Incidence and prevalence of ANCA-associated vasculitis in Oslo, Norway, applying different criteria-based case definitions: a population-based cohort study
Objective To provide complete data on the incidence and prevalence of antineutrophil cytoplasmic antibody-associated vasculitides (AAV) over the years 2000–2016 in the Oslo area, Norway, with 528 924 adults (aged 18+) in 2016.Methods From administrative databases, we identified all cases with Intern...
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BMJ Publishing Group
2025-07-01
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| Series: | RMD Open |
| Online Access: | https://rmdopen.bmj.com/content/11/3/e005526.full |
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| author | Øyvind Molberg Cathrine Brunborg Ragnar Gunnarsson Garen Torhild Karin R Kilian Sigrun Skaar Holme |
| author_facet | Øyvind Molberg Cathrine Brunborg Ragnar Gunnarsson Garen Torhild Karin R Kilian Sigrun Skaar Holme |
| author_sort | Øyvind Molberg |
| collection | DOAJ |
| description | Objective To provide complete data on the incidence and prevalence of antineutrophil cytoplasmic antibody-associated vasculitides (AAV) over the years 2000–2016 in the Oslo area, Norway, with 528 924 adults (aged 18+) in 2016.Methods From administrative databases, we identified all cases with International Classification of Disease, 10th Revision (ICD-10) codes indicative of necrotising small vessel vasculitis during 2000–2016 in the Oslo area. We manually chart reviewed every case identified through the ICD-10 search to confirm (or reject) a clinical AAV diagnosis. Cases with confirmed clinical AAV were classified by the European Medicine Agency (EMA) algorithm and the 2022 American College of Rheumatology/European Alliance of Associations for Rheumatology (ACR/EULAR 2022) classification criteria.Results Among 469 cases with an ICD-10 code indicative of small vessel vasculitis, chart review confirmed AAV in 133 cases with 97 having new onset during the study. Of these 97 incident cases, 57 (60%) were classified as granulomatosis with polyangiitis (GPA), 29 (31%) as microscopic polyangiitis (MPA) and 9 (9%) as eosinophilic granulomatosis with polyangiitis (EGPA) per ACR/EULAR 2022 criteria, while 2 remained unclassified. There was an 11% discordance in AAV case classification between the 2022 criteria and the EMA algorithm. The mean annual incidence of AAV in adults was 12.2 per million (7.3 for GPA, 3.7 for MPA and 1.2 for EGPA). Across the study period, incidence rates increased numerically, and prevalence peaked at 143.7 AAV cases/million adults in 2016.Conclusion This population-based study adds new evidence that AAV increases in Europe and indicates that using the ACR/EULAR 2022 criteria shifts cases from GPA to MPA relative to the EMA algorithm, affecting epidemiology estimates. |
| format | Article |
| id | doaj-art-3cf4e9aebcdd4f05ac2f1b0b8fca30bc |
| institution | Kabale University |
| issn | 2056-5933 |
| language | English |
| publishDate | 2025-07-01 |
| publisher | BMJ Publishing Group |
| record_format | Article |
| series | RMD Open |
| spelling | doaj-art-3cf4e9aebcdd4f05ac2f1b0b8fca30bc2025-08-20T03:30:08ZengBMJ Publishing GroupRMD Open2056-59332025-07-0111310.1136/rmdopen-2025-005526Incidence and prevalence of ANCA-associated vasculitis in Oslo, Norway, applying different criteria-based case definitions: a population-based cohort studyØyvind Molberg0Cathrine Brunborg1Ragnar Gunnarsson2Garen Torhild3Karin R Kilian4Sigrun Skaar Holme5Department of Rheumatology, Oslo University Hospital, Oslo, NorwayBiostatistics, Epidemiology and Health Economics, Oslo University Hospital, Oslo, NorwayDepartment of Rheumatology, Oslo University Hospital, Oslo, NorwayDepartment of Rheumatology, Oslo University Hospital, Oslo, NorwayDepartment of Rheumatology, Oslo University Hospital, Oslo, NorwayDepartment of Teleradiology, Unilabs Røntgen Majorstuen, Oslo, NorwayObjective To provide complete data on the incidence and prevalence of antineutrophil cytoplasmic antibody-associated vasculitides (AAV) over the years 2000–2016 in the Oslo area, Norway, with 528 924 adults (aged 18+) in 2016.Methods From administrative databases, we identified all cases with International Classification of Disease, 10th Revision (ICD-10) codes indicative of necrotising small vessel vasculitis during 2000–2016 in the Oslo area. We manually chart reviewed every case identified through the ICD-10 search to confirm (or reject) a clinical AAV diagnosis. Cases with confirmed clinical AAV were classified by the European Medicine Agency (EMA) algorithm and the 2022 American College of Rheumatology/European Alliance of Associations for Rheumatology (ACR/EULAR 2022) classification criteria.Results Among 469 cases with an ICD-10 code indicative of small vessel vasculitis, chart review confirmed AAV in 133 cases with 97 having new onset during the study. Of these 97 incident cases, 57 (60%) were classified as granulomatosis with polyangiitis (GPA), 29 (31%) as microscopic polyangiitis (MPA) and 9 (9%) as eosinophilic granulomatosis with polyangiitis (EGPA) per ACR/EULAR 2022 criteria, while 2 remained unclassified. There was an 11% discordance in AAV case classification between the 2022 criteria and the EMA algorithm. The mean annual incidence of AAV in adults was 12.2 per million (7.3 for GPA, 3.7 for MPA and 1.2 for EGPA). Across the study period, incidence rates increased numerically, and prevalence peaked at 143.7 AAV cases/million adults in 2016.Conclusion This population-based study adds new evidence that AAV increases in Europe and indicates that using the ACR/EULAR 2022 criteria shifts cases from GPA to MPA relative to the EMA algorithm, affecting epidemiology estimates.https://rmdopen.bmj.com/content/11/3/e005526.full |
| spellingShingle | Øyvind Molberg Cathrine Brunborg Ragnar Gunnarsson Garen Torhild Karin R Kilian Sigrun Skaar Holme Incidence and prevalence of ANCA-associated vasculitis in Oslo, Norway, applying different criteria-based case definitions: a population-based cohort study RMD Open |
| title | Incidence and prevalence of ANCA-associated vasculitis in Oslo, Norway, applying different criteria-based case definitions: a population-based cohort study |
| title_full | Incidence and prevalence of ANCA-associated vasculitis in Oslo, Norway, applying different criteria-based case definitions: a population-based cohort study |
| title_fullStr | Incidence and prevalence of ANCA-associated vasculitis in Oslo, Norway, applying different criteria-based case definitions: a population-based cohort study |
| title_full_unstemmed | Incidence and prevalence of ANCA-associated vasculitis in Oslo, Norway, applying different criteria-based case definitions: a population-based cohort study |
| title_short | Incidence and prevalence of ANCA-associated vasculitis in Oslo, Norway, applying different criteria-based case definitions: a population-based cohort study |
| title_sort | incidence and prevalence of anca associated vasculitis in oslo norway applying different criteria based case definitions a population based cohort study |
| url | https://rmdopen.bmj.com/content/11/3/e005526.full |
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