{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,7]],"date-time":"2026-07-07T15:18:29Z","timestamp":1783437509356,"version":"3.54.6"},"reference-count":32,"publisher":"Oxford University Press (OUP)","issue":"7","license":[{"start":{"date-parts":[[2026,5,25]],"date-time":"2026-05-25T00:00:00Z","timestamp":1779667200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"name":"County Council Research Foundation in the Uppsala region"},{"name":"CUWX foundation"},{"name":"Swedish Kidney Foundation"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2026,7,1]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Introduction<\/jats:title>\n                    <jats:p>To describe the clinical characteristics of a Swedish cohort of patients with anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV) and kidney involvement and to study long-term survival and predictors of all-cause mortality.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>An observational study including patients with AAV and kidney involvement between 1993 and 2023 from two centres in mid-Sweden. Data were obtained from electronic health care records and follow-up visits. Multivariable Cox regression models and Kaplan-Meier analysis were performed to evaluate risk factors of interest predicting all-cause mortality.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>Among 190 patients (56% men, median age 67 years, 43% PR3-ANCA (Proteinase 3 Anti-Neutrophil Cytoplasmic Antibody), and 57% MPO -ANCA (Myeloperoxidase Anti-Neutrophil Cytoplasmic Antibody) positive, estimated glomerular filtration rate at diagnosis was 23 ml\/min\/1.73 m2 [interquartile range (IQR) 11\u201342] and 16% required dialysis. Median follow-up 20 was 7.5 years (IQR 3.6\u201312.3) and 106 deaths occurred. Main causes of death were infections (28%), cardiovascular disease (24%) and malignancy (18%). Cox hazard regression models showed that older age [hazard ratio (HR) 1.10, 95% CI 1.06\u20131.14] was a strong, consistent, and independent predictor of all-cause mortality, irrespective of sex, ANCA type, and kidney function. In the Uppsala cohort (n\u00a0=\u00a091) serum albumin levels at diagnosis emerged also as an independent risk factor when adjusting for age, kidney function and other inflammatory parameters (HR 0.86, 95% CI 0.79\u20130.94).<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>Long-term patient and kidney survival was associated mainly with age and serum albumin levels at diagnosis in this Swedish cohort of patients with AAV. Infections and cardiovascular disease were the most common causes of death, underscoring the importance of infection monitoring and cardiovascular risk management.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1093\/ckj\/sfag159","type":"journal-article","created":{"date-parts":[[2026,5,19]],"date-time":"2026-05-19T11:34:50Z","timestamp":1779190490000},"source":"Crossref","is-referenced-by-count":0,"title":["Long-term survival and mortality predictors in a Swedish cohort of patients with ANCA-positive vasculitis and severe kidney involvement"],"prefix":"10.1093","volume":"19","author":[{"ORCID":"https:\/\/orcid.org\/0009-0002-8493-3897","authenticated-orcid":false,"given":"Klytaimnistra","family":"Voudouri","sequence":"first","affiliation":[{"name":"Department of Medical Sciences, Renal Medicine, Uppsala University , Uppsala ,","place":["Sweden"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ylva","family":"\u00d6stlund","sequence":"additional","affiliation":[{"name":"Department of Molecular and Clinical Medicine\/Nephrology, Institute of Medicine, Sahlgrenska Academy, University of Gothenburg , Gothenburg ,","place":["Sweden"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Karlo","family":"Mihovilovic","sequence":"additional","affiliation":[{"name":"Department of Molecular and Clinical Medicine\/Nephrology, Institute of Medicine, Sahlgrenska Academy, University of Gothenburg , Gothenburg ,","place":["Sweden"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Daniel Kitamura","family":"Bylund","sequence":"additional","affiliation":[{"name":"Department of Molecular and Clinical Medicine\/Nephrology, Institute of Medicine, Sahlgrenska Academy, University of Gothenburg , Gothenburg ,","place":["Sweden"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maria K","family":"Svensson","sequence":"additional","affiliation":[{"name":"Department of Medical Sciences, Renal Medicine, Uppsala University , Uppsala ,","place":["Sweden"]},{"name":"Uppsala Clinical Research Center , Uppsala ,","place":["Sweden"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Aso","family":"Saeed","sequence":"additional","affiliation":[{"name":"Department of Molecular and Clinical Medicine\/Nephrology, Institute of Medicine, Sahlgrenska Academy, University of Gothenburg , Gothenburg ,","place":["Sweden"]}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","published-online":{"date-parts":[[2026,5,25]]},"reference":[{"key":"2026070711064642000_bib1","doi-asserted-by":"publisher","first-page":"2573","DOI":"10.3390\/jcm11092573","article-title":"Systematic review and metaanalysis of worldwide incidence and prevalence of Antineutrophil Cytoplasmic Antibody (ANCA) associated vasculitis","volume":"11","author":"Redondo-Rodriguez","year":"2022","journal-title":"J Clin Med"},{"key":"2026070711064642000_bib2","doi-asserted-by":"publisher","first-page":"iii42","DOI":"10.1093\/rheumatology\/keaa089","article-title":"An update on the epidemiology of ANCA-associated vasculitis","volume":"59","author":"Mohammad","year":"2020","journal-title":"Rheumatology"},{"key":"2026070711064642000_bib3","doi-asserted-by":"publisher","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":"2026070711064642000_bib4","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":"2026070711064642000_bib5","doi-asserted-by":"publisher","first-page":"488","DOI":"10.1136\/ard.2010.137778","article-title":"Long-term patient survival in ANCA-associated vasculitis","volume":"70","author":"Flossmann","year":"2011","journal-title":"Ann Rheum Dis"},{"key":"2026070711064642000_bib6","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":"2026070711064642000_bib7","doi-asserted-by":"publisher","first-page":"e251","DOI":"10.1016\/j.lpm.2015.02.019","article-title":"Risks of treatments and long-term outcomes of systemic ANCA-associated vasculitis","volume":"44","author":"Flossmann","year":"2015","journal-title":"La Presse M\u00e9dicale"},{"key":"2026070711064642000_bib8","doi-asserted-by":"publisher","first-page":"477","DOI":"10.1016\/j.autrev.2012.08.006","article-title":"Renal involvement in anti-neutrophil cytoplasmic autoantibody associated vasculitis","volume":"12","author":"Sinico","year":"2013","journal-title":"Autoimmun Rev"},{"key":"2026070711064642000_bib9","doi-asserted-by":"publisher","first-page":"7319","DOI":"10.3390\/ijms21197319","article-title":"Immunopathogenesis of ANCA-associated vasculitis","volume":"21","author":"Kronbichler","year":"2020","journal-title":"Int J Mol Sci"},{"key":"2026070711064642000_bib10","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":"2026070711064642000_bib11","doi-asserted-by":"publisher","first-page":"1178","DOI":"10.1136\/annrheumdis-2014-206404","article-title":"Rituximab versus cyclophosphamide in ANCA-associated renal vasculitis: 2-year results of a randomised trial","volume":"74","author":"Jones","year":"2015","journal-title":"Ann Rheum Dis"},{"key":"2026070711064642000_bib12","doi-asserted-by":"publisher","first-page":"1483","DOI":"10.1093\/ndt\/gfae018","article-title":"Long-term outcome of kidney function in patients with ANCA-associated vasculitis","volume":"39","author":"Sanchez-Alamo","year":"2024","journal-title":"Nephrol Dial Transplant"},{"key":"2026070711064642000_bib13","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":"2026070711064642000_bib14","doi-asserted-by":"publisher","first-page":"2308","DOI":"10.1093\/rheumatology\/kez589","article-title":"All-cause and cause-specific mortality in ANCA-associated vasculitis: overall and according to ANCA type","volume":"59","author":"Wallace","year":"2020","journal-title":"Rheumatology"},{"key":"2026070711064642000_bib15","doi-asserted-by":"publisher","first-page":"e000435","DOI":"10.1136\/rmdopen-2017-000435","article-title":"Long-term patient survival in a Swedish population-based cohort of patients with ANCA-associated vasculitis","volume":"3","author":"Heijl","year":"2017","journal-title":"RMD Open"},{"key":"2026070711064642000_bib16","doi-asserted-by":"crossref","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":"2026070711064642000_bib17","doi-asserted-by":"publisher","first-page":"102910","DOI":"10.1016\/j.jaut.2022.102910","article-title":"Epidemiology of granulomatosis with polyangiitis and microscopic polyangiitis in adults in France","volume":"133","author":"Bataille","year":"2022","journal-title":"J Autoimmun"},{"key":"2026070711064642000_bib18","doi-asserted-by":"publisher","first-page":"683","DOI":"10.1038\/nrrheum.2017.140","article-title":"Revised 2017 international consensus on testing of ANCAs in granulomatosis with polyangiitis and microscopic polyangiitis","volume":"13","author":"Bossuyt","year":"2017","journal-title":"Nat Rev Rheumatol"},{"key":"2026070711064642000_bib19","doi-asserted-by":"publisher","first-page":"2994","DOI":"10.1681\/ASN.2021070988","article-title":"A unifying approach for GFR estimation: recommendations of the NKF-ASN Task Force on reassessing the inclusion of race in diagnosing kidney disease","volume":"32","author":"Delgado","year":"2021","journal-title":"J Am Soc Nephrol"},{"key":"2026070711064642000_bib20","doi-asserted-by":"crossref","first-page":"gfae069","DOI":"10.1093\/ndt\/gfae069.019","article-title":"#1484 Development of a relapse risk score in patients with ANCA-associated vasculitis treated with cyclophosphamide induction","volume":"39","author":"Lugli","year":"2024","journal-title":"Nephrol Dial Transplant"},{"key":"2026070711064642000_bib21","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 registry","volume":"51","author":"Iudici","year":"2021","journal-title":"Semin Arthritis Rheum"},{"key":"2026070711064642000_bib22","doi-asserted-by":"publisher","first-page":"35","DOI":"10.1186\/s12890-022-01829-y","article-title":"Pulmonary involvement of ANCA-associated vasculitis in adult Chinese patients","volume":"22","author":"Zhou","year":"2022","journal-title":"BMC Pulm Med"},{"key":"2026070711064642000_bib23","doi-asserted-by":"publisher","first-page":"3452","DOI":"10.1002\/art.34562","article-title":"Classification of antineutrophil cytoplasmic autoantibody vasculitides: the role of antineutrophil cytoplasmic autoantibody specificity for myeloperoxidase or proteinase 3 in disease recognition and prognosis","volume":"64","author":"Lionaki","year":"2012","journal-title":"Arthritis Rheumatism"},{"key":"2026070711064642000_bib24","doi-asserted-by":"publisher","first-page":"621","DOI":"10.7326\/0003-4819-143-9-200511010-00005","article-title":"Predictors of relapse and treatment resistance in antineutrophil cytoplasmic antibody\u2013associated small-vessel vasculitis","volume":"143","author":"Hogan","year":"2005","journal-title":"Ann Intern Med"},{"key":"2026070711064642000_bib25","doi-asserted-by":"publisher","first-page":"1144","DOI":"10.1016\/j.kint.2023.02.029","article-title":"Proteinuria and hematuria after remission induction are associated with outcome in ANCA-associated vasculitis","volume":"103","author":"Benichou","year":"2023","journal-title":"Kidney Int"},{"key":"2026070711064642000_bib26","doi-asserted-by":"publisher","first-page":"e030131","DOI":"10.1161\/JAHA.123.030131","article-title":"Albuminuria: an underappreciated risk factor for cardiovascular disease","volume":"13","author":"Barzilay","year":"2024","journal-title":"J Am Heart Assoc"},{"key":"2026070711064642000_bib27","doi-asserted-by":"publisher","first-page":"653","DOI":"10.1016\/j.autrev.2018.01.022","article-title":"Mortality in systemic necrotizing vasculitides: a retrospective analysis of the French Vasculitis Study Group registry","volume":"17","author":"Jardel","year":"2018","journal-title":"Autoimmun Rev"},{"key":"2026070711064642000_bib28","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":"2026070711064642000_bib29","doi-asserted-by":"publisher","first-page":"266","DOI":"10.1002\/jca.21318","article-title":"Plasmapheresis rescue therapy in progressive systemic ANCA-associated vasculitis: single-center results of stepwise escalation of immunosuppression: plasmapheresis in ANCA-Associated Vasculitis","volume":"29","author":"De\u00a0Joode","year":"2014","journal-title":"J Clin Apheresis"},{"key":"2026070711064642000_bib30","doi-asserted-by":"publisher","first-page":"i68","DOI":"10.1093\/rheumatology\/keae612","article-title":"Does plasma exchange have a role in ANCA-associated vasculitis? Viewpoint 2: plasma exchange may be useful under some circumstances","volume":"64","author":"Odler","year":"2025","journal-title":"Rheumatology"},{"key":"2026070711064642000_bib31","doi-asserted-by":"publisher","first-page":"e064604","DOI":"10.1136\/bmj-2021-064604","article-title":"The effects of plasma exchange in patients with ANCA-associated vasculitis: an updated systematic review and meta-analysis","volume":"376","author":"Walsh","year":"2022","journal-title":"BMJ"},{"key":"2026070711064642000_bib32","doi-asserted-by":"publisher","first-page":"399","DOI":"10.3390\/kidneydial2030035","article-title":"Should we still use therapeutic plasma exchange for rapidly progressive glomerulonephritis in ANCA associated vasculitis?","volume":"2","author":"Kaplan","year":"2022","journal-title":"Kidney Dialysis"}],"container-title":["Clinical Kidney Journal"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/academic.oup.com\/ckj\/advance-article-pdf\/doi\/10.1093\/ckj\/sfag159\/68383158\/sfag159.pdf","content-type":"application\/pdf","content-version":"am","intended-application":"syndication"},{"URL":"https:\/\/academic.oup.com\/ckj\/article-pdf\/19\/7\/sfag159\/68383158\/sfag159.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"syndication"},{"URL":"https:\/\/academic.oup.com\/ckj\/article-pdf\/19\/7\/sfag159\/68383158\/sfag159.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,7,7]],"date-time":"2026-07-07T15:12:14Z","timestamp":1783437134000},"score":1,"resource":{"primary":{"URL":"https:\/\/academic.oup.com\/ckj\/article\/doi\/10.1093\/ckj\/sfag159\/8692780"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,5,25]]},"references-count":32,"journal-issue":{"issue":"7","published-print":{"date-parts":[[2026,7,1]]}},"URL":"https:\/\/doi.org\/10.1093\/ckj\/sfag159","relation":{},"ISSN":["2048-8505","2048-8513"],"issn-type":[{"value":"2048-8505","type":"print"},{"value":"2048-8513","type":"electronic"}],"subject":[],"published-other":{"date-parts":[[2026,7]]},"published":{"date-parts":[[2026,5,25]]},"article-number":"sfag159"}}