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Epidemiology and clinical characteristics of biopsy-confirmed adult-onset IgA vasculitis in southern Sweden.

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Peer-reviewed

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Authors

Thalen, Michaela 
Segelmark, Mårten 
Sode, Jacob 

Abstract

OBJECTIVE: Immunoglobulin A vasculitis (IgAV) is the most prevalent primary childhood vasculitis in Sweden, but is considerably rarer in adults. This study aims to describe the epidemiology, clinical characteristics and renal outcome of adult-onset IgAV in Skåne, Sweden. METHODS: The study area consisted of Skåne, the southernmost region of Sweden, with a population ≥18 years of 990 464 on 31 December 2010. Adult patients assigned the International Classification of Diseases-10 code for IgAV (D69.0) from 2000 through 2019 were retrospectively identified in a population-based database. Medical records were reviewed to validate the diagnosis of IgAV and extract data. Only patients with clinical manifestations of IgAV and biopsy-confirmed disease were included. The annual incidence and point prevalence of biopsy-confirmed IgAV were estimated. RESULTS: Fifty-nine patients (19 women) were classified as having adult-onset IgAV. The incidence was 3 per 1 000 000 and was higher among men than women (4 vs 2/1 000 000, p=0.004). Ninety-seven per cent of patients presented with non-thrombocytopenic purpura, 78% with renal involvement, 59% with arthritis/arthralgia and 39% with gastrointestinal symptoms. Fifteen per cent developed chronic kidney disease stage ≥G3 a and one patient progressed to end-stage kidney disease during follow-up. CONCLUSION: Adult-onset IgAV is rare in southern Sweden with the incidence higher in men than in women. IgAV frequently affects the kidneys and leads to chronic kidney disease in adults, although the long-term renal outcome appears favourable compared with other small-vessel vasculitides affecting the kidneys.

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Peer reviewed: True


Acknowledgements: We thank Morteza Najibi, PhD and Raïssa de Boer, PhD, for help with statistics and data management. Part of this work was presented as a poster at the 20th International Vasculitis and ANCA Workshop, 2022, Dublin, Ireland.


Funder: Stiftelsen Konung Gustaf V:s 80-årsfond; FundRef: http://dx.doi.org/10.13039/501100007857; Grant(s): FAI-2022- 0871

Keywords

epidemiology, incidence, systemic vasculitis, Male, Adult, Humans, Female, Child, IgA Vasculitis, Retrospective Studies, Sweden, Immunoglobulin A, Vasculitis, Biopsy, Renal Insufficiency, Chronic

Journal Title

RMD Open

Conference Name

Journal ISSN

2056-5933
2056-5933

Volume Title

10

Publisher

BMJ
Sponsorship
Alfred Österlund Foundation (NA)
Anna-Greta Crafoords Stiftelse för Reumatologisk Forskning (20212003)