Incidence and prevalence of ANCA-associated vasculitis in Oslo, Norway, applying different criteria-based case definitions: a population-based cohort study

Abstract

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.

Keywords: Classification; Epidemiology; Incidence; Prevalence; Systemic vasculitis.

Figure 1. Overview of strategy for identifying all ANCA-vasculitis cases resident in Oslo 2000–2016 and classification by the EMA and 2022 ACR/EULAR criteria of all incident cases in Oslo identified with AAV phenotypes. ICD10, International Classification of Disease, 10th Revision; AAV, ANCA-associated vasculitis; ACR/EULAR 2022, 2022 American College of Rheumatology/European Alliance of Associations for Rheumatology classification criteria; ANCA, antineutrophil cytoplasmic antibody; EGPA, eosinophilic granulomatosis with polyangiitis; EMA, European Medicines Agency; GPA, granulomatosis with polyangiitis; M30.0, PAN; M30.1, polyarteritis with lung involvement (Churg–Strauss syndrome/EGPA); M30.8, other conditions related to polyarteritis nodosa; M31.3, GPA; M31.7, MPA; M31.8, other specified necrotising vasculopathies; M31.9, necrotising vasculopathy, unspecified; MPA, microscopic polyangiitis; N, number; PAN, polyarteritis nodosa.

Figure 2. Age-specific incidence by sex and by diagnosis. Age-specific incidence rates for (A) sex and (B) phenotype/diagnosis. AAV, antineutrophil cytoplasmic-associated vasculitis; EGPA, eosinophilic granulomatosis with polyangiitis; GPA, granulomatosis with polyangiitis; MPA, microscopic polyangiitis.