{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,14]],"date-time":"2026-05-14T11:18:22Z","timestamp":1778757502516,"version":"3.51.4"},"reference-count":80,"publisher":"Oxford University Press (OUP)","license":[{"start":{"date-parts":[[2026,3,31]],"date-time":"2026-03-31T00:00:00Z","timestamp":1774915200000},"content-version":"am","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"name":"European Renal Association"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:p>Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV) are systemic small-vessel necrotizing vasculitides, including microscopic polyangiitis (MPA), granulomatosis with polyangiitis (GPA), and eosinophilic granulomatosis with polyangiitis (EGPA). Genetic factors, particularly human leukocyte antigen variants, contribute to disease susceptibility and define distinct myeloperoxidase- and proteinase 3-associated phenotypes. This review summarizes current understanding of organ-specific manifestations, therapeutic advances, and prognostic determinants of AAV, with particular relevance for nephrologists. Kidney involvement is common, particularly in MPA, whereas GPA and EGPA more frequently present with extrarenal manifestations. Pulmonary involvement is also frequent, ranging from alveolar haemorrhage and interstitial lung disease (ILD) in MPA to granulomatous nodules and airway disease in GPA, while asthma dominates in EGPA. Other frequent manifestations include ear, nose, and throat (ENT), peripheral nervous system, cutaneous, and constitutional manifestations, with ENT activity and non-haemorrhagic respiratory manifestations correlating with relapse risk. Current treatment emphasizes rituximab or cyclophosphamide with reduced-dose glucocorticoids for induction, avacopan for glucocorticoid-sparing strategies, and rituximab for maintenance. Avacopan has shown benefits beyond renal outcomes. Furthermore, anti-interleukin-5 therapies have recently been approved for the treatment of EGPA. Contrarily, ILD remains a therapeutic challenge, with limited evidence for antifibrotic agents. AAV patients are at high risk of infections, thromboembolism, and malignancy, influenced by both disease activity and treatment exposure. Mortality remains high, driven mainly by infections, with kidney and lung involvement serving as major prognostic determinants. For nephrologists, early recognition of extrarenal manifestations, vigilance for relapse beyond the kidney, and multidisciplinary collaboration are crucial for optimizing long-term outcomes.<\/jats:p>","DOI":"10.1093\/ndt\/gfag077","type":"journal-article","created":{"date-parts":[[2026,3,31]],"date-time":"2026-03-31T11:31:30Z","timestamp":1774956690000},"source":"Crossref","is-referenced-by-count":0,"title":["Extrarenal disease in ANCA-associated vasculitis: challenges and evolving therapeutic strategies on behalf of the Immunonephrology Working Group (IWG) of the ERA"],"prefix":"10.1093","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-5963-3246","authenticated-orcid":false,"given":"Aglaia","family":"Chalkia","sequence":"first","affiliation":[{"name":"General Hospital of Athens Hippokration Nephrology Department, , Athens ,","place":["Greece"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Eleni","family":"Frangou","sequence":"additional","affiliation":[{"name":"Limassol General Hospital Department of Nephrology, , State Health Service Organization, Limassol ,","place":["Cyprus"]},{"name":"University of Nicosia Medical School , Nicosia ,","place":["Cyprus"]},{"name":"Medical School, National and Kapodistrian University of Athens , Athens ,","place":["Greece"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Annette","family":"Bruchfeld","sequence":"additional","affiliation":[{"name":"Medicine and Caring Sciences, Link\u00f6ping University Department of Health, , Link\u00f6ping ,","place":["Sweden"]},{"name":"Karolinska University Hospital and CLINTEC Karolinska Institutet Department of Renal Medicine, , Stockholm ,","place":["Sweden"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5830-9663","authenticated-orcid":false,"given":"Fernando","family":"Caravaca-Fontan","sequence":"additional","affiliation":[{"name":"Hospital Universitario 12 de Octubre Nephrology Department, , Madrid ,","place":["Spain"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0362-8154","authenticated-orcid":false,"given":"Safak","family":"Mirioglu","sequence":"additional","affiliation":[{"name":"Istanbul Faculty of Medicine, Istanbul University Division of Nephrology, Department of Internal Medicine, , Istanbul ,","place":["Turkey"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Sarah","family":"Moran","sequence":"additional","affiliation":[{"name":"Cork University Hospital, University College Cork , Cork \u00a0","place":["Republic of Ireland"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7582-8476","authenticated-orcid":false,"given":"Luis\u00a0F","family":"Quintana","sequence":"additional","affiliation":[{"name":"Hospital Cl\u00ednic de Barcelona, University of Barcelona Complex Glomerular Disease Unit (CSUR), Department of Nephrology and Renal Transplantation, , Barcelona, IDIBAPS ,","place":["Spain"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5990-494X","authenticated-orcid":false,"given":"Stefanie","family":"Steiger","sequence":"additional","affiliation":[{"name":"Hospital of the Ludwig-Maximilians-University Division of Nephrology, Department of Internal Medicine IV, , Munich ,","place":["Germany"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2291-1692","authenticated-orcid":false,"given":"Kate I","family":"Stevens","sequence":"additional","affiliation":[{"name":"The Glasgow Renal and Transplant Unit, Queen Elizabeth University Hospital , Glasgow ,","place":["UK"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9920-2195","authenticated-orcid":false,"given":"Onno Y K","family":"Teng","sequence":"additional","affiliation":[{"name":"Leiden University Medical Center Center of Expertise for Lupus-, Vasculitis- and Complement-Mediated Systemic Diseases, Department of Internal Medicine\u2014Nephrology Section, , Leiden ,","place":["The Netherlands"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"David R W","family":"Jayne","sequence":"additional","affiliation":[{"name":"University of Cambridge Department of Medicine, , Cambridge ,","place":["UK"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Andreas","family":"Kronbichler","sequence":"additional","affiliation":[{"name":"Nephrology and Hypertension, Medical University Innsbruck Department of Internal Medicine IV, , Innsbruck ,","place":["Austria"]}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"286","published-online":{"date-parts":[[2026,3,31]]},"reference":[{"key":"2026051406284689400_bib1","doi-asserted-by":"publisher","first-page":"102495","DOI":"10.1016\/j.autrev.2020.102495","article-title":"Clinical associations of renal involvement in ANCA-associated vasculitis","volume":"19","author":"Kronbichler","year":"2020","journal-title":"Autoimmun Rev"},{"key":"2026051406284689400_bib2","doi-asserted-by":"publisher","first-page":"683","DOI":"10.1016\/S0140-6736(23)01736-1","article-title":"Diagnosis and management of ANCA-associated vasculitis","volume":"403","author":"Kronbichler","year":"2024;","journal-title":"Lancet"},{"key":"2026051406284689400_bib3","doi-asserted-by":"publisher","first-page":"1655","DOI":"10.1093\/ndt\/gfac320","article-title":"Long-term outcomes and prognostic factors for survival of patients with ANCA-associated vasculitis","volume":"38","author":"S\u00e1nchez\u00a0\u00c1lamo","year":"2023","journal-title":"Nephrol Dial Transplant"},{"key":"2026051406284689400_bib4","doi-asserted-by":"publisher","first-page":"1458","DOI":"10.3899\/jrheum.161224","article-title":"Pulmonary involvement in antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitis: the influence of ANCA subtype","volume":"44","author":"Mohammad","year":"2017","journal-title":"J Rheumatol"},{"key":"2026051406284689400_bib5","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":"2026051406284689400_bib6","doi-asserted-by":"publisher","first-page":"315","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":"81","author":"Robson","year":"2022","journal-title":"Ann Rheum Dis"},{"key":"2026051406284689400_bib7","doi-asserted-by":"publisher","first-page":"1141","DOI":"10.1164\/rccm.202308-1426OC","article-title":"Alveolar hemorrhage in antineutrophil cytoplasmic antibody-associated vasculitis: results of an international randomized controlled trial (PEXIVAS)","volume":"209","author":"Fussner","year":"2024","journal-title":"Am J Respir Crit Care Med"},{"key":"2026051406284689400_bib8","doi-asserted-by":"publisher","first-page":"e46813","DOI":"10.7759\/cureus.46813","article-title":"Unilateral diffuse alveolar hemorrhage as an initial presentation of microscopic polyangiitis","volume":"15","author":"Behera","year":"2023","journal-title":"Cureus"},{"key":"2026051406284689400_bib9","doi-asserted-by":"publisher","first-page":"400","DOI":"10.1002\/art.41983","article-title":"2022 American College of Rheumatology\/European Alliance of Associations for Rheumatology classification criteria for microscopic polyangiitis","volume":"74","author":"Suppiah","year":"2022","journal-title":"Arthritis Rheumatol"},{"key":"2026051406284689400_bib10","doi-asserted-by":"publisher","first-page":"i42","DOI":"10.1093\/rheumatology\/keae557","article-title":"Interstitial lung disease, antineutrophil cystoplasmic antibodies and microscopic polyangiitis","volume":"64","author":"Specks","year":"2025","journal-title":"Rheumatology (Oxford)"},{"key":"2026051406284689400_bib11","doi-asserted-by":"publisher","first-page":"599","DOI":"10.1056\/NEJMoa2023386","article-title":"Avacopan for the treatment of ANCA-associated vasculitis","volume":"384","author":"Jayne","year":"2021","journal-title":"N Engl J Med"},{"key":"2026051406284689400_bib12","doi-asserted-by":"publisher","first-page":"e11795","DOI":"10.1002\/acr2.11795","article-title":"Treatment with avacopan in patients with respiratory tract manifestations of antineutrophil cytoplasmic antibody-associated vasculitis","volume":"7","author":"Specks","year":"2025","journal-title":"ACR Open Rheumatol"},{"key":"2026051406284689400_bib13","doi-asserted-by":"publisher","first-page":"270","DOI":"10.1002\/art.37721","article-title":"Eosinophilic granulomatosis with polyangiitis (Churg-Strauss): clinical characteristics and long-term followup of the 383 patients enrolled in the French Vasculitis Study Group cohort","volume":"65","author":"Comarmond","year":"2013","journal-title":"Arthritis Rheum"},{"key":"2026051406284689400_bib14","doi-asserted-by":"publisher","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":"2026051406284689400_bib15","doi-asserted-by":"publisher","first-page":"41","DOI":"10.20517\/2574-1209.2021.41","article-title":"Ear, nose, and throat in ANCA-associated vasculitis: a comprehensive review","volume":"5","author":"Padoan","year":"2021","journal-title":"Vessel Plus"},{"key":"2026051406284689400_bib16","doi-asserted-by":"publisher","first-page":"152751","DOI":"10.1016\/j.semarthrit.2025.152751","article-title":"Nervous system involvement in ANCA-associated vasculitis: single center experience from Latin America","volume":"73","author":"Quintero-Giraldo","year":"2025","journal-title":"Semin Arthritis Rheum"},{"key":"2026051406284689400_bib17","doi-asserted-by":"publisher","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":"2026051406284689400_bib18","doi-asserted-by":"publisher","first-page":"573","DOI":"10.1016\/j.rdc.2017.06.006","article-title":"Central nervous system disease in antineutrophil cytoplasmic antibodies-associated vasculitis","volume":"43","author":"Graf","year":"2017","journal-title":"Rheum Dis Clin North Am"},{"key":"2026051406284689400_bib19","doi-asserted-by":"publisher","first-page":"1741","DOI":"10.1002\/art.41310","article-title":"Cutaneous manifestations of antineutrophil cytoplasmic antibody-associated vasculitis","volume":"72","author":"Micheletti","year":"2020","journal-title":"Arthritis Rheumatol"},{"key":"2026051406284689400_bib20","doi-asserted-by":"publisher","first-page":"2517","DOI":"10.1093\/rheumatology\/keac663","article-title":"Ocular manifestations of ANCA-associated vasculitis","volume":"62","author":"Junek","year":"2023","journal-title":"Rheumatology (United Kingdom)"},{"key":"2026051406284689400_bib21","doi-asserted-by":"publisher","first-page":"627","DOI":"10.1002\/art.27263","article-title":"Cardiac involvement in Churg-Strauss syndrome","volume":"62","author":"Dennert","year":"2010","journal-title":"Arthritis Rheum"},{"key":"2026051406284689400_bib22","doi-asserted-by":"publisher","first-page":"2256","DOI":"10.1093\/ndt\/gfaf070","article-title":"Cardiovascular involvement in ANCA-associated vasculitis","volume":"40","author":"Sarmad","year":"2025","journal-title":"Nephrol Dial Transplant"},{"key":"2026051406284689400_bib23","doi-asserted-by":"publisher","first-page":"559","DOI":"10.1038\/s41584-022-00819-y","article-title":"Genetics of ANCA-associated vasculitis: role in pathogenesis, classification and management","volume":"18","author":"Trivioli","year":"2022","journal-title":"Nat Rev Rheumatol"},{"key":"2026051406284689400_bib24","doi-asserted-by":"publisher","first-page":"468","DOI":"10.1007\/s00439-004-1092-z","article-title":"New genomic region for Wegener\u2019s granulomatosis as revealed by an extended association screen with 202 apoptosis-related genes","volume":"114","author":"Jagiello","year":"2004","journal-title":"Hum Genet"},{"key":"2026051406284689400_bib25","doi-asserted-by":"publisher","first-page":"3760","DOI":"10.1002\/art.27742","article-title":"Alpha1 -antitrypsin deficiency-related alleles Z and S and the risk of Wegener\u2019s granulomatosis","volume":"62","author":"Mahr","year":"2010","journal-title":"Arthritis Rheum"},{"key":"2026051406284689400_bib26","doi-asserted-by":"publisher","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":"2026051406284689400_bib27","doi-asserted-by":"publisher","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":"2026051406284689400_bib28","doi-asserted-by":"publisher","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":"2026051406284689400_bib29","doi-asserted-by":"publisher","first-page":"844","DOI":"10.1038\/ki.1995.360","article-title":"The PiZ gene of \u03b11-antitrypsin as a determinant of outcome in PR3-ANCA-positive vasculitis","volume":"48","author":"Segelmark","year":"1995","journal-title":"Kidney Int"},{"key":"2026051406284689400_bib30","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":"2026051406284689400_bib31","doi-asserted-by":"publisher","first-page":"1144","DOI":"10.1136\/annrheumdis-2018-214263","article-title":"Association of MUC5B promoter polymorphism with interstitial lung disease in myeloperoxidase-antineutrophil cytoplasmic antibody-associated vasculitis","volume":"78","author":"Namba","year":"2019","journal-title":"Ann Rheum Dis"},{"key":"2026051406284689400_bib32","doi-asserted-by":"publisher","first-page":"1293","DOI":"10.2215\/CJN.13071211","article-title":"Association of HLA genes with clinical outcomes of ANCA-associated vasculitis","volume":"7","author":"Chang","year":"2012","journal-title":"Clin J Am Soc Nephrol"},{"key":"2026051406284689400_bib33","doi-asserted-by":"publisher","first-page":"1684","DOI":"10.1016\/j.jaci.2016.08.051","article-title":"Association of a TNFSF13B (BAFF) regulatory region single nucleotide polymorphism with response to rituximab in antineutrophil cytoplasmic antibody-associated vasculitis","volume":"139","author":"Alberici","year":"2017","journal-title":"J Allergy Clin Immunol"},{"key":"2026051406284689400_bib34","doi-asserted-by":"publisher","first-page":"1517","DOI":"10.1681\/ASN.2021081142","article-title":"Immunological interaction of HLA-DPB1 and proteinase 3 in ANCA vasculitis is associated with clinical disease activity","volume":"33","author":"Chen","year":"2022","journal-title":"J Am Soc Nephrol"},{"key":"2026051406284689400_bib35","doi-asserted-by":"publisher","first-page":"e762","DOI":"10.1016\/S2665-9913(24)00187-5","article-title":"Data-driven subclassification of ANCA-associated vasculitis: model-based clustering of a federated international cohort","volume":"6","author":"Gisslander","year":"2024","journal-title":"Lancet Rheumatol"},{"key":"2026051406284689400_bib36","doi-asserted-by":"publisher","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":"2026051406284689400_bib37","doi-asserted-by":"publisher","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":"2026051406284689400_bib38","doi-asserted-by":"publisher","first-page":"1243","DOI":"10.1136\/annrheumdis-2019-216863","article-title":"Rituximab as therapy to induce remission after relapse in ANCA-associated vasculitis","volume":"79","author":"Smith","year":"2020","journal-title":"Ann Rheum Dis"},{"key":"2026051406284689400_bib39","doi-asserted-by":"publisher","first-page":"2461","DOI":"10.1002\/art.21142","article-title":"Randomized trial of cyclophosphamide versus methotrexate for induction of remission in early systemic antineutrophil cytoplasmic antibody-associated vasculitis","volume":"52","author":"De\u00a0Groot","year":"2005","journal-title":"Arthritis Rheum"},{"key":"2026051406284689400_bib40","doi-asserted-by":"publisher","first-page":"399","DOI":"10.1136\/annrheumdis-2018-214245","article-title":"Mycophenolate mofetil versus cyclophosphamide for remission induction in ANCA-associated vasculitis: a randomised, non-inferiority trial","volume":"78","author":"Jones","year":"2019","journal-title":"Ann Rheum Dis"},{"key":"2026051406284689400_bib41","doi-asserted-by":"publisher","first-page":"622","DOI":"10.1056\/NEJMoa1803537","article-title":"Plasma exchange and glucocorticoids in severe ANCA-associated vasculitis","volume":"382","author":"Walsh","year":"2020","journal-title":"N Engl J Med"},{"key":"2026051406284689400_bib42","doi-asserted-by":"publisher","first-page":"2178","DOI":"10.1001\/jama.2021.6615","article-title":"Effect of reduced-dose vs high-dose glucocorticoids added to rituximab on remission induction in ANCA-associated vasculitis A randomized clinical trial","volume":"325","author":"Furuta","year":"2021","journal-title":"JAMA"},{"key":"2026051406284689400_bib43","doi-asserted-by":"publisher","DOI":"10.1136\/ard-2022-223764","article-title":"EULAR recommendations for the management of ANCA-associated vasculitis: 2022 update","author":"Hellmich","year":"2023","journal-title":"Ann Rheum Dis"},{"key":"2026051406284689400_bib44","doi-asserted-by":"publisher","first-page":"S71","DOI":"10.1016\/j.kint.2023.10.008","article-title":"KDIGO 2024 clinical practice guideline for the management of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis","volume":"105","author":"Floege","year":"2024","journal-title":"Kidney Int"},{"key":"2026051406284689400_bib45","doi-asserted-by":"publisher","first-page":"944","DOI":"10.1093\/ndt\/gfad237","article-title":"ANCA-associated vasculitis-treatment standard","volume":"39","author":"Chalkia","year":"2024","journal-title":"Nephrol Dial Transplant"},{"key":"2026051406284689400_bib46","doi-asserted-by":"publisher","first-page":"1783","DOI":"10.1016\/j.ekir.2024.03.022","article-title":"Real-world experience with avacopan in antineutrophil cytoplasmic autoantibody-associated vasculitis","author":"Zonozi","year":"2024","journal-title":"Kidney Int Rep"},{"key":"2026051406284689400_bib47","doi-asserted-by":"publisher","first-page":"1473","DOI":"10.1093\/ndt\/gfae020","article-title":"Avacopan for ANCA-associated vasculitis with hypoxic pulmonary haemorrhage","volume":"39","author":"Chalkia","year":"2024","journal-title":"Nephrol Dial Transplant"},{"key":"2026051406284689400_bib48","doi-asserted-by":"publisher","first-page":"3747","DOI":"10.1016\/j.ekir.2025.07.015","article-title":"Novel treatment paradigms: complement inhibition in antineutrophil cytoplasmic autoantibody vasculitis","volume":"10","author":"Wu","year":"2025","journal-title":"Kidney Int Rep"},{"key":"2026051406284689400_bib49","doi-asserted-by":"publisher","DOI":"10.1002\/acr2.11795","article-title":"Treatment with avacopan in patients with respiratory tract manifestations of antineutrophil cytoplasmic antibody-associated vasculitis","volume":"7","author":"Specks","year":"2025","journal-title":"ACR Open Rheumatol"},{"key":"2026051406284689400_bib50","doi-asserted-by":"publisher","first-page":"1771","DOI":"10.1056\/NEJMoa1404231","article-title":"Rituximab versus azathioprine for maintenance in ANCA-associated vasculitis","volume":"371","author":"Guillevin","year":"2014","journal-title":"N Engl J Med"},{"key":"2026051406284689400_bib51","doi-asserted-by":"publisher","first-page":"937","DOI":"10.1136\/ard-2022-223559","article-title":"Rituximab versus azathioprine for maintenance of remission for patients with ANCA-associated vasculitis and relapsing disease: an international randomised controlled trial","volume":"82","author":"Smith","year":"2023","journal-title":"Ann Rheum Dis"},{"key":"2026051406284689400_bib52","doi-asserted-by":"publisher","first-page":"1249","DOI":"10.7326\/ANNALS-24-03947","article-title":"Rituximab versus conventional therapy for remission induction in eosinophilic granulomatosis with polyangiitis","volume":"178","author":"Terrier","year":"2025","journal-title":"Ann Intern Med"},{"key":"2026051406284689400_bib53","doi-asserted-by":"publisher","first-page":"952","DOI":"10.1136\/annrheumdis-2017-211897","article-title":"Scheduled rituximab maintenance reduces relapse rate in eosinophilic granulomatosis with polyangiitis","volume":"77","author":"Emmi","year":"2018","journal-title":"Ann Rheum Dis"},{"key":"2026051406284689400_bib54","doi-asserted-by":"publisher","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":"2026051406284689400_bib55","doi-asserted-by":"publisher","first-page":"911","DOI":"10.1056\/NEJMoa2311155","article-title":"Benralizumab versus mepolizumab for eosinophilic granulomatosis with polyangiitis","volume":"390","author":"Wechsler","year":"2024","journal-title":"N Engl J Med"},{"key":"2026051406284689400_bib56","doi-asserted-by":"publisher","DOI":"10.1177\/1479973121994562","article-title":"Impact of interstitial lung disease on mortality in ANCA-associated vasculitis: a systematic literature review and meta-analysis","author":"Zhou","year":"2021","journal-title":"Chron Respir Dis"},{"key":"2026051406284689400_bib57","doi-asserted-by":"publisher","DOI":"10.1002\/art.70078","article-title":"Interstitial lung disease in ANCA-associated vasculitis: a European multicentre study","author":"Chalkia","year":"2026","journal-title":"Arthritis Rheumatol"},{"key":"2026051406284689400_bib58","doi-asserted-by":"publisher","first-page":"436","DOI":"10.1097\/MCP.0000000000000979","article-title":"Interstitial lung disease in patients with anti-neutrophil cytoplasm antibody-associated vasculitis: an update on pathogenesis and treatment","volume":"29","author":"Turgeon","year":"2023","journal-title":"Curr Opin Pulm Med"},{"key":"2026051406284689400_bib59","doi-asserted-by":"publisher","first-page":"2518","DOI":"10.1056\/NEJMoa1903076","article-title":"Nintedanib for systemic sclerosis-associated interstitial lung disease","volume":"380","author":"Distler","year":"2019","journal-title":"N Engl J Med"},{"key":"2026051406284689400_bib60","doi-asserted-by":"publisher","DOI":"10.3390\/ph17091147","article-title":"Safety of nintedanib in a patient with chronic pulmonary fibrosis and kidney disease","volume":"17","author":"Maggisano","year":"2024","journal-title":"Pharmaceuticals"},{"key":"2026051406284689400_bib61","doi-asserted-by":"publisher","first-page":"106553","DOI":"10.1016\/j.ejim.2025.106553","article-title":"ANCA-associated vasculitis and interstitial lung disease: challenges and open questions","author":"Prieto-Gonz\u00e1lez","year":"2025","journal-title":"Eur J Intern Med"},{"key":"2026051406284689400_bib62","doi-asserted-by":"publisher","first-page":"117","DOI":"10.1177\/01455613211036246","article-title":"Current therapeutic approaches to subglottic stenosis in patients with GPA: a systematic review","volume":"103","author":"Almuhanna","year":"2024","journal-title":"Ear Nose Throat J"},{"key":"2026051406284689400_bib63","doi-asserted-by":"publisher","first-page":"103018","DOI":"10.1016\/j.autrev.2021.103018","article-title":"Current clinical and therapeutic approach to tumour-like mass lesions in granulomatosis with polyangiitis","volume":"21","author":"Padoan","year":"2022","journal-title":"Autoimmun Rev"},{"key":"2026051406284689400_bib64","doi-asserted-by":"publisher","first-page":"681","DOI":"10.1136\/ard-2022-223401","article-title":"Risk factors for serious infections in ANCA-associated vasculitis","volume":"82","author":"Odler","year":"2023","journal-title":"Ann Rheum Dis"},{"key":"2026051406284689400_bib65","doi-asserted-by":"publisher","first-page":"1440","DOI":"10.1136\/annrheumdis-2017-212861","article-title":"Trimethoprim\u2013sulfamethoxazole prophylaxis prevents severe\/life-threatening infections following rituximab in antineutrophil cytoplasm antibody-associated vasculitis","volume":"77","author":"Kronbichler","year":"2018","journal-title":"Ann Rheum Dis"},{"key":"2026051406284689400_bib66","doi-asserted-by":"publisher","first-page":"1313","DOI":"10.1093\/rheumatology\/kead377","article-title":"Increased risk of cardiovascular disease preceding diagnosis of incident ANCA-associated vasculitis: a Danish nationwide study","volume":"63","author":"Nygaard","year":"2024","journal-title":"Rheumatology (Oxford)"},{"key":"2026051406284689400_bib67","doi-asserted-by":"publisher","first-page":"rkaa056","DOI":"10.1093\/rap\/rkaa056","article-title":"Venous thromboembolism in anti-neutrophil cytoplasmic antibody-associated vasculitis: an underlying prothrombotic condition?","volume":"4","author":"Antovic","year":"2020","journal-title":"Rheumatol Adv Pract"},{"key":"2026051406284689400_bib68","doi-asserted-by":"publisher","first-page":"4616","DOI":"10.1093\/rheumatology\/keab057","article-title":"Venous thromboembolism in ANCA-associated vasculitis: a population-based cohort study","volume":"60","author":"Liapi","year":"2021","journal-title":"Rheumatology United Kingdom"},{"key":"2026051406284689400_bib69","doi-asserted-by":"publisher","first-page":"272","DOI":"10.1016\/j.ero.2025.08.007","article-title":"Long-term risk of malignancies in ANCA-associated vasculitis","volume":"1","author":"Sanchez\u00a0Alamo","year":"2025","journal-title":"EULAR Rheumatol Open"},{"key":"2026051406284689400_bib70","doi-asserted-by":"publisher","first-page":"542","DOI":"10.1002\/art.33361","article-title":"Risk factors for relapse of antineutrophil cytoplasmic antibody-associated vasculitis","volume":"64","author":"Walsh","year":"2012","journal-title":"Arthritis Rheum"},{"key":"2026051406284689400_bib71","doi-asserted-by":"publisher","first-page":"644","DOI":"10.1038\/ki.2009.218","article-title":"The clinical course of ANCA small-vessel vasculitis on chronic dialysis","volume":"76","author":"Lionaki","year":"2009","journal-title":"Kidney Int"},{"key":"2026051406284689400_bib72","doi-asserted-by":"publisher","first-page":"1431","DOI":"10.1002\/art.42843","article-title":"Risk of relapse of antineutrophil cytoplasmic antibody-associated vasculitis in a randomized controlled trial of plasma exchange and glucocorticoids","volume":"76","author":"Junek","year":"2024","journal-title":"Arthritis Rheumatol"},{"key":"2026051406284689400_bib73","doi-asserted-by":"publisher","first-page":"1989","DOI":"10.1093\/rheumatology\/keae409","article-title":"Relapses and serious adverse events during rituximab maintenance therapy in ANCA-associated vasculitis: a multicentre retrospective study","volume":"64","author":"Gialouri","year":"2025","journal-title":"Rheumatology (Oxford)"},{"key":"2026051406284689400_bib74","doi-asserted-by":"publisher","first-page":"217","DOI":"10.1097\/RHU.0000000000001030","article-title":"Evaluation of PR3-ANCA status after Rituximab for ANCA-associated vasculitis","volume":"25","author":"McClure","year":"2019","journal-title":"J Clin Rheumatol"},{"key":"2026051406284689400_bib75","doi-asserted-by":"publisher","DOI":"10.1136\/rmdopen-2025-006434","article-title":"Concordance between presenting features and relapse in granulomatosis with polyangiitis: implications for risk assessment and counselling","volume":"11","author":"Iudici","year":"2025","journal-title":"RMD Open"},{"key":"2026051406284689400_bib76","doi-asserted-by":"publisher","DOI":"10.3389\/fimmu.2025.1655326","article-title":"Antineutrophil cytoplasmic antibody-associated vasculitis: insights into relapse risk and future management directions","author":"Alberici","year":"2025","journal-title":"Front Immunol"},{"key":"2026051406284689400_bib77","doi-asserted-by":"publisher","first-page":"1665","DOI":"10.2215\/CJN.03190321","article-title":"Disease activity and adverse events in patients with ANCA-associated vasculitides undergoing long-term dialysis","volume":"16","author":"Kauffmann","year":"2021","journal-title":"Clin J Am Soc Nephrol"},{"key":"2026051406284689400_bib78","doi-asserted-by":"publisher","first-page":"919","DOI":"10.1007\/s40620-019-00642-x","article-title":"Kidney transplantation in ANCA-associated vasculitis","volume":"32","author":"Sagmeister","year":"2019","journal-title":"J Nephrol"},{"key":"2026051406284689400_bib79","doi-asserted-by":"publisher","first-page":"63","DOI":"10.1093\/ndt\/gfaa303","article-title":"Increasing incidence and improved survival in ANCA-associated vasculitis\u2014a Danish nationwide study","volume":"37","author":"Nelveg-Kristensen","year":"2022","journal-title":"Nephrol Dial Transplant"},{"key":"2026051406284689400_bib80","doi-asserted-by":"publisher","DOI":"10.1111\/1756-185X.70139","article-title":"Burden of infection-related hospitalization and the impact of multidisciplinary care in ANCA-associated vasculitis: a retrospective cohort","volume":"28","author":"Yeung","year":"2025","journal-title":"Int J Rheum Dis"}],"container-title":["Nephrology Dialysis Transplantation"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/academic.oup.com\/ndt\/advance-article-pdf\/doi\/10.1093\/ndt\/gfag077\/67694653\/gfag077.pdf","content-type":"application\/pdf","content-version":"am","intended-application":"syndication"},{"URL":"https:\/\/academic.oup.com\/ndt\/advance-article-pdf\/doi\/10.1093\/ndt\/gfag077\/67694653\/gfag077.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,5,14]],"date-time":"2026-05-14T10:28:58Z","timestamp":1778754538000},"score":1,"resource":{"primary":{"URL":"https:\/\/academic.oup.com\/ndt\/advance-article\/doi\/10.1093\/ndt\/gfag077\/8566312"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,3,31]]},"references-count":80,"URL":"https:\/\/doi.org\/10.1093\/ndt\/gfag077","relation":{},"ISSN":["0931-0509","1460-2385"],"issn-type":[{"value":"0931-0509","type":"print"},{"value":"1460-2385","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,3,31]]},"article-number":"gfag077"}}