Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 2023 Mar;9(1):e002949.
doi: 10.1136/rmdopen-2022-002949.

Stable incidence but increase in prevalence of ANCA-associated vasculitis in southern Sweden: a 23-year study

Jens Rathmann  1 , Mårten Segelmark  2 , Martin Englund  3 , Aladdin J Mohammad  4   5
Affiliations

Stable incidence but increase in prevalence of ANCA-associated vasculitis in southern Sweden: a 23-year study

Jens Rathmann et al. RMD Open. 2023 Mar.

Abstract

Objective: To update the epidemiology of anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV) in a defined geographical area of southern Sweden.

Methods: The study area comprised 14 municipalities with a combined adult population (≥18 years) of 623 872 in 2019. All cases diagnosed with AAV in 1997-2019 in the study area were included in the estimate of incidence. Diagnosis of AAV was verified by case record review, and cases were classified using the European Medicines Agency algorithm. Point prevalence was estimated on 01 January 2020.

Results: Three hundred and seventy-four patients (median age 67.5 years, 47% female) were diagnosed with new-onset AAV during the study period. One hundred and ninety-two were classified as granulomatosis with polyangiitis (GPA), 159 as microscopic polyangiitis (MPA) and 23 as EGPA. The average annual incidence/million adults was 30.1 (95% CI 27.0 to 33.1) for AAV: 15.4 (95% CI 13.3 to 17.6) for GPA, 12.8 (95% CI 10.8 to 14.8) for MPA and 1.8 (95% CI 1.1 to 2.6) for eosinophilic GPA (EGPA). Incidence was stable during the study period, 30.3/million 1997-2003, 30.4/million 2004-2011 and 29.5/million 2012-2019. The incidence increased with age and was highest in age group 70-84 years (96/million adults). On 1 January 2020, the prevalence was 428/million adults and was higher in males than in females (480 vs 378/million).

Conclusions: The incidence of AAV in southern Sweden was found stable over the course of 23 years; while the prevalence has increased, which might indicate better management and treatment of AAV resulting in improved survival.

Keywords: Epidemiology; Granulomatosis with polyangiitis; Systemic vasculitis.

PubMed Disclaimer

Conflict of interest statement

Competing interests: None declared.

Figures

Figure 1
Figure 1
Study area A and B within the Skåne region in the southernmost part of Sweden.
Figure 2
Figure 2
Incidence of AAV per million adults, stratified by 3 years overlapping, for all AAV and different phenotypes. AAV, anti-neutrophil cytoplasmic antibody-associated vasculitis; EGPA, eosinophilic granulomatosis with polyangiitis; GPA, granulomatosis with polyangiitis; MPA, microscopic polyangiitis.
Figure 3
Figure 3
Age specific incidence rates in respect to phenotype, serology and sex. AAV, anti-neutrophil cytoplasmic antibody-associated vasculitis; EGPA, eosinophilic granulomatosis with polyangiitis; GPA, granulomatosis with polyangiitis; MPA, microscopic polyangiitis.
Figure 4
Figure 4
Seasonal variations. Incidence ratios with 95% CI of AAV-diagnoses in seasons (winter is reference). AAV, anti-neutrophil cytoplasmic antibody-associated vasculitis.
See this image and copyright information in PMC

References

    1. Jennette JC, Falk RJ, Bacon PA. Overview of the 2012 revised international chapel hill consensus conference nomenclature of vasculitides. Clin Exp Nephrol 2013;17:603–6. 10.1007/s10157-013-0869-6 - DOI - PMC - PubMed
    1. Radice A, Sinico RA. Antineutrophil cytoplasmic antibodies (ANCA). Autoimmunity 2005;38:93–103. 10.1080/08916930400022673 - DOI - PubMed
    1. Little MA, Nightingale P, Verburgh CA, et al. . Early mortality in systemic vasculitis: relative contribution of adverse events and active vasculitis. Ann Rheum Dis 2010;69:1036–43. 10.1136/ard.2009.109389 - DOI - PubMed
    1. Flossmann O, Berden A, de Groot K, et al. . Long-Term patient survival in ANCA-associated vasculitis. Ann Rheum Dis 2011;70:488–94. 10.1136/ard.2010.137778 - DOI - PubMed
    1. Mohammad AJ. An update on the epidemiology of ANCA-associated vasculitis. Rheumatology (Oxford) 2020;59:iii42–50. 10.1093/rheumatology/keaa089 - DOI - PubMed

Publication types