks of Wegener\u2019s Granulomatosis among Close Relatives of Patients with the Disease","volume":"58","author":"Knight","year":"2008","journal-title":"Arthritis Rheum."},{"key":"ref_44","doi-asserted-by":"crossref","first-page":"661","DOI":"10.1002\/art.24328","article-title":"Familial Associations of Rheumatoid Arthritis with Autoimmune Diseases and Related Conditions","volume":"60","author":"Hemminki","year":"2009","journal-title":"Arthritis Rheum."},{"key":"ref_45","doi-asserted-by":"crossref","first-page":"624848","DOI":"10.3389\/fimmu.2021.624848","article-title":"Antineutrophil Cytoplasmic Antibody-Associated Vasculitis Update: Genetic Pathogenesis","volume":"12","author":"Li","year":"2021","journal-title":"Front. Immunol."},{"key":"ref_46","doi-asserted-by":"crossref","first-page":"468","DOI":"10.1007\/s00439-004-1092-z","article-title":"New Genomic Region for Wegener?S Granulomatosis as Revealed by an Extended Association Screen with 202 Apoptosis-Related Genes","volume":"114","author":"Jagiello","year":"2004","journal-title":"Hum. Genet."},{"key":"ref_47","doi-asserted-by":"crossref","first-page":"972","DOI":"10.1136\/ard.2007.077693","article-title":"The Wegener\u2019s Granulomatosis Quantitative Trait Locus on Chromosome 6p21.3 as Characterised by tagSNP Genotyping","volume":"67","author":"Heckmann","year":"2008","journal-title":"Ann. Rheum. Dis."},{"key":"ref_48","doi-asserted-by":"crossref","first-page":"422","DOI":"10.1016\/j.humimm.2011.02.017","article-title":"The Association of HLA-DRB1 Alleles with Antineutrophil Cytoplasmic Antibody-Associated Systemic Vasculitis in Chinese Patients","volume":"72","author":"Luo","year":"2011","journal-title":"Hum. Immunol."},{"key":"ref_49","doi-asserted-by":"crossref","first-page":"81","DOI":"10.1038\/sj.gene.6364262","article-title":"Association of HLA-DRB1*0901-DQB1*0303 Haplotype with Microscopic Polyangiitis in Japanese","volume":"7","author":"Tsuchiya","year":"2006","journal-title":"Genes Immun."},{"key":"ref_50","doi-asserted-by":"crossref","first-page":"3159","DOI":"10.1002\/art.22834","article-title":"HLA\u2013DRB4 as a Genetic Risk Factor for Churg-Strauss Syndrome","volume":"56","author":"Vaglio","year":"2007","journal-title":"Arthritis Rheum."},{"key":"ref_51","doi-asserted-by":"crossref","first-page":"329","DOI":"10.1002\/art.23209","article-title":"Associations of Churg-Strauss Syndrome with the HLA\u2013DRB1 Locus, and Relationship to the Genetics of Antineutrophil Cytoplasmic Antibody\u2013Associated Vasculitides: Comment on the Article by Vaglio et Al","volume":"58","author":"Wieczorek","year":"2008","journal-title":"Arthritis Rheum."},{"key":"ref_52","doi-asserted-by":"crossref","first-page":"9543","DOI":"10.1128\/MCB.25.21.9543-9553.2005","article-title":"CTLA-4 and PD-1 Receptors Inhibit T-Cell Activation by Distinct Mechanisms","volume":"25","author":"Parry","year":"2005","journal-title":"Mol. Cell. Biol."},{"key":"ref_53","doi-asserted-by":"crossref","first-page":"201","DOI":"10.1016\/S1074-7613(02)00362-X","article-title":"The Interaction Properties of Costimulatory Molecules Revisited","volume":"17","author":"Collins","year":"2002","journal-title":"Immunity"},{"key":"ref_54","doi-asserted-by":"crossref","first-page":"121","DOI":"10.1186\/1471-2350-10-121","article-title":"Confirmation of the Genetic Association of CTLA4 and PTPN22 with ANCA-Associated Vasculitis","volume":"10","author":"Carr","year":"2009","journal-title":"BMC Med. Genet."},{"key":"ref_55","doi-asserted-by":"crossref","first-page":"1502","DOI":"10.1093\/rheumatology\/kep280","article-title":"CT60 and +49 Polymorphisms of CTLA 4 Are Associated with ANCA-Positive Small Vessel Vasculitis","volume":"48","author":"Kamesh","year":"2009","journal-title":"Rheumatology"},{"key":"ref_56","doi-asserted-by":"crossref","first-page":"890","DOI":"10.1136\/annrheumdis-2012-202803","article-title":"Genetics of Toll like Receptor 9 in ANCA Associated Vasculitides","volume":"73","author":"Husmann","year":"2014","journal-title":"Ann. Rheum. Dis."},{"key":"ref_57","doi-asserted-by":"crossref","first-page":"4039","DOI":"10.1002\/art.21487","article-title":"The PTPN22 620W Allele Is a Risk Factor for Wegener\u2019s Granulomatosis","volume":"52","author":"Jagiello","year":"2005","journal-title":"Arthritis Rheum."},{"key":"ref_58","doi-asserted-by":"crossref","first-page":"805","DOI":"10.1093\/rheumatology\/ker446","article-title":"PTPN22 R620W Polymorphism in the ANCA-Associated Vasculitides","volume":"51","author":"Martorana","year":"2012","journal-title":"Rheumatology"},{"key":"ref_59","doi-asserted-by":"crossref","first-page":"2473","DOI":"10.1046\/j.1523-1755.2000.00430.x","article-title":"Proteinase 3 Gene Polymorphisms and Wegener\u2019s Granulomatosis","volume":"58","author":"Gencik","year":"2000","journal-title":"Kidney Int."},{"key":"ref_60","doi-asserted-by":"crossref","first-page":"1054","DOI":"10.1002\/art.40034","article-title":"Identification of Functional and Expression Polymorphisms Associated with Risk for Antineutrophil Cytoplasmic Autoantibody\u2013Associated Vasculitis","volume":"69","author":"Merkel","year":"2017","journal-title":"Arthritis Rheumatol."},{"key":"ref_61","doi-asserted-by":"crossref","first-page":"543","DOI":"10.1111\/j.1365-2796.1994.tb00842.x","article-title":"Strong Link between the Alpha1 -antitrypsin PiZ Allele and Wegener\u2019s Granulomatosis","volume":"236","author":"Elzouki","year":"1994","journal-title":"J. Intern. Med."},{"key":"ref_62","doi-asserted-by":"crossref","first-page":"244","DOI":"10.1006\/clim.2000.4962","article-title":"A Linkage Disequilibrium between Genes at the Serine Protease Inhibitor Gene Cluster on Chromosome 14q32.1 Is Associated with Wegener\u2019s Granulomatosis","volume":"98","author":"Borgmann","year":"2001","journal-title":"Clin. Immunol."},{"key":"ref_63","doi-asserted-by":"crossref","first-page":"5120","DOI":"10.1038\/s41467-019-12515-9","article-title":"Genome-Wide Association Study of Eosinophilic Granulomatosis with Polyangiitis Reveals Genomic Loci Stratified by ANCA Status","volume":"10","author":"Lyons","year":"2019","journal-title":"Nat. Commun."},{"key":"ref_64","doi-asserted-by":"crossref","first-page":"214","DOI":"10.1056\/NEJMoa1108735","article-title":"Genetically Distinct Subsets within ANCA-Associated Vasculitis","volume":"367","author":"Lyons","year":"2012","journal-title":"N. Engl. J. Med."},{"key":"ref_65","doi-asserted-by":"crossref","first-page":"2457","DOI":"10.1002\/art.38036","article-title":"Association of Granulomatosis with Polyangiitis (Wegener\u2019s) With HLA\u2013DPB1*04 and SEMA6A Gene Variants: Evidence From Genome-Wide Analysis","volume":"65","author":"Xie","year":"2013","journal-title":"Arthritis Rheum."},{"key":"ref_66","first-page":"246","article-title":"Role of Epigenetics in Biology and Human Diseases","volume":"20","author":"Moosavi","year":"2016","journal-title":"Iran. Biomed. J."},{"key":"ref_67","doi-asserted-by":"crossref","first-page":"396","DOI":"10.1038\/nature05913","article-title":"Perceptions of Epigenetics","volume":"447","author":"Bird","year":"2007","journal-title":"Nature"},{"key":"ref_68","doi-asserted-by":"crossref","first-page":"106262","DOI":"10.1016\/j.intimp.2020.106262","article-title":"The Epigenetic Face of Lupus: Focus on Antigen-Presenting Cells","volume":"81","author":"Wardowska","year":"2020","journal-title":"Int. Immunopharmacol."},{"key":"ref_69","doi-asserted-by":"crossref","first-page":"102315","DOI":"10.1016\/j.jaut.2019.102315","article-title":"Epigenetic Perspectives on Systemic Autoimmune Disease","volume":"104","author":"Karagianni","year":"2019","journal-title":"J. Autoimmun."},{"key":"ref_70","doi-asserted-by":"crossref","first-page":"1175","DOI":"10.1681\/ASN.2016050548","article-title":"Gene-Specific DNA Methylation Changes Predict Remission in Patients with ANCA-Associated Vasculitis","volume":"28","author":"Jones","year":"2017","journal-title":"J. Am. Soc. Nephrol."},{"key":"ref_71","doi-asserted-by":"crossref","first-page":"3209","DOI":"10.1172\/JCI40034","article-title":"Epigenetic Basis for Aberrant Upregulation of Autoantigen Genes in Humans with ANCA Vasculitis","volume":"120","author":"Ciavatta","year":"2010","journal-title":"J. Clin. Investig."},{"key":"ref_72","doi-asserted-by":"crossref","first-page":"85","DOI":"10.1186\/s13148-016-0251-0","article-title":"Histone Modification Signature at Myeloperoxidase and Proteinase 3 in Patients with Anti-Neutrophil Cytoplasmic Autoantibody-Associated Vasculitis","volume":"8","author":"Yang","year":"2016","journal-title":"Clin. Epigenetics"},{"key":"ref_73","doi-asserted-by":"crossref","first-page":"631","DOI":"10.1189\/jlb.0813452","article-title":"Molecular Mechanisms Regulating the Synergism between IL-32\u03b3 and NOD for the Activation of Eosinophils","volume":"95","author":"Wong","year":"2013","journal-title":"J. Leukoc. Biol."},{"key":"ref_74","doi-asserted-by":"crossref","first-page":"781","DOI":"10.1042\/bj2090781","article-title":"Purification and Some Properties of Myeloperoxidase and Eosinophil Peroxidase from Human Blood","volume":"209","author":"Olsen","year":"1983","journal-title":"Biochem. J."},{"key":"ref_75","doi-asserted-by":"crossref","first-page":"447","DOI":"10.1021\/tx9003775","article-title":"Myeloperoxidase: An Oxidative Pathway for Generating Dysfunctional High-Density Lipoprotein","volume":"23","author":"Shao","year":"2010","journal-title":"Chem. Res. Toxicol."},{"key":"ref_76","doi-asserted-by":"crossref","first-page":"343","DOI":"10.1615\/CritRevImmunol.v25.i5.10","article-title":"Immune Functions of Proteinase 3","volume":"25","author":"Sugawara","year":"2005","journal-title":"Crit. Rev. Immunol."},{"key":"ref_77","doi-asserted-by":"crossref","first-page":"2172","DOI":"10.1111\/j.1523-1755.2004.00640.x","article-title":"Membrane Proteinase 3 Expression and ANCA-Induced Neutrophil Activation","volume":"65","author":"Schreiber","year":"2004","journal-title":"Kidney Int."},{"key":"ref_78","doi-asserted-by":"crossref","first-page":"177","DOI":"10.1189\/jlb.69.2.177","article-title":"Proteinase 3, Wegener\u2019s Autoantigen: From Gene to Antigen","volume":"69","author":"Limburg","year":"2001","journal-title":"J. Leukoc. Biol."},{"key":"ref_79","doi-asserted-by":"crossref","first-page":"10696","DOI":"10.1073\/pnas.1320833111","article-title":"G-CSF Induces Membrane Expression of a Myeloperoxidase Glycovariant That Operates as an E-Selectin Ligand on Human Myeloid Cells","volume":"111","author":"Silvescu","year":"2014","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"ref_80","doi-asserted-by":"crossref","first-page":"573","DOI":"10.1681\/ASN.2012090898","article-title":"Thymic deletion and regulatory T cells prevent antimyeloperoxidase GN","volume":"24","author":"Tan","year":"2013","journal-title":"J. Am. Soc. Nephrol."},{"key":"ref_81","doi-asserted-by":"crossref","first-page":"21","DOI":"10.1016\/j.cellimm.2013.04.007","article-title":"Myeloperoxidase (MPO)-specific CD4+ T cells contribute to MPO-anti-neutrophil cytoplasmic antibody (ANCA) associated glomerulonephritis","volume":"282","author":"Gan","year":"2013","journal-title":"Cell. Immunol."},{"key":"ref_82","doi-asserted-by":"crossref","first-page":"327","DOI":"10.1111\/j.1365-2249.2009.04072.x","article-title":"Anti-PR3 immune responses induce segmental and necrotizing glomerulonephritis","volume":"159","author":"Primo","year":"2010","journal-title":"Clin. Exp. Immunol."},{"key":"ref_83","doi-asserted-by":"crossref","first-page":"973","DOI":"10.1016\/j.kint.2023.01.023","article-title":"Identification of proteinase 3 autoreactive CD4+T cells and their T-cell receptor repertoires in antineutrophil cytoplasmic antibody-associated vasculitis","volume":"103","author":"Sharma","year":"2023","journal-title":"Kidney Int."},{"key":"ref_84","doi-asserted-by":"crossref","first-page":"1271","DOI":"10.4049\/jimmunol.153.3.1271","article-title":"Anti-Neutrophil Cytoplasmic Antibodies Engage and Activate Human Neutrophils via Fc Gamma RIIa","volume":"153","author":"Porges","year":"1994","journal-title":"J. Immunol."},{"key":"ref_85","doi-asserted-by":"crossref","first-page":"661","DOI":"10.1097\/01.ASN.0000050223.34749.F4","article-title":"Activation of the Gi Heterotrimeric G Protein by ANCA IgG F(Ab\u2032)2 Fragments Is Necessary but Not Sufficient to Stimulate the Recruitment of Those Downstream Mediators Used by Intact ANCA IgG","volume":"14","author":"Williams","year":"2003","journal-title":"J. Am. Soc. Nephrol."},{"key":"ref_86","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1016\/j.jaut.2014.12.002","article-title":"Glycans in the Immune System and The Altered Glycan Theory of Autoimmunity: A Critical Review","volume":"57","author":"Maverakis","year":"2015","journal-title":"J. Autoimmun."},{"key":"ref_87","doi-asserted-by":"crossref","first-page":"4115","DOI":"10.1073\/pnas.87.11.4115","article-title":"Anti-Neutrophil Cytoplasmic Autoantibodies Induce Neutrophils to Degranulate and Produce Oxygen Radicals in Vitro","volume":"87","author":"Falk","year":"1990","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"ref_88","doi-asserted-by":"crossref","first-page":"317","DOI":"10.1016\/j.it.2013.02.008","article-title":"Pattern recognition receptor function in neutrophils","volume":"34","author":"Thomas","year":"2013","journal-title":"Trends Immunol."},{"key":"ref_89","doi-asserted-by":"crossref","first-page":"e1008230","DOI":"10.1371\/journal.ppat.1008230","article-title":"CXCR4 and MIF are required for neutrophil extracellular trap release triggered by Plasmodium-infected erythrocytes","volume":"16","author":"Rodrigues","year":"2020","journal-title":"PLoS Pathog."},{"key":"ref_90","doi-asserted-by":"crossref","first-page":"2399","DOI":"10.1681\/ASN.2014070673","article-title":"Neutrophil Extracellular Trap-Related Extracellular Histones Cause Vascular Necrosis in Severe GN","volume":"26","author":"Kumar","year":"2015","journal-title":"J. Am. Soc. Nephrol."},{"key":"ref_91","doi-asserted-by":"crossref","first-page":"E9618","DOI":"10.1073\/pnas.1708247114","article-title":"Necroptosis controls NET generation and mediates complement activation, endothelial damage, and autoimmune vasculitis","volume":"114","author":"Schreiber","year":"2017","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"ref_92","doi-asserted-by":"crossref","first-page":"3007","DOI":"10.1182\/blood-2012-03-416156","article-title":"Neutrophil Extracellular Traps Mediate Transfer of Cytoplasmic Neutrophil Antigens to Myeloid Dendritic Cells toward ANCA Induction and Associated Autoimmunity","volume":"120","author":"Sangaletti","year":"2012","journal-title":"Blood"},{"key":"ref_93","doi-asserted-by":"crossref","first-page":"623","DOI":"10.1038\/nm.1959","article-title":"Netting Neutrophils in Autoimmune Small-Vessel Vasculitis","volume":"15","author":"Kessenbrock","year":"2009","journal-title":"Nat. Med."},{"key":"ref_94","doi-asserted-by":"crossref","first-page":"466","DOI":"10.1111\/pin.12434","article-title":"Presence of Citrullinated Histone H3-Positive Neutrophils in Microscopic Polyangiitis from the Early Phase: An Autopsy Proven Case","volume":"66","author":"Matsuda","year":"2016","journal-title":"Pathol. Int."},{"key":"ref_95","doi-asserted-by":"crossref","first-page":"913","DOI":"10.1007\/s10067-017-3546-4","article-title":"Neutrophil extracellular traps in neuropathy with anti-neutrophil cytoplasmic autoantibody-associated microscopic polyangiitis","volume":"36","author":"Takeuchi","year":"2017","journal-title":"Clin. Rheumatol."},{"key":"ref_96","doi-asserted-by":"crossref","first-page":"149560","DOI":"10.1155\/2012\/149560","article-title":"Deoxyribonuclease is a potential counter regulator of aberrant neutrophil extracellular traps formation after major trauma","volume":"2012","author":"Meng","year":"2012","journal-title":"Mediat. Inflamm."},{"key":"ref_97","doi-asserted-by":"crossref","first-page":"2085","DOI":"10.1093\/rheumatology\/kev217","article-title":"Increased levels of neutrophil extracellular trap remnants in the circulation of patients with small vessel vasculitis, but an inverse correlation to anti-neutrophil cytoplasmic antibodies during remission","volume":"54","author":"Kurz","year":"2015","journal-title":"Rheumatology"},{"key":"ref_98","doi-asserted-by":"crossref","first-page":"990","DOI":"10.1681\/ASN.2013060606","article-title":"Enhanced formation and disordered regulation of NETs in myeloperoxidase-ANCA-associated microscopic polyangiitis","volume":"25","author":"Nakazawa","year":"2014","journal-title":"J. Am. Soc. Nephrol."},{"key":"ref_99","doi-asserted-by":"crossref","unstructured":"Argyropoulou, O.D., Goules, A.V., Boutzios, G., Tsirogianni, A., Sfontouris, C., Manoussakis, M.N., Vlachoyiannopoulos, P.G., Tzioufas, A.G., and Kapsogeorgou, E.K. (2021). Occurrence and Antigenic Specificity of Perinuclear Anti-Neutrophil Cytoplasmic Antibodies (P-ANCA) in Systemic Autoimmune Diseases. Cells, 10.","DOI":"10.3390\/cells10082128"},{"key":"ref_100","doi-asserted-by":"crossref","first-page":"e23","DOI":"10.1136\/annrheumdis-2016-210890","article-title":"Testing for antineutrophil cytoplasmic antibodies (ANCAs) in patients with systemic vasculitides and other diseases","volume":"76","author":"Novikov","year":"2017","journal-title":"Ann. Rheum. Dis."},{"key":"ref_101","doi-asserted-by":"crossref","first-page":"e000058","DOI":"10.1136\/bmjresp-2014-000058","article-title":"Antineutrophil cytoplasmic antibody-positive conversion and microscopic polyangiitis development in patients with idiopathic pulmonary fibrosis","volume":"2","author":"Kagiyama","year":"2015","journal-title":"BMJ Open Respir. Res."},{"key":"ref_102","doi-asserted-by":"crossref","first-page":"92","DOI":"10.1186\/s13075-016-0994-1","article-title":"Impaired Phagocytosis and Reactive Oxygen Species Production in Phagocytes Is Associated with Systemic Vasculitis","volume":"18","author":"Johansson","year":"2016","journal-title":"Arthritis Res. Ther."},{"key":"ref_103","doi-asserted-by":"crossref","first-page":"982","DOI":"10.1080\/07391102.2020.1820381","article-title":"Binding of raloxifene to human complement fragment 5a (hC5a): A perspective on cytokine storm and COVID19","volume":"40","author":"Mishra","year":"2022","journal-title":"J. Biomol. Struct. Dyn."},{"key":"ref_104","doi-asserted-by":"crossref","first-page":"289","DOI":"10.1681\/ASN.2008050497","article-title":"C5a receptor mediates neutrophil activation and ANCA-induced glomerulonephritis","volume":"20","author":"Schreiber","year":"2009","journal-title":"J. Am. Soc. Nephrol."},{"key":"ref_105","doi-asserted-by":"crossref","first-page":"E1461","DOI":"10.1073\/pnas.1417100112","article-title":"L-selectin shedding is activated specifically within transmigrating pseudopods of monocytes to regulate cell polarity in vitro","volume":"112","author":"Rzeniewicz","year":"2015","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"ref_106","doi-asserted-by":"crossref","first-page":"57","DOI":"10.1186\/1742-2094-11-57","article-title":"Comparison of microglia and infiltrating CD11c\u207a cells as antigen presenting cells for T cell proliferation and cytokine response","volume":"11","author":"Wlodarczyk","year":"2014","journal-title":"J. Neuroinflammation"},{"key":"ref_107","doi-asserted-by":"crossref","first-page":"996","DOI":"10.1189\/jlb.71.6.996","article-title":"Wegener\u2019s Granulomatosis: Antiproteinase 3 Antibodies Induce Monocyte Cytokine and Prostanoid Release\u2014Role of Autocrine Cell Activation","volume":"71","author":"Hattar","year":"2002","journal-title":"J. Leukoc. Biol."},{"key":"ref_108","doi-asserted-by":"crossref","first-page":"1698","DOI":"10.1002\/1529-0131(200107)44:7<1698::AID-ART294>3.0.CO;2-J","article-title":"Antineutrophil Cytoplasmic Antibodies Induce Human Monocytes to Produce Oxygen Radicals in Vitro","volume":"44","author":"Weidner","year":"2001","journal-title":"Arthritis Rheum."},{"key":"ref_109","doi-asserted-by":"crossref","first-page":"65","DOI":"10.1111\/cei.12625","article-title":"CD14 Expression Is Increased on Monocytes in Patients with Anti-Neutrophil Cytoplasm Antibody (ANCA)-Associated Vasculitis and Correlates with the Expression of ANCA Autoantigens","volume":"181","author":"Tarzi","year":"2015","journal-title":"Clin. Exp. Immunol."},{"key":"ref_110","doi-asserted-by":"crossref","first-page":"78","DOI":"10.1080\/14397595.2018.1554228","article-title":"Disease Activity, Treatment and Long-Term Prognosis of Adult Juvenile Idiopathic Arthritis Patients Compared with Rheumatoid Arthritis Patients","volume":"30","author":"Matsumoto","year":"2020","journal-title":"Mod. Rheumatol."},{"key":"ref_111","doi-asserted-by":"crossref","first-page":"237","DOI":"10.1136\/ard.58.4.237","article-title":"Monocyte Activation in Patients with Wegener\u2019s Granulomatosis","volume":"58","author":"Kobold","year":"1999","journal-title":"Ann. Rheum. Dis."},{"key":"ref_112","doi-asserted-by":"crossref","first-page":"40","DOI":"10.1681\/ASN.V7140","article-title":"Circulating Leukocyte Integrin Expression in Wegener\u2019s Granulomatosis","volume":"7","author":"Haller","year":"1996","journal-title":"J. Am. Soc. Nephrol."},{"key":"ref_113","doi-asserted-by":"crossref","first-page":"378","DOI":"10.1038\/s41584-023-00958-w","article-title":"Evidence-Based Guideline for the Diagnosis and Management of Eosinophilic Granulomatosis with Polyangiitis","volume":"19","author":"Emmi","year":"2023","journal-title":"Nat. Rev. Rheumatol."},{"key":"ref_114","doi-asserted-by":"crossref","unstructured":"Watanabe, R., and Hashimoto, M. (2023). Eosinophilic Granulomatosis with Polyangiitis: Latest Findings and Updated Treatment Recommendations. J. Clin. Med., 12.","DOI":"10.3390\/jcm12185996"},{"key":"ref_115","doi-asserted-by":"crossref","first-page":"e143366","DOI":"10.1172\/jci.insight.143366","article-title":"A Role for IL-33\u2013Activated ILC2s in Eosinophilic Vasculitis","volume":"6","author":"Kotas","year":"2021","journal-title":"JCI Insight"},{"key":"ref_116","doi-asserted-by":"crossref","first-page":"4043","DOI":"10.4049\/jimmunol.1400732","article-title":"IL-5 Triggers a Cooperative Cytokine Network That Promotes Eosinophil Precursor Maturation","volume":"193","author":"Fulkerson","year":"2014","journal-title":"J. Immunol."},{"key":"ref_117","doi-asserted-by":"crossref","first-page":"1921","DOI":"10.1056\/NEJMoa1702079","article-title":"Mepolizumab or Placebo for Eosinophilic Granulomatosis with Polyangiitis","volume":"376","author":"Wechsler","year":"2017","journal-title":"N. Engl. J. Med."},{"key":"ref_118","doi-asserted-by":"crossref","first-page":"1683","DOI":"10.1002\/art.41727","article-title":"Eosinophil ETosis\u2013Mediated Release of Galectin-10 in Eosinophilic Granulomatosis with Polyangiitis","volume":"73","author":"Fukuchi","year":"2021","journal-title":"Arthritis Rheumatol."},{"key":"ref_119","doi-asserted-by":"crossref","first-page":"550","DOI":"10.4049\/jimmunol.1800107","article-title":"Charcot\u2013Leyden Crystals Activate the NLRP3 Inflammasome and Cause IL-1\u03b2 Inflammation in Human Macrophages","volume":"202","author":"Ataide","year":"2019","journal-title":"J. Immunol."},{"key":"ref_120","doi-asserted-by":"crossref","first-page":"432","DOI":"10.3389\/fimmu.2014.00432","article-title":"T Cell-acrophage Interactions and Granuloma Formation in Vasculitis","volume":"5","author":"Hilhorst","year":"2014","journal-title":"Front. Immunol."},{"key":"ref_121","doi-asserted-by":"crossref","first-page":"13","DOI":"10.55563\/clinexprheumatol\/6y0bjb","article-title":"TIM-3 Regulates the NETs-Mediated Dendritic Cell Activation in Myeloperoxidase-ANCA-Associated Vasculitis","volume":"39","author":"Su","year":"2021","journal-title":"Clin. Exp. Rheumatol."},{"key":"ref_122","doi-asserted-by":"crossref","first-page":"155221","DOI":"10.1016\/j.cyto.2020.155221","article-title":"Impaired HVJ-Stimulated Interferon Producing Capacity in MPO-ANCA-Associated Vasculitis with Rapidly Progressive Glomerulonephritis Lead to Susceptibility to Infection","volume":"136","author":"Uno","year":"2020","journal-title":"Cytokine"},{"key":"ref_123","doi-asserted-by":"crossref","first-page":"642127","DOI":"10.3389\/fimmu.2021.642127","article-title":"Emerging Cellular Therapies for Anti-Myeloperoxidase Vasculitis and Other Autoimmune Diseases","volume":"12","author":"Odobasic","year":"2021","journal-title":"Front. Immunol."},{"key":"ref_124","doi-asserted-by":"crossref","first-page":"102","DOI":"10.3389\/fimmu.2017.00102","article-title":"Dysregulated Responsiveness of Circulating Dendritic Cells to Toll-Like Receptors in ANCA-Associated Vasculitis","volume":"8","author":"Braudeau","year":"2017","journal-title":"Front. Immunol."},{"key":"ref_125","doi-asserted-by":"crossref","first-page":"2151","DOI":"10.1093\/ndt\/gfp019","article-title":"Dendritic Cells in Renal Biopsies of Patients with ANCA-Associated Vasculitis","volume":"24","author":"Wilde","year":"2009","journal-title":"Nephrol. Dial. Transplant."},{"key":"ref_126","doi-asserted-by":"crossref","first-page":"32","DOI":"10.1186\/s12865-014-0032-5","article-title":"An Increase of CD83+ Dendritic Cells Ex Vivo Correlates with Increased Regulatory T Cells in Patients with Active Eosinophilic Granulomatosis and Polyangiitis","volume":"15","author":"Tsurikisawa","year":"2014","journal-title":"BMC Immunol."},{"key":"ref_127","doi-asserted-by":"crossref","first-page":"1145257","DOI":"10.3389\/fmed.2023.1145257","article-title":"Eosinophilic granulomatosis with polyangiitis\u2014Advances in pathogenesis, diagnosis, and treatment","volume":"10","author":"Fijolek","year":"2023","journal-title":"Front. Med."},{"key":"ref_128","doi-asserted-by":"crossref","first-page":"79","DOI":"10.1016\/j.jaut.2008.03.006","article-title":"MPO-ANCA induces IL-17 production by activated neutrophils in vitro via classical complement pathway-dependent manner","volume":"31","author":"Hoshino","year":"2008","journal-title":"J. Autoimmun."},{"key":"ref_129","doi-asserted-by":"crossref","first-page":"227","DOI":"10.1111\/j.1365-2249.1995.tb08343.x","article-title":"Activation of granulocytes by anti-neutrophil cytoplasmic antibodies (ANCA) in Wegener\u2019s granulomatosis: A predominant role for the IgG3 subclass of ANCA","volume":"101","author":"Mulder","year":"1995","journal-title":"Clin. Exp. Immunol."},{"key":"ref_130","doi-asserted-by":"crossref","first-page":"631055","DOI":"10.3389\/fimmu.2021.631055","article-title":"New Insights into Novel Therapeutic Targets in ANCA-Associated Vasculitis","volume":"12","author":"Nozaki","year":"2021","journal-title":"Front. Immunol."},{"key":"ref_131","doi-asserted-by":"crossref","first-page":"503","DOI":"10.1093\/ckj\/sfz029","article-title":"T-Lymphocyte in ANCA-Associated Vasculitis: What Do We Know? A Pathophysiological and Therapeutic Approach","volume":"12","year":"2019","journal-title":"Clin. Kidney J."},{"key":"ref_132","doi-asserted-by":"crossref","first-page":"332","DOI":"10.1046\/j.1365-2249.2001.125002332.x","article-title":"Th2 Dominance in Nasal Mucosa in Patients with Wegener\u2019s Granulomatosis","volume":"125","author":"Balding","year":"2001","journal-title":"Clin. Exp. Immunol."},{"key":"ref_133","doi-asserted-by":"crossref","first-page":"938","DOI":"10.1038\/sj.ki.5001670","article-title":"Persistent Expansion of CD4+ Effector Memory T Cells in Wegener\u2019s Granulomatosis","volume":"70","author":"Abdulahad","year":"2006","journal-title":"Kidney Int."},{"key":"ref_134","doi-asserted-by":"crossref","first-page":"724","DOI":"10.1002\/eji.201646810","article-title":"Skewing toward Treg and Th2 Responses Is a Characteristic Feature of Sustained Remission in ANCA-positive Granulomatosis with Polyangiitis","volume":"47","author":"Szczeklik","year":"2017","journal-title":"Eur. J. Immunol."},{"key":"ref_135","doi-asserted-by":"crossref","first-page":"204","DOI":"10.1186\/ar2923","article-title":"T Cells in ANCA-Associated Vasculitis: What Can We Learn from Lesional versus Circulating T Cells?","volume":"12","author":"Wilde","year":"2010","journal-title":"Arthritis Res. Ther."},{"key":"ref_136","doi-asserted-by":"crossref","first-page":"1825","DOI":"10.1093\/ndt\/gfl097","article-title":"Persistent T-Cell Activation and Clinical Correlations in Patients with ANCA-Associated Systemic Vasculitis","volume":"21","author":"Marinaki","year":"2006","journal-title":"Nephrol. Dial. Transplant."},{"key":"ref_137","doi-asserted-by":"crossref","first-page":"181","DOI":"10.1111\/j.1365-2249.2005.02731.x","article-title":"Abnormalities of CD4+ T Cell Subpopulations in ANCA-Associated Vasculitis","volume":"140","author":"Marinaki","year":"2005","journal-title":"Clin. Exp. Immunol."},{"key":"ref_138","doi-asserted-by":"crossref","first-page":"113","DOI":"10.1046\/j.1365-2249.1998.00695.x","article-title":"Costimulatory Molecules in Wegener\u2019s Granulomatosis (WG): Lack of Expression of CD28 and Preferential up-Regulation of Its Ligands B7-1 (CD80) and B7-2 (CD86) on T Cells","volume":"114","author":"Moosig","year":"2001","journal-title":"Clin. Exp. Immunol."},{"key":"ref_139","doi-asserted-by":"crossref","first-page":"1717","DOI":"10.1016\/S0002-9440(10)61118-2","article-title":"Peripheral Blood and Granuloma CD4+CD28\u2212 T Cells Are a Major Source of Interferon-\u03b3 and Tumor Necrosis Factor-\u03b1 in Wegener\u2019s Granulomatosis","volume":"160","author":"Komocsi","year":"2002","journal-title":"Am. J. Pathol."},{"key":"ref_140","doi-asserted-by":"crossref","first-page":"144","DOI":"10.1016\/j.clim.2007.12.004","article-title":"Expansion of Circulating NKG2D+ Effector Memory T-Cells and Expression of NKG2D-Ligand MIC in Granulomaous Lesions in Wegener\u2019s Granulomatosis","volume":"127","author":"Capraru","year":"2008","journal-title":"Clin. Immunol."},{"key":"ref_141","doi-asserted-by":"crossref","unstructured":"\u017babi\u0144ska, M., Ko\u015bcielska-Kasprzak, K., Krajewska, J., Bartoszek, D., Augustyniak-Bartosik, H., and Krajewska, M. (2021). Immune Cells Profiling in ANCA-Associated Vasculitis Patients\u2014Relation to Disease Activity. Cells, 10.","DOI":"10.3390\/cells10071773"},{"key":"ref_142","doi-asserted-by":"crossref","first-page":"6232","DOI":"10.1073\/pnas.0808144106","article-title":"In Vivo Activated Monocytes from the Site of Inflammation in Humans Specifically Promote Th17 Responses","volume":"106","author":"Evans","year":"2009","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"ref_143","doi-asserted-by":"crossref","first-page":"61","DOI":"10.1159\/000210656","article-title":"Cytokine Production Profile of CD4+ T Cells from Patients with Active Churg-Strauss Syndrome Tends Toward Th17","volume":"149","author":"Saito","year":"2009","journal-title":"Int. Arch. Allergy Immunol."},{"key":"ref_144","doi-asserted-by":"crossref","first-page":"2209","DOI":"10.1093\/ndt\/gfp783","article-title":"Serum IL-17 and IL-23 Levels and Autoantigen-Specific Th17 Cells Are Elevated in Patients with ANCA-Associated Vasculitis","volume":"25","author":"Nogueira","year":"2010","journal-title":"Nephrol. Dial. Transplant."},{"key":"ref_145","doi-asserted-by":"crossref","first-page":"796640","DOI":"10.3389\/fimmu.2021.796640","article-title":"Natural Killer Cells in Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis\u2014A Review of the Literature","volume":"12","author":"Fuchs","year":"2022","journal-title":"Front. Immunol."},{"key":"ref_146","doi-asserted-by":"crossref","first-page":"3399","DOI":"10.4049\/jimmunol.1301508","article-title":"Tissue-Specific Microvascular Endothelial Cells Show Distinct Capacity to Activate NK Cells: Implications for the Pathophysiology of Granulomatosis with Polyangiitis","volume":"192","author":"Tognarelli","year":"2014","journal-title":"J. Immunol."},{"key":"ref_147","doi-asserted-by":"crossref","first-page":"11","DOI":"10.1038\/s41581-018-0074-7","article-title":"Effector and Regulatory B Cells in Immune-Mediated Kidney Disease","volume":"15","author":"Oleinika","year":"2019","journal-title":"Nat. Rev. Nephrol."},{"key":"ref_148","first-page":"156","article-title":"Regulatory B Cells and Mechanisms","volume":"35","author":"Yassin","year":"2015","journal-title":"Int. Rev. Immunol."},{"key":"ref_149","doi-asserted-by":"crossref","first-page":"77","DOI":"10.1016\/j.autrev.2011.08.002","article-title":"Immune Regulatory Mechanisms in ANCA-Associated Vasculitides","volume":"11","author":"Lepse","year":"2011","journal-title":"Autoimmun. Rev."},{"key":"ref_150","doi-asserted-by":"crossref","first-page":"211","DOI":"10.1056\/NEJMoa0909169","article-title":"Rituximab versus Cyclophosphamide in ANCA-Associated Renal Vasculitis","volume":"363","author":"Jones","year":"2010","journal-title":"N. Engl. J. Med."},{"key":"ref_151","doi-asserted-by":"crossref","first-page":"221","DOI":"10.1056\/NEJMoa0909905","article-title":"Rituximab versus Cyclophosphamide for ANCA-Associated Vasculitis","volume":"363","author":"Stone","year":"2010","journal-title":"N. Engl. J. Med."},{"key":"ref_152","doi-asserted-by":"crossref","first-page":"1153","DOI":"10.1093\/rheumatology\/keu452","article-title":"Long-Term Follow-up of Patients Who Received Repeat-Dose Rituximab as Maintenance Therapy for ANCA-Associated Vasculitis","volume":"54","author":"Alberici","year":"2015","journal-title":"Rheumatology"}],"container-title":["International Journal of Molecular Sciences"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/1422-0067\/26\/13\/6065\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,9]],"date-time":"2025-10-09T17:57:52Z","timestamp":1760032672000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/1422-0067\/26\/13\/6065"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2025,6,24]]},"references-count":152,"journal-issue":{"issue":"13","published-online":{"date-parts":[[2025,7]]}},"alternative-id":["ijms26136065"],"URL":"https:\/\/doi.org\/10.3390\/ijms26136065","relation":{},"ISSN":["1422-0067"],"issn-type":[{"value":"1422-0067","type":"electronic"}],"subject":[],"published":{"date-parts":[[2025,6,24]]}}}