{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,30]],"date-time":"2026-10-30T18:34:58Z","timestamp":1793385298904,"version":"4.5.0"},"reference-count":173,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2024,8,27]],"date-time":"2024-08-27T00:00:00Z","timestamp":1724716800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["frontiersin.org"],"crossmark-restriction":true},"short-container-title":["Front. Med."],"abstract":"<jats:p>Granulomatosis with polyangiitis (GPA) is a rare systemic disease characterized by granulomatous inflammation of the respiratory tract and necrotizing vasculitis of small and medium vessels often associated with the production of anti-neutrophil cytoplasmic antibodies (ANCA) directed mainly against leukocyte proteinase 3 (PR3). Usually, it involves upper airways, lungs, and kidneys, however any organ may be affected. The diagnosis is based on clinical, radiological, and serological findings. Biopsies, although strongly recommended, are not always feasible and often provides non-specific features. ANCA plays a crucial role in the diagnosis of GPA; nevertheless, ANCA detection is not a substitute for biopsy, which plays an important role in suspected cases, particularly when histological confirmation cannot be obtained. Significant advances have been made in classification criteria and phenotyping of the disease, particularly in determining the nuances between PR3-ANCA and myeloperoxidase (MPO)-ANCA vasculitis. This has led to better characterization of patients and the development of targeted treatment in the future. In addition, better identification of cytokine and immunological profiles may result in immuno-phenotyping becoming a new approach to identify patients with ANCA-associated vasculitis (AAV). Due to the chronic relapsing\u2013remitting nature, strict follow-up of GPA is necessary to provide appropriate management. The search for the accurate marker of disease activity and to predict relapse is still ongoing and no predictor has been found to reliably guide therapeutic decision-making.<\/jats:p>","DOI":"10.3389\/fmed.2024.1369233","type":"journal-article","created":{"date-parts":[[2024,8,27]],"date-time":"2024-08-27T15:39:28Z","timestamp":1724773168000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":55,"title":["Granulomatosis with polyangiitis: clinical characteristics and updates in diagnosis"],"prefix":"10.3389","volume":"11","author":[{"given":"Malgorzata","family":"Potentas-Policewicz","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Justyna","family":"Fijolek","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1965","published-online":{"date-parts":[[2024,8,27]]},"reference":[{"key":"ref1","doi-asserted-by":"publisher","first-page":"572","DOI":"10.1016\/j.jbspin.2020.06.005","article-title":"Granulomatosis with polyangiitis (Wegener\u2019s)","volume":"87","author":"Puechal","year":"2020","journal-title":"Joint Bone Spine"},{"key":"ref2","doi-asserted-by":"publisher","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":"ref3","doi-asserted-by":"publisher","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":"AJKD"},{"key":"ref4","doi-asserted-by":"publisher","first-page":"22","DOI":"10.1038\/s41584-021-00718-3","article-title":"Global epidemiology of vasculitis","volume":"18","author":"Watts","year":"2022","journal-title":"Nature Rev"},{"key":"ref5","doi-asserted-by":"publisher","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":"ref6","doi-asserted-by":"publisher","first-page":"131","DOI":"10.1007\/5584_2018_239","article-title":"Epidemiology of granulomatosis with polyangiitis in Poland, 2011-2015","volume":"1116","author":"Kanecki","year":"2018","journal-title":"Adv Exp Med Biol"},{"key":"ref7","doi-asserted-by":"publisher","first-page":"53","DOI":"10.1007\/s40266-018-0617-4","article-title":"Aging in primary systemic vasculitis: implications for diagnosis, clinical manifestations, and management","volume":"36","author":"Berti","year":"2019","journal-title":"Drugs Aging"},{"key":"ref8","doi-asserted-by":"publisher","first-page":"414","DOI":"10.1002\/1529-0131(200002)43:2<414::AID-ANR23>3.0.CO;2-0","article-title":"Epidemiology of systemic vasculitis: a ten-year study in the United Kingdom","volume":"43","author":"Watts","year":"2000","journal-title":"Arthritis Rheum"},{"key":"ref9","doi-asserted-by":"publisher","first-page":"108325","DOI":"10.1016\/j.clim.2019.108325","article-title":"Childhood GPA, EGPA, and MPA","volume":"211","author":"Jariwala","year":"2020","journal-title":"Clin Immunol"},{"key":"ref10","doi-asserted-by":"publisher","first-page":"434","DOI":"10.1055\/s-0038-1660874","article-title":"Granulomatosis with polyangiitis (Wegener\u2019s granulomatosis): evolving concepts in treatment","volume":"39","author":"Lynch","year":"2018","journal-title":"Semin Respir Crit Care Med"},{"key":"ref11","doi-asserted-by":"publisher","first-page":"599","DOI":"10.1016\/j.jbspin.2015.07.014","article-title":"Patient-driven online survey on the clinical manifestations and diagnostic delay of granulomatosis with polyangiitis","volume":"83","author":"Jacyshyn","year":"2016","journal-title":"Joint Bone Spine"},{"key":"ref12","doi-asserted-by":"publisher","first-page":"11","DOI":"10.2478\/rir-2023-0003","article-title":"Pathogenesis of anti-neutrophil cytoplasmic antibody-associated vasculitis","volume":"4","author":"Sun","year":"2023","journal-title":"Rheumatol Immunol Res"},{"key":"ref13","doi-asserted-by":"publisher","first-page":"25","DOI":"10.3390\/antib12010025","article-title":"New insights into pathogenesis and treatment of ANCA-associated vasculitis: autoantibodies and beyond","volume":"12","author":"Paroli","year":"2023","journal-title":"Antibodies"},{"key":"ref14","doi-asserted-by":"publisher","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":"ref15","article-title":"STEAP3, FZD2 and EGFLAM are novel genetic risk loci for granulomatosis with polyangiitis: a genome wide association study from UK biobank (abstract)","volume":"73","author":"Hocaoglu","year":"2021","journal-title":"Arthritis Rheumatol"},{"key":"ref16","doi-asserted-by":"publisher","first-page":"104581","DOI":"10.1016\/j.ebiom.2023.104581","article-title":"Identifying shared genetic architecture between rheumatoid arthritis and other conditions: a phenome-wide association study with genetic risk scores","volume":"92","author":"Zhang","year":"2023","journal-title":"Lancet"},{"key":"ref17","doi-asserted-by":"publisher","first-page":"e40786","DOI":"10.7759\/cureus.40786","article-title":"Familial association of granulomatosis with polyangiitis: a case-based review of literature","volume":"15","author":"Farrukh","year":"2023","journal-title":"Cureus"},{"key":"ref18","doi-asserted-by":"publisher","first-page":"1121","DOI":"10.1016\/j.autrev.2014.08.017","article-title":"Granulomatosis with polyangiitis (Wegener): clinical aspects and treatment","volume":"13","author":"Comarmond","year":"2014","journal-title":"Autoimmun Rev"},{"key":"ref19","doi-asserted-by":"publisher","first-page":"S16","DOI":"10.3949\/ccjm.79.s3.04","article-title":"Upper airway manifestations of granulomatosis with polyangiitis","volume":"79","author":"Alam","year":"2012","journal-title":"Cleve Clin J Med"},{"key":"ref20","doi-asserted-by":"publisher","first-page":"339","DOI":"10.1016\/j.semarthrit.2021.02.002","article-title":"Granulomatosis with polyangiitis: study of 795 patients from the French Vasculitis study group","volume":"51","author":"Iudici","year":"2021","journal-title":"Sem Arthritis Rheum."},{"key":"ref21","doi-asserted-by":"publisher","first-page":"e6083","DOI":"10.1097\/MD.0000000000006083","article-title":"Clinical characteristics and outcome of Spanish patients with ANCA-associated vasculitides","volume":"96","author":"Solans-Laqu\u00e9","year":"2017","journal-title":"Medicine (Baltimore)"},{"key":"ref22","doi-asserted-by":"publisher","first-page":"2553","DOI":"10.1007\/s10067-019-04538-w","article-title":"Clinical characteristics of polish patients with ANCA-associated vasculitides-retrospective analysis of POLVAS registry","volume":"38","author":"Wojcik","year":"2019","journal-title":"Clin Rheumatol"},{"key":"ref23","doi-asserted-by":"publisher","first-page":"104039","DOI":"10.1016\/j.lpm.2020.104039","article-title":"Lung involvement in ANCA-associated vasculitis","volume":"49","author":"Sacoto","year":"2020","journal-title":"Presse Med"},{"key":"ref24","doi-asserted-by":"publisher","first-page":"676","DOI":"10.2214\/AJR.08.1837","article-title":"Wegener\u2019s granulomatosis in the chest: high-resolution CT findings","volume":"192","author":"Ananthakrishnan","year":"2009","journal-title":"AJR"},{"key":"ref25","doi-asserted-by":"publisher","first-page":"471","DOI":"10.1016\/j.ejrad.2004.04.016","article-title":"Pulmonary manifestations of Wegener granulomatosis: CT findings in 57 patients and a review of the literature","volume":"53","author":"Lohrmann","year":"2005","journal-title":"Eur J Radiol"},{"key":"ref26","doi-asserted-by":"publisher","first-page":"573","DOI":"10.1016\/j.ccm.2019.05.012","article-title":"Update on the management of respiratory manifestations of the antineutrophil cytoplasmic antibodies-associated vasculitides","volume":"40","author":"Thompson","year":"2019","journal-title":"Clin Chest Med"},{"key":"ref27","doi-asserted-by":"publisher","first-page":"1973","DOI":"10.1148\/rg.2021210132","article-title":"From head to toe: granulomatosis with polyangiitis","volume":"41","author":"Guzman-Soto","year":"2021","journal-title":"Radiographics"},{"key":"ref28","doi-asserted-by":"publisher","first-page":"2042","DOI":"10.3899\/jrheum.151355","article-title":"Intratracheal dilation-injection technique in the treatment of granulomatosis with polyangiitis patients with subglottic stenosis","volume":"43","author":"Fijolek","year":"2016","journal-title":"J Rheumatol"},{"key":"ref29","doi-asserted-by":"publisher","first-page":"755","DOI":"10.1016\/j.rdc.2007.09.004","article-title":"Airway involvement in Wegener\u2019s granulomatosis","volume":"33","author":"Polychronopoulos","year":"2007","journal-title":"Rheum Dis Clin N Am"},{"key":"ref30","doi-asserted-by":"publisher","first-page":"70","DOI":"10.1016\/j.semarthrit.2017.12.002","article-title":"Bronchiectasis is highly prevalent in anti-MPO ANCA-associated vasculitis and is associated with a distinct disease presentation","volume":"48","author":"Neel","year":"2018","journal-title":"Sem Arthritis Rheum"},{"key":"ref31","doi-asserted-by":"publisher","first-page":"522","DOI":"10.1164\/ajrccm.151.2.7842215","article-title":"Tracheobronchial involvement in Wegener\u2019s granulomatosis","volume":"151","author":"Daum","year":"1995","journal-title":"Am J Respir Crit Care Med"},{"key":"ref32","doi-asserted-by":"publisher","first-page":"2203","DOI":"10.1093\/rheumatology\/kez217","article-title":"Subglottic stenosis and endobronchial disease in granulomatosis with polyangiitis","volume":"58","author":"Quinn","year":"2019","journal-title":"Rheumatology (Oxford)"},{"key":"ref33","doi-asserted-by":"publisher","first-page":"12","DOI":"10.1159\/000486899","article-title":"The respiratory system in autoimmune vascular diseases","volume":"96","author":"Nasser","year":"2018","journal-title":"Respiration"},{"key":"ref34","first-page":"821","article-title":"A rare case of bronchial stenosis in Wegener\u2019s granulomatosis. Dramatic response to intravenous cyclophosphamide and oral cotrimoxazole","volume":"19","author":"Hirsch","year":"1992","journal-title":"J Rheumatol"},{"key":"ref35","doi-asserted-by":"publisher","first-page":"277","DOI":"10.1177\/021849230201000323","article-title":"Wegener\u2019s granulomatosis presenting as necrosis of the left mainstem bronchus","volume":"10","author":"Schneider","year":"2002","journal-title":"Asian Cardiovasc Thorac Ann"},{"key":"ref36","doi-asserted-by":"publisher","first-page":"1301","DOI":"10.1016\/j.rmed.2013.03.017","article-title":"The \u201cforgotten zone\u201d: acquired disorders of the trachea in adults","volume":"107","author":"Al-Qadi","year":"2013","journal-title":"Respir Med"},{"key":"ref37","doi-asserted-by":"publisher","first-page":"e29909","DOI":"10.7759\/cureus.29909","article-title":"Granulomatosis with polyangiitis presenting with diffuse alveolar hemorrhage: a systematic review","volume":"14","author":"Da Silva","year":"2022","journal-title":"Cureus"},{"key":"ref38","doi-asserted-by":"publisher","first-page":"459","DOI":"10.1055\/s-0038-1673387","article-title":"Microscopic polyangiitis: new insights into pathogenesis, clinical features and therapy","volume":"39","author":"Karras","year":"2018","journal-title":"Semin Respir Crit Care Med"},{"key":"ref39","doi-asserted-by":"publisher","first-page":"157","DOI":"10.1093\/mr\/road001","article-title":"Evaluation of prognostic factors for patients with eosinophilic granulomatosis with polyangiitis recruited at the pneumonological Centre and mainly ANCA negativity: a retrospective analysis of a single cohort in Poland","volume":"34","author":"Fijolek","year":"2023","journal-title":"Mod Rheumatol"},{"key":"ref40","doi-asserted-by":"publisher","first-page":"1166","DOI":"10.1136\/annrheumdis-2015-208073","article-title":"Clinical outcomes of treatment of anti-neutrophil cytoplasmic antibody (ANCA) \u2013 associated vasculitis based on ANCA type","volume":"75","author":"Unizony","year":"2016","journal-title":"Ann Rheum Dis"},{"key":"ref41","doi-asserted-by":"publisher","first-page":"222","DOI":"10.1097\/00005792-200007000-00003","article-title":"Microscopic polyangiitis with alveolar hemorrhage. A study of 29 cases and review of the literature","volume":"79","author":"Lauque","year":"2000","journal-title":"Medicine"},{"key":"ref42","doi-asserted-by":"publisher","first-page":"102397","DOI":"10.1016\/j.jaut.2019.102397","article-title":"Alveolar haemorrhage in ANCA-associated vasculitis: long-term outcome and mortality predictors","volume":"108","author":"Quartuccio","year":"2020","journal-title":"J Autoimmun"},{"key":"ref43","doi-asserted-by":"publisher","first-page":"1467","DOI":"10.1002\/art.39562","article-title":"Diffuse alveolar hemorrhage secondary to antineutrophil cytoplasmic antibody-associated vasculitis: predictors of respiratory failure and clinical outcomes","volume":"68","author":"Cartin-Ceba","year":"2016","journal-title":"Arthritis Rheumatol"},{"key":"ref44","doi-asserted-by":"publisher","first-page":"59","DOI":"10.1016\/j.rmed.2017.06.003","article-title":"Immune diffuse alveolar hemorrhage: clinical presentation and outcome","volume":"129","author":"Quadrelli","year":"2017","journal-title":"Respir Med"},{"key":"ref45","doi-asserted-by":"publisher","first-page":"722","DOI":"10.1016\/j.autrev.2017.05.008","article-title":"Interstitial lung disease in ANCA vasculitis","volume":"16","author":"Alba","year":"2017","journal-title":"Autoimmun Rev"},{"key":"ref46","first-page":"S221","article-title":"Epidemiology and management of interstitial lung disease in ANCA-associated vasculitis","volume":"38","author":"Sebastiani","year":"2020","journal-title":"Clin Exp Rheumatol"},{"key":"ref47","doi-asserted-by":"publisher","first-page":"1503","DOI":"10.1183\/09031936.00148409","article-title":"The oxidation induced by antimyeloperoxidase antibodies triggers fibrosis in microscopic polyangiitis","volume":"37","author":"Guilpain","year":"2011","journal-title":"Eur Respir J"},{"key":"ref48","doi-asserted-by":"publisher","first-page":"1","DOI":"10.1177\/1479973121994562","article-title":"Impact of interstitial lung disease on mortality in ANCA-associated vasculitis: a systematic literature review and meta-analysis","volume":"18","author":"Zhou","year":"2021","journal-title":"Chronic Respir Dis"},{"key":"ref49","doi-asserted-by":"publisher","first-page":"716","DOI":"10.1093\/rheumatology\/keac339","article-title":"Interstitial lung disease in ANCA-associated vasculitis: associated factors, radiographic features and mortality","volume":"62","author":"Doliner","year":"2023","journal-title":"Rheumatology"},{"key":"ref50","doi-asserted-by":"publisher","first-page":"821","DOI":"10.55563\/clinexprheumatol\/xu4hmh","article-title":"Interstitial lung disease in microscopic polyangiitis and granulomatosis with polyangiitis: demographic, clinical, serological and radiological features of an Italian cohort from the Italian Society for Rheumatology","volume":"41","author":"Manfredi","year":"2023","journal-title":"Clin Exp Rheumatol"},{"key":"ref51","doi-asserted-by":"publisher","first-page":"102338","DOI":"10.1016\/j.jaut.2019.102338","article-title":"Usual interstitial pneumonia in ANCA-associated vasculitis: a poor prognostic factor","volume":"106","author":"Maillet","year":"2020","journal-title":"J Autoimmun"},{"key":"ref52","doi-asserted-by":"publisher","first-page":"116","DOI":"10.1183\/09031936.00110109","article-title":"Prevalence and outcome of pulmonary fibrosis in microscopic polyangiitis","volume":"36","author":"Tzelepis","year":"2010","journal-title":"Eur Respir J"},{"key":"ref53","doi-asserted-by":"publisher","first-page":"257","DOI":"10.1159\/000529085","article-title":"Patterns of interstitial lung disease and prognosis in antineutrophil cytoplasmic antibody-associated vasculitis","volume":"102","author":"Zhou","year":"2023","journal-title":"Respiration"},{"key":"ref54","doi-asserted-by":"publisher","first-page":"340","DOI":"10.1097\/MD.0000000000000217","article-title":"Pulmonary fibrosis in antineutrophil cytoplasmic antibodies (ANCA) \u2013 associated vasculitis","volume":"93","author":"Comarmond","year":"2014","journal-title":"Medicine (Baltimore)"},{"key":"ref55","doi-asserted-by":"publisher","first-page":"100","DOI":"10.1002\/cre2.706","article-title":"Oral granulomatosis with polyangiitis a systematic review","volume":"9","author":"Labrador","year":"2023","journal-title":"Clin Exp Dent Res"},{"key":"ref56","doi-asserted-by":"publisher","first-page":"884188","DOI":"10.3389\/fmed.2022.884188","article-title":"Diagnostic and therapeutic approach in ANCA-associated glomerulonephritis: a review on management strategies","volume":"9","author":"Molnar","year":"2022","journal-title":"Front Med"},{"key":"ref57","doi-asserted-by":"publisher","first-page":"1700","DOI":"10.1002\/art.39637","article-title":"Factors determining the clinical utility of serial measurements of antineutrophil cytoplasmic antibodies targeting proteinase 3","volume":"68","author":"Fussner","year":"2016","journal-title":"Arthritis Rheumatol"},{"key":"ref58","doi-asserted-by":"publisher","first-page":"1209","DOI":"10.3899\/jrheum.141513","article-title":"Cardiac involvement in granulomatosis with polyangiitis","volume":"42","author":"McGeoch","year":"2015","journal-title":"J Rheumatol"},{"key":"ref59","doi-asserted-by":"publisher","first-page":"170","DOI":"10.1016\/j.ijcard.2015.06.087","article-title":"Prevalence and prognostic relevance of cardiac involvement in ANCA-associated vasculitis: eosinophilic granulomatosis with polyangiitis and granulomatosis with polyangiitis","volume":"199","author":"Hazebroek","year":"2015","journal-title":"Int J Cardiol"},{"key":"ref60","doi-asserted-by":"publisher","first-page":"1053","DOI":"10.1007\/s10554-020-02066-2","article-title":"Cardiac magnetic resonance imaging for the detection of myocardial involvement in granulomatosis with polyangiitis","volume":"37","author":"Giollo","year":"2021","journal-title":"Int J Cardiovasc Imaging"},{"key":"ref61","doi-asserted-by":"publisher","first-page":"1166","DOI":"10.3389\/fneur.2018.01166","article-title":"Central nervous system involvement in ANCA-associated vasculitis: what neurologists need to know","volume":"9","author":"Zheng","year":"2019","journal-title":"Front Neurol"},{"key":"ref62","doi-asserted-by":"publisher","first-page":"40","DOI":"10.1097\/BOR.0000000000000559","article-title":"Vasculitis and peripheral neuropathy","volume":"31","author":"Graf","year":"2019","journal-title":"Curr Opin Rheumatol"},{"key":"ref63","doi-asserted-by":"publisher","first-page":"e002953","DOI":"10.1136\/rmdopen-2022-002953","article-title":"Score to assess the probability of relapse in granulomatosis with polyangiitis and microscopic polyangiitis","volume":"9","author":"Samson","year":"2023","journal-title":"RMD Open"},{"key":"ref64","doi-asserted-by":"publisher","first-page":"16533","DOI":"10.20452\/pamw.16533","article-title":"Functional magnetic resonance imaging (fMRI) for assessment of the autonomic dysfunction in patients with ANCA-associated vasculitides","volume":"14","author":"Wludarczyk","year":"2023","journal-title":"Pol Arch Intern Med"},{"key":"ref65","doi-asserted-by":"publisher","first-page":"311","DOI":"10.1016\/j.jare.2020.05.009","article-title":"Three patterns of cutaneous involvement in granulomatosis with polyangiitis","volume":"24","author":"Ragab","year":"2020","journal-title":"J Adv Res"},{"key":"ref66","doi-asserted-by":"publisher","first-page":"167","DOI":"10.31348\/2018\/5\/1","article-title":"Ocular manifestations of granulomatosis with polyangiitis","volume":"74","author":"Rihova","year":"2018","journal-title":"Ces A Slov Oftal"},{"key":"ref67","doi-asserted-by":"publisher","first-page":"1751","DOI":"10.1007\/s00417-018-4014-9","article-title":"Clinical features of different orbital manifestations of granulomatosis with polyangiitis","volume":"256","author":"Ismailova","year":"2018","journal-title":"Graefes Arch Clin Exp Ophtalmol"},{"key":"ref68","doi-asserted-by":"publisher","first-page":"19","DOI":"10.1097\/MD.0b013e318205a4c6","article-title":"The five - factor score revisited: assessment of prognoses of systemic necrotizing vasculitides based on the French Vasculitis study group (FVSG) cohort","volume":"90","author":"Guillevin","year":"2011","journal-title":"Medicine (Baltimore)"},{"key":"ref69","doi-asserted-by":"publisher","first-page":"270","DOI":"10.5114\/pg.2016.57887","article-title":"Gastrointestinal tract involvement in granulomatosis with polyangiitis","volume":"4","author":"Masiak","year":"2016","journal-title":"Gastroenterology Rev"},{"key":"ref70","doi-asserted-by":"publisher","first-page":"529","DOI":"10.3899\/jrheum.170249","article-title":"Frequency, diagnosis, treatment and outcome of gastrointestinal disease in granulomatosis with polyangiitis and microscopic polyangiitis","volume":"45","author":"Eriksson","year":"2018","journal-title":"J Rheumatol"},{"key":"ref71","doi-asserted-by":"publisher","first-page":"187","DOI":"10.1002\/art.1780370206","article-title":"Nomenclature of systemic vasculitides. Proposal of an international consensus conference","volume":"37","author":"Jennette","year":"1994","journal-title":"Arthritis Rheum"},{"key":"ref72","doi-asserted-by":"publisher","first-page":"1615","DOI":"10.1093\/rheumatology\/kem146a","article-title":"BSR and BHPR guidelines for the management of adults with ANCA associated vasculitis","volume":"46","author":"Lapraik","year":"2007","journal-title":"Rheumatology"},{"key":"ref73","doi-asserted-by":"publisher","first-page":"30","DOI":"10.1136\/ard-2022-223764","article-title":"EULAR recommendations for the management of ANCA-associated vasculitis: 2022 update","volume":"83","author":"Hellmich","year":"2023","journal-title":"Ann Rheum Dis"},{"key":"ref74","doi-asserted-by":"publisher","first-page":"1101","DOI":"10.1002\/art.1780330807","article-title":"The American College of Rheumatology 1990 criteria for the classification of Wegener\u2019s granulomatosis","volume":"33","author":"Leavitt","year":"1990","journal-title":"Arthritis Rheum"},{"key":"ref75","doi-asserted-by":"publisher","first-page":"222","DOI":"10.1136\/ard.2006.054593","article-title":"Development and validation of a consensus methodology for the classification of the ANCA-associated vasculitides and polyarteritis nodosa for epidemiological studies","volume":"66","author":"Watts","year":"2007","journal-title":"Ann Rheum Dis"},{"key":"ref76","doi-asserted-by":"publisher","first-page":"393","DOI":"10.1002\/art.41986","article-title":"2022 American College of Rheumatology\/European Alliance of associations for rheumatology classification criteria for granulomatosis with polyangiitis","volume":"74","author":"Robson","year":"2022","journal-title":"Arthritis Rheumatol"},{"key":"ref77","doi-asserted-by":"publisher","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":"ref78","doi-asserted-by":"publisher","first-page":"1787","DOI":"10.1093\/rheumatology\/keac623","article-title":"Diagnosing and treating ANCA-associated vasculitis: an updated review for clinical practice","volume":"62","author":"Chevet","year":"2023","journal-title":"Rheumatology (Oxford)"},{"key":"ref79","doi-asserted-by":"publisher","first-page":"218","DOI":"10.5114\/ceji.2019.92494","article-title":"Antineutrophil cytoplasmic antibodies \u2013 their role in pathogenesis, diagnosis and treatment monitoring of ANCA-associated vasculitis","volume":"45","author":"Fijolek","year":"2020","journal-title":"Cent Eur J Immunol"},{"key":"ref80","doi-asserted-by":"publisher","first-page":"683","DOI":"10.1038\/nrrheum.2017.140","article-title":"Position paper: revised 2017 international consensus on testing of ANCA in granulomatosis with polyangiitis and microscopic polyangiitis","volume":"13","author":"Bossuyt","year":"2017","journal-title":"Nat Rev Rheumatol"},{"key":"ref81","doi-asserted-by":"publisher","first-page":"102579","DOI":"10.1016\/j.jaut.2020.102579","article-title":"Anti-neutrophil cytoplasmic antibodies predate symptom onset of ANCA-associated vasculitis","volume":"117","author":"Berglin","year":"2021","journal-title":"Case Control Study J Autoimmun"},{"key":"ref82","doi-asserted-by":"publisher","first-page":"102618","DOI":"10.1016\/j.autrev.2020.102618","article-title":"2020 international consensus on ANCA testing beyond systemic vasculitis","volume":"19","author":"Moiseev","year":"2020","journal-title":"Autoimmun Rev"},{"key":"ref83","doi-asserted-by":"publisher","first-page":"103100","DOI":"10.1016\/j.autrev.2022.103100","article-title":"Performance of MPO-ANCA and PR3-ANCA immunoassays for the stratification of specific ANCA-associated vasculitis: a systematic review and meta-analysis","volume":"21","author":"Walker","year":"2022","journal-title":"Autoimmun Rev"},{"key":"ref84","doi-asserted-by":"publisher","first-page":"e5096","DOI":"10.1097\/MD.0000000000005096","article-title":"Diagnosing ANCA-associated vasculitis in ANCA positive patients. A retrospective analysis on the role of clinical symptoms and the ANCA titre","volume":"95","author":"Houben","year":"2016","journal-title":"Medicine (Baltimore)"},{"key":"ref85","doi-asserted-by":"publisher","first-page":"1135","DOI":"10.3389\/fimmu.2019.01135","article-title":"PTX3 intercepts vascular inflammation in systemic immune-mediated diseases","volume":"10","author":"Ramirez","year":"2019","journal-title":"Front Immunol"},{"key":"ref86","doi-asserted-by":"publisher","first-page":"293","DOI":"10.1080\/03009742.2022.2045790","article-title":"Pentraxin-3 \u2013 a potential biomarker in ANCA-associated vasculitis","volume":"52","author":"Jonasdottir","year":"2023","journal-title":"Scand J Rheumatol"},{"key":"ref87","doi-asserted-by":"publisher","first-page":"e0147091","DOI":"10.1371\/journal.pone.0147091","article-title":"Detection of anti-pentraxin-3 autoantibodies in ANCA-associated vasculitis","volume":"11","author":"Simon","year":"2016","journal-title":"PLoS One"},{"key":"ref88","doi-asserted-by":"publisher","first-page":"S3","DOI":"10.3949\/ccjm.79.s3.01","article-title":"Clinical features and diagnosis of small-vessel vasculitis","volume":"79","author":"Langford","year":"2012","journal-title":"Cleve Clin J Med"},{"key":"ref89","doi-asserted-by":"publisher","first-page":"57","DOI":"10.5334\/jbr-btr.36","article-title":"Thoracic involvement in systemic primary vasculitis: radiological patterns and follow-up","volume":"97","author":"Serra","year":"2014","journal-title":"JBR-BTR"},{"key":"ref90","first-page":"26034","article-title":"Update in the study of granulomatosis with polyangiitis (Wegener\u2019s granulomatosis)","volume":"25","author":"De Guevara","year":"2019","journal-title":"Rev Chil Radiol"},{"key":"ref91","doi-asserted-by":"publisher","first-page":"347","DOI":"10.1007\/s13244-014-0335-3","article-title":"Clinical and radiological features of idiopathic interstitial pneumonias (IIPs): a pictorial review","volume":"5","author":"Palmucci","year":"2014","journal-title":"Insights Imaging"},{"key":"ref92","doi-asserted-by":"publisher","first-page":"34","DOI":"10.1097\/00005382-200301000-00005","article-title":"Computed tomography features of the thoracic manifestations of Wegener granulomatosis","volume":"18","author":"Sheenan","year":"2003","journal-title":"J Thorac Imag"},{"key":"ref93","doi-asserted-by":"publisher","first-page":"399","DOI":"10.3389\/fimmu.2014.00399","article-title":"Non-invasive imaging of vascular inflammation","volume":"5","author":"Ammirati","year":"2014","journal-title":"Front Immunol"},{"key":"ref94","doi-asserted-by":"publisher","first-page":"143","DOI":"10.1186\/s13244-022-01284-7","article-title":"Imaging of intestinal vasculitis focusing on MR and CT enterography: a two-way street between radiologic findings and clinical data","volume":"13","author":"Amouei","year":"2022","journal-title":"Insights Imag"},{"key":"ref95","doi-asserted-by":"publisher","first-page":"125","DOI":"10.1016\/j.autrev.2013.09.009","article-title":"FDG-PET\/CT in patients with ANCA-associated vasculitis: case-series and literature review","volume":"13","author":"Soussan","year":"2014","journal-title":"Autoimmun Rev"},{"key":"ref96","first-page":"342","article-title":"Characterization of F-18 fluorodeoxyglucose PET\/CT in granulomatosis with polyangiitis","volume":"32","author":"Nelson","year":"2016","journal-title":"Sarcoidosis Vasc Diffuse Lung Dis"},{"key":"ref97","doi-asserted-by":"publisher","first-page":"351","DOI":"10.1186\/s13023-020-01621-3","article-title":"French recommendations for the management of systemic necrotizing vasculitides (polyarteritis nodosa and ANCA-associated vasculitides)","volume":"15","author":"Terrier","year":"2020","journal-title":"Orphanet J Rare Dis"},{"key":"ref98","doi-asserted-by":"publisher","first-page":"2649","DOI":"10.1093\/eurheartj\/ehx321","article-title":"Diagnosis and management of myocardial involvement in systemic immune-mediated diseases: a position statement of the European Society of Cardiology Working Group on myocardial and pericardial disease","volume":"38","author":"Caforio","year":"2017","journal-title":"Eur Heart J"},{"key":"ref99","doi-asserted-by":"publisher","first-page":"e308","DOI":"10.1093\/ehjci\/jeac134","article-title":"Cardiovascular magnetic resonance in autoimmune rheumatic diseases: a clinical consensus document by the European Association of Cardiovascular Imaging","volume":"23","author":"Mavrogeni","year":"2022","journal-title":"Eur Heart J"},{"key":"ref100","first-page":"51","article-title":"The significance of cardiac magnetic resonance in detection and monitoring of the treatment efficacy of heart involvement in eosinophilic granulomatosis with polyangiitis","volume":"33","author":"Fijolek","year":"2016","journal-title":"Sarcoidosis Vasc Diffuse Lung Dis"},{"key":"ref101","doi-asserted-by":"publisher","first-page":"947","DOI":"10.1093\/rheumatology\/kew490","article-title":"Cardiac involvement in granulomatosis with polyangiitis: a magnetic resonance imaging study of 31 consecutive patients","volume":"56","author":"Pugnet","year":"2017","journal-title":"Rheumatology (Oxford)"},{"key":"ref102","doi-asserted-by":"publisher","first-page":"34","DOI":"10.1007\/s11926-020-00915-6","article-title":"Imaging in vasculitis","volume":"22","author":"Guggenberger","year":"2020","journal-title":"Curr Rheumatol Rep"},{"key":"ref103","doi-asserted-by":"publisher","first-page":"227","DOI":"10.1007\/s40123-019-0176-8","article-title":"Ocular manifestations of granulomatosis with polyangiitis: a review of the literature","volume":"8","author":"Sfiniadaki","year":"2019","journal-title":"Ophtalmol Ther"},{"key":"ref104","doi-asserted-by":"publisher","first-page":"S3","DOI":"10.21037\/cdt.2018.02.01","article-title":"Nonivasive aortic imaging","volume":"8","author":"Baliyan","year":"2018","journal-title":"Cardiovasc Diagn Ther"},{"key":"ref105","doi-asserted-by":"publisher","first-page":"27","DOI":"10.1007\/5584_2017_43","article-title":"Echocardiographic assessment in patients with granulomatosis with polyangiitis","volume":"1022","author":"Zycinska","year":"2017","journal-title":"Adv Exp Med Biol"},{"key":"ref106","doi-asserted-by":"publisher","first-page":"198","DOI":"10.15557\/JoU.2018.0030","article-title":"International evidence-based recommendations for lung ultrasound in internal medicine (POLUUS-IM)","volume":"18","author":"Buda","year":"2018","journal-title":"J Ultrason"},{"key":"ref107","doi-asserted-by":"publisher","first-page":"e0222189","DOI":"10.1371\/journal.pone.0222189","article-title":"Utility of lung ultrasound in ANCA-associated vasculitis with lung involvement","volume":"14","author":"Buda","year":"2018","journal-title":"PLoS One"},{"key":"ref108","doi-asserted-by":"publisher","first-page":"00141","DOI":"10.1183\/23120541-00141-2023","article-title":"Role of bronchoscopy for respiratory involvement in granulomatosis with polyangiitis and microscopic polyangiitis","volume":"9","author":"Villeneuve","year":"2023","journal-title":"Eur Respir J Open Res"},{"key":"ref109","doi-asserted-by":"publisher","first-page":"115","DOI":"10.1097\/01.md.0000158825.87055.0b","article-title":"Presentation and outcome of gastrointestinal involvement in systemic necrotizing vasculitides. Analysis of 62 patients with polyarteritis nodosa, microscopic polyangiitis, Wegener granulomatosis, Churg-Strauss syndrome, or rheumatoid arthritis-associated vasculitis","volume":"84","author":"Pagnoux","year":"2005","journal-title":"Medicine (Baltimore)"},{"key":"ref110","doi-asserted-by":"publisher","first-page":"542","DOI":"10.5009\/gnl15198","article-title":"Endoscopic findings of upper gastrointestinal involvement in primary vasculitis","volume":"10","author":"Gong","year":"2014","journal-title":"Gut Liver"},{"key":"ref111","doi-asserted-by":"publisher","first-page":"777","DOI":"10.1002\/acr.23332","article-title":"Accuracy of magnetic resonance imaging for grading subglottic stenosis in patients with granulomatosis with polyangiitis: correlation with pulmonary function tests and laryngoscopy","volume":"70","author":"Henes","year":"2018","journal-title":"Arthritis Care Res"},{"key":"ref112","doi-asserted-by":"publisher","first-page":"315","DOI":"10.1097\/00000478-199104000-00001","article-title":"Surgical pathology of the lung in Wegener\u2019s granulomatosis. Review of 87 open lung biopsies from 67 patients","volume":"15","author":"Travis","year":"1991","journal-title":"Am J Surg Pathol"},{"key":"ref113","doi-asserted-by":"publisher","first-page":"488","DOI":"10.7326\/0003-4819-116-6-488","article-title":"Wegener granulomatosis: an analysis of 158 patients","volume":"116","author":"Hoffman","year":"1992","journal-title":"Ann Intern Med"},{"key":"ref114","doi-asserted-by":"publisher","first-page":"258","DOI":"10.5603\/ARM.2019.0064","article-title":"Difficulties in recognizing granulomatosis with polyangiitis (GPA) in elderly patients undergoing diagnostic thoracotomy twice \u2013 a report of two cases","volume":"87","author":"Fijolek","year":"2019","journal-title":"Adv Respir Med"},{"key":"ref115","doi-asserted-by":"publisher","first-page":"230","DOI":"10.5114\/reum.2017.71638","article-title":"The usefulness of histopathological examinations of non-renal biopsies in the diagnosis of granulomatosis with polyangiitis","volume":"55","author":"Masiak","year":"2017","journal-title":"Reumatologia"},{"key":"ref116","doi-asserted-by":"publisher","first-page":"S53","DOI":"10.1016\/s0385-8146(01)00072-4","article-title":"Histological findings in biopsies from patients with cytoplasmic-antineutrophil cytoplasmic antibody (cANCA) positive Wegener\u2019s granulomatosis","volume":"28","author":"Maguchi","year":"2001","journal-title":"Auris Nasus Larynx"},{"key":"ref117","doi-asserted-by":"publisher","first-page":"1","DOI":"10.1093\/rap\/rkac045","article-title":"Identifying relevant determinants of in-hospital time to diagnosis for ANCA-associated vasculitis patients","volume":"6","author":"Dirikgil","year":"2022","journal-title":"Rheumatol Adv Pract"},{"key":"ref118","doi-asserted-by":"publisher","first-page":"2738","DOI":"10.1183\/09031936.97.10122738","article-title":"Efficacy of transbronchial biopsy in pulmonary vasculitides","volume":"10","author":"Schnabel","year":"1997","journal-title":"Eur Respir J"},{"key":"ref119","doi-asserted-by":"publisher","first-page":"S1","DOI":"10.1016\/j.kint.2021.05.021","article-title":"KDIGO 2021 clinical practice guideline for the Management of Glomerular Diseases","volume":"100","author":"Rovin","year":"2021","journal-title":"Kidney Int"},{"key":"ref120","doi-asserted-by":"publisher","first-page":"953","DOI":"10.1093\/ndt\/16.5.953","article-title":"Renal histopathology and clinical course in 94 patients with Wegener\u2019s granulomatosis","volume":"16","author":"Aasarod","year":"2001","journal-title":"Nephrol Dial Transpl"},{"key":"ref121","doi-asserted-by":"publisher","first-page":"1628","DOI":"10.1681\/ASN.2010050477","article-title":"Histopathologic classification of ANCA-associated glomerulonephritis","volume":"21","author":"Berden","year":"2010","journal-title":"J Am Soc Nephrol"},{"key":"ref122","doi-asserted-by":"publisher","first-page":"1177","DOI":"10.1016\/j.kint.2018.07.020","article-title":"Development and validation of a renal risk score in ANCA-associated glomerulonephritis","volume":"94","author":"Brix","year":"2018","journal-title":"Kidney Int"},{"key":"ref123","doi-asserted-by":"publisher","first-page":"2146","DOI":"10.1016\/j.ekir.2023.07.021","article-title":"Repeat kidney biopsies in anti-neutrophil cytoplasmic autoantibody-associated vasculitis: clinical and histologic progression","volume":"8","author":"Aqell","year":"2023","journal-title":"Kidney Int Rep."},{"key":"ref124","doi-asserted-by":"publisher","first-page":"861","DOI":"10.1586\/14737175.2016.1165095","article-title":"Neurological manifestations in ANCA-associated vasculitis \u2013 assessment and treatment","volume":"16","author":"Wludarczyk","year":"2016","journal-title":"Expert Rev Neurother"},{"key":"ref125","doi-asserted-by":"publisher","first-page":"e615","DOI":"10.1212\/NXI.0000000000000615","article-title":"Peripheral neuropathy in antineutrophil cytoplasmic antibody-associated vasculitides. Insights from the DCVAS study","volume":"6","author":"Bischof","year":"2019","journal-title":"Neurol Neuroimmunol Neuroinflamm"},{"key":"ref126","doi-asserted-by":"publisher","first-page":"21","DOI":"10.1007\/s40120-021-00315-7","article-title":"ANCA-associated vasculitic neuropathies: a review","volume":"11","author":"Koike","year":"2022","journal-title":"Neurol Ther"},{"key":"ref127","doi-asserted-by":"publisher","first-page":"699","DOI":"10.1093\/rheumatology\/keaa233","article-title":"Muscle biopsy in anti-neutrophil cytoplasmic antibody-associated vasculitis: diagnostic yield depends on anti-neutrophil cytoplasmic antibody type, sex, and neutrophil count","volume":"60","author":"Lacou","year":"2021","journal-title":"Rheumatology"},{"key":"ref128","doi-asserted-by":"publisher","first-page":"1741","DOI":"10.1002\/art.41310","article-title":"The DCVAS investigators. Cutaneous manifestations of antineutrophil cytoplasmic antibody-associated vasculitis","volume":"72","author":"Micheletti","year":"2020","journal-title":"Arthritis Rheumatol"},{"key":"ref129","doi-asserted-by":"publisher","first-page":"181","DOI":"10.1007\/s12016-020-08788-4","article-title":"Cutaneous vasculitis: review on diagnosis and clinicopathologic correlations","volume":"61","author":"Frumholtz","year":"2021","journal-title":"Clin Rev Allergy Immunol"},{"key":"ref130","doi-asserted-by":"publisher","first-page":"1881","DOI":"10.3390\/diagnostics12081881","article-title":"Chest pain of atypical cause in a young man","volume":"12","author":"Fijolek","year":"2022","journal-title":"Diagnostics"},{"key":"ref131","doi-asserted-by":"publisher","first-page":"312","DOI":"10.1016\/j.carpath.2009.06.011","article-title":"Cardiac involvement in Wegener granulomatosis diagnosed at autopsy","volume":"19","author":"Mukhopadhyay","year":"2010","journal-title":"Cardiovasc Pathol"},{"key":"ref132","doi-asserted-by":"publisher","first-page":"623","DOI":"10.1093\/rheumatology\/keh548","article-title":"Impairment of lung function, health status and functional capacity in patients with ANCA-associated vasculitis","volume":"44","author":"Newall","year":"2005","journal-title":"Rheumatology (Oxford)"},{"key":"ref133","article-title":"Pulmonary function testing (PFT) in patients with antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitis (AAV): correlation with radiologic imaging (abstract)","volume":"74","author":"Dieterich","year":"2022","journal-title":"Arthritis Rheumatol"},{"key":"ref134","doi-asserted-by":"publisher","first-page":"2953","DOI":"10.1002\/art.39786","article-title":"Myeloperoxidase-antineutrophil cytoplasmic antibody (ANCA)-positive granulomatosis with polyangiitis (Wegener\u2019s) is a clinically distinct subset of ANCA-associated vasculitis. A retrospective analysis of 315 patients from a German Vasculitis referral center","volume":"68","author":"Schirmer","year":"2016","journal-title":"Arthritis Rheumatol"},{"key":"ref135","doi-asserted-by":"publisher","first-page":"3452","DOI":"10.1002\/art.34562","article-title":"Classification of antineutrophil cytoplasmic autoantibody vasculitides","volume":"64","author":"Lionaki","year":"2012","journal-title":"Arthritis Rheum"},{"key":"ref136","doi-asserted-by":"publisher","first-page":"1114","DOI":"10.1002\/art.40471","article-title":"Brief report: circulating cytokine profiles and antineutrophil cytoplasmic antibody specificity in patients with antineutrophil cytoplasmic antibody-associated vasculitis","volume":"70","author":"Berti","year":"2018","journal-title":"Arthritis Rheumatol"},{"key":"ref137","doi-asserted-by":"publisher","first-page":"701","DOI":"10.1016\/j.semarthrit.2018.05.003","article-title":"Myeloperoxidase-ANCA-positive granulomatosis with polyangiitis is a distinct subset of ANCA-associated vasculitis: a retrospective analysis of 455 patients from a single center in China","volume":"48","author":"Chang","year":"2018","journal-title":"Sem Arthritis Rheum."},{"key":"ref138","doi-asserted-by":"publisher","first-page":"1707","DOI":"10.1093\/rheumatology\/kez148","article-title":"Subclassifying ANCA-associated vasculitis: a unifying view of disease spectrum","volume":"58","author":"Mahr","year":"2019","journal-title":"Rheumatology (Oxford)"},{"key":"ref139","doi-asserted-by":"publisher","first-page":"2676","DOI":"10.1016\/j.ekir.2022.09.011","article-title":"Relevance of combined clinicopathologic phenotype and antineutrophil cytoplasmic autoantibody serotype in the diagnosis of antineutrophil cytoplasmic autoantibody vasculitis","volume":"7","author":"Alba","year":"2022","journal-title":"Kidney Int Rep"},{"key":"ref140","doi-asserted-by":"publisher","first-page":"693","DOI":"10.1016\/j.kint.2017.03.014","article-title":"Patients double-seropositive for ANCA and anti-GBM antibodies have varied renal survival, frequency of relapse, and outcomes compared to single-seropositive patients","volume":"92","author":"McAdoo","year":"2017","journal-title":"Kidney Int"},{"key":"ref141","doi-asserted-by":"crossref","first-page":"S62","DOI":"10.55563\/clinexprheumatol\/d01o72","article-title":"Subphenotypes of ANCA-associated vasculitis identified by latent class analysis","volume":"39","author":"Wojcik","year":"2021","journal-title":"Clin Exp Rheumatol"},{"key":"ref142","doi-asserted-by":"publisher","first-page":"545","DOI":"10.1093\/rheumatology\/kez327","article-title":"Significant association between clinical characteristics and immuno-phenotypes in patients with ANCA-associated vasculitis","volume":"59","author":"Matsumoto","year":"2020","journal-title":"Rheumatology (Oxford)"},{"key":"ref143","doi-asserted-by":"publisher","first-page":"1583","DOI":"10.1136\/annrheumdis-2016-209133","article-title":"EULAR\/ERA-EDTA recommendations for the management of ANCA-associated vasculitis","volume":"75","author":"Yates","year":"2016","journal-title":"Ann Rheum Dis"},{"key":"ref144","doi-asserted-by":"publisher","first-page":"450","DOI":"10.1016\/S0197-2456(02)00209-X","article-title":"Design of the Wegener s granulomatosis Etanercept trial WGET","volume":"23","year":"2020","journal-title":"Control Clin Trials"},{"key":"ref145","doi-asserted-by":"publisher","first-page":"310","DOI":"10.1136\/ard.2008.088096","article-title":"EULAR recommendations for the management of primary small and medium vessel vasculitis","volume":"68","author":"Mukhtyar","year":"2009","journal-title":"Ann Rheum Dis"},{"key":"ref146","doi-asserted-by":"publisher","first-page":"17","DOI":"10.1097\/00005792-199601000-00003","article-title":"Prognostic factors in polyarteritis nodosa and Churg-Strauss syndrome. A prospective study in 342 patients","volume":"75","author":"Guillevin","year":"1996","journal-title":"Medicine (Baltimore)"},{"key":"ref147","doi-asserted-by":"publisher","first-page":"283","DOI":"10.1093\/ndt\/gfy300","article-title":"Urinary soluble CD163 and monocyte chemoattractant protein-1 in the identification of subtle renal flare in anti-neutrophil cytoplasmic antibody-associated vasculitis","volume":"35","author":"Moran","year":"2020","journal-title":"Nephrol Dial Transplant"},{"key":"ref148","doi-asserted-by":"publisher","first-page":"1","DOI":"10.1136\/annrheumdis-2022-222479","article-title":"Association of baseline soluble immune checkpoints with the risk of relapse in PR3-ANCA vasculitis following induction of remission","author":"Gamerith","year":"2022","journal-title":"Ann Rheum Dis"},{"key":"ref149","doi-asserted-by":"publisher","first-page":"736","DOI":"10.1002\/art.42388","article-title":"Autoreactive plasmablasts after B cell depletion with rituximab and relapses in antineutrophil cytoplasmic antibody-associated vasculitis","volume":"75","author":"Berti","year":"2023","journal-title":"Arthritis Rheumatol"},{"key":"ref150","doi-asserted-by":"publisher","first-page":"945","DOI":"10.33549\/physiolres.933765","article-title":"Serum hepcidin is increased in ANCA-associated vasculitis and correlates with activity markers","volume":"67","author":"Prikryl","year":"2018","journal-title":"Physiol Res"},{"key":"ref151","doi-asserted-by":"publisher","first-page":"586","DOI":"10.34067\/KID.0006432020","article-title":"Biomarkers in ANCA-associated vasculitis: potential pitfalls and future prospects","volume":"2","author":"Morris","year":"2021","journal-title":"Kidney"},{"key":"ref152","doi-asserted-by":"publisher","first-page":"596503","DOI":"10.1155\/2014\/596503","article-title":"Biomarkers predict relapse in granulomatosis with polyangiitis","volume":"2014","author":"Hogan","year":"2014","journal-title":"J Biomark"},{"key":"ref153","doi-asserted-by":"publisher","first-page":"e15051","DOI":"10.7717\/peerj.15051","article-title":"Metabolomics combined with clinical analysis explores metabolic changes and potential serum metabolite biomarkers of antineutrophil cytoplasmic antibody-associated vasculitis with renal impairment","volume":"11","author":"Liu","year":"2023","journal-title":"Peer J"},{"key":"ref154","doi-asserted-by":"publisher","first-page":"e002935","DOI":"10.1136\/rmdopen-2022-002935","article-title":"Risk of relapse of ANCA-associated vasculitis among patients homozygous for the proteinase 3 gene Val119Ile polymorphism","volume":"9","author":"Moura","year":"2023","journal-title":"RMD Open"},{"key":"ref155","first-page":"72076","article-title":"There is no benefit in routinely monitoring ANCA titers in patients with granulomatosis with polyangiitis","volume":"33","author":"Verstockt","year":"2015","journal-title":"Clin Exp Rheumatol"},{"key":"ref156","doi-asserted-by":"publisher","first-page":"129","DOI":"10.1186\/s13075-017-1321-1","article-title":"Negative anti-neutrophil cytoplasm antibody at switch to maintenance therapy is associated with a reduced risk of relapse","volume":"19","author":"Morgan","year":"2017","journal-title":"Arthritis Res Ther"},{"key":"ref157","doi-asserted-by":"publisher","first-page":"3297","DOI":"10.1007\/s10067-019-04693-0","article-title":"Antineutrophil cytoplasmic antibodies and their relationship with disease activity and presence of staphylococcal superantigens in nasal swabs in patients having granulomatosis with polyangiitis: results of a study involving 115 patients from a single center","volume":"38","author":"Fijolek","year":"2019","journal-title":"Clin Rheumatol"},{"key":"ref158","doi-asserted-by":"publisher","first-page":"537","DOI":"10.1681\/ASN.2013111233","article-title":"ANCA as a predictor of relapse: useful in patients with renal involvement but not in patients with nonrenal disease","volume":"26","author":"Kemna","year":"2015","journal-title":"J Am Soc Nephrol"},{"key":"ref159","doi-asserted-by":"publisher","first-page":"1408","DOI":"10.1093\/ndt\/gfaa066","article-title":"PR3-ANCAs predict relapses in ANCA-associated vasculitis patients after rituximab","volume":"36","author":"Van Dam","year":"2021","journal-title":"Nephrol Dial Transplant"},{"key":"ref160","doi-asserted-by":"publisher","first-page":"417","DOI":"10.1056\/NEJMoa1213277","article-title":"Efficacy of remission-induction regimens for ANCA-associated vasculitis","volume":"369","author":"Specks","year":"2013","journal-title":"N Engl J Med"},{"key":"ref161","doi-asserted-by":"publisher","first-page":"643.e9","DOI":"10.1016\/j.amjmed.2006.08.016","article-title":"ANCA are detectable in nearly all patients with active severe Wegener\u2019s granulomatosis","volume":"120","author":"Finkielman","year":"2007","journal-title":"Am J Med"},{"key":"ref162","doi-asserted-by":"publisher","first-page":"2457","DOI":"10.1007\/s10067-022-06159-2","article-title":"Predicting factors for relapse in patients with granulomatosis with polyangiitis: results from a long-term cohort","volume":"41","author":"Nejad","year":"2022","journal-title":"Clin Rheumatol"},{"key":"ref163","doi-asserted-by":"publisher","first-page":"1827","DOI":"10.1136\/ard.2008.101279","article-title":"Modification and validation of the Birmingham Vasculitis activity score (version 3)","volume":"68","author":"Mukhtyar","year":"2009","journal-title":"Ann Rheum Dis"},{"key":"ref164","doi-asserted-by":"publisher","first-page":"1001","DOI":"10.1002\/acr.23912","article-title":"Comparison of the Birmingham Vasculitis activity score and the five factor score to assess survival in antineutrophil cytoplasmic antibody-associated vasculitis: a study of 550 patients from Spain (REVAS registry)","volume":"72","author":"Solans-Laque","year":"2019","journal-title":"Arthritis Care Res"},{"key":"ref165","doi-asserted-by":"publisher","first-page":"e0270142","DOI":"10.1371\/journal.pone.0270142","article-title":"Increased risk of venous thromboembolism in patients with granulomatosis with polyangiitis: a population-based study","volume":"17","author":"Marozoff","year":"2022","journal-title":"PLoS One"},{"key":"ref166","doi-asserted-by":"publisher","first-page":"16478","DOI":"10.20452\/pamw.16478","article-title":"Influence of smoking on disease activity and prognosis of antineutrophil cytoplasmic antibody-associated vasculitis: a retrospective cohort study","volume":"133","author":"Patel","year":"2023","journal-title":"Pol Arch Intern Med"},{"key":"ref167","first-page":"31","article-title":"Development and validation of a disease extent index for Wegener\u2019s granulomatosis","volume":"55","author":"De Groot","year":"2001","journal-title":"Clin Nephrol"},{"key":"ref168","doi-asserted-by":"publisher","first-page":"371","DOI":"10.1002\/art.1780400222","article-title":"Development and initial validation of the vasculitis damage index for the standardized clinical assessment of damage in the systemic vasculitides","volume":"40","author":"Exley","year":"1997","journal-title":"Arthritis Rheum"},{"key":"ref169","doi-asserted-by":"publisher","first-page":"e28305","DOI":"10.1097\/MD.0000000000028305","article-title":"Mortality predictors in ANCA-associated vasculitis","volume":"100","author":"Dagostin","year":"2021","journal-title":"Medicine (Baltimore)"},{"key":"ref170","doi-asserted-by":"publisher","first-page":"1158","DOI":"10.1002\/acr.24958","article-title":"Remission and low disease activity in granulomatosis with polyangiitis and microscopic polyangiitis: prevalence and impact on damage accrual","volume":"75","author":"Delvino","year":"2023","journal-title":"Arthritis Care Res"},{"key":"ref171","doi-asserted-by":"publisher","first-page":"1480","DOI":"10.3899\/jrheum.110276","article-title":"The OMERACT core set of outcome measures for use in clinical trials of ANCA-associated vasculitis","volume":"38","author":"Merkel","year":"2011","journal-title":"J Rheumatol"},{"key":"ref172","doi-asserted-by":"publisher","first-page":"152021","DOI":"10.1016\/j.semarthrit.2022.152021","article-title":"An international Delphi exercise to identify items of importance to measuring response to treatment in ANCA-associated vasculitis","volume":"55","author":"Quinn","year":"2022","journal-title":"Semin Arthritis Rheum"},{"key":"ref173","doi-asserted-by":"publisher","first-page":"1717","DOI":"10.1007\/s00296-021-04966-5","article-title":"Classificationa of ANCA- associated vasculitis: differences based on ANCA specificity and clinicopathologic phenotype","volume":"41","author":"Dominguez-Quintana","year":"2021","journal-title":"Rheumatol Int"}],"container-title":["Frontiers in Medicine"],"original-title":[],"link":[{"URL":"https:\/\/www.frontiersin.org\/articles\/10.3389\/fmed.2024.1369233\/full","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2024,8,27]],"date-time":"2024-08-27T15:39:39Z","timestamp":1724773179000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.frontiersin.org\/articles\/10.3389\/fmed.2024.1369233\/full"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2024,8,27]]},"references-count":173,"alternative-id":["10.3389\/fmed.2024.1369233"],"URL":"https:\/\/doi.org\/10.3389\/fmed.2024.1369233","relation":{},"ISSN":["2296-858X"],"issn-type":[{"value":"2296-858X","type":"electronic"}],"subject":[],"published":{"date-parts":[[2024,8,27]]},"article-number":"1369233"}}