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Epidemiology of anti-neutrophil cytoplasmic antibody-associated vasculitis in Lithuania, 2012–2021: a retrospective cohort study

Year: 2025
Type: article
Study design: Observational Study
Abstract: OBJECTIVES: The aim of this study was to assess the incidence, prevalence and mortality of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) in Lithuania. DESIGN: Retrospective cohort study. SETTING: Lithuanian National Health Insurance Fund and Causes of Death registries, covering 1 January 2012 through 31 December 2021. PARTICIPANTS: version (ICD-10)) recorded between 1 January 2012 and 31 December 2021; 2) diagnosis of AAV was recorded in the database at least twice with at least 1-month period between the two timepoints; 3) a record of at least once prescribed reimbursed medication-glucocorticoids (prednisolone or methylprednisolone), conventional synthetic disease-modifying anti-rheumatic drugs (methotrexate, azathioprine, hydroxychloroquine and cyclophosphamide) or biological disease-modifying anti-rheumatic drug (rituximab or available biosimilars); and 4) age >18 years at the time of diagnosis. OUTCOME MEASURES: Data for the analysis included sex, age, ICD-10 code of AAV, the first date of AAV diagnosis and date of death. The study period was subdivided into periods 1 (2013-2015), 2 (2016-2018) and 3 (2019-2021). Temporal trends of the incidence of AAV were assessed. Point prevalence data were recorded. Sex- and age-standardised mortality ratios (SMRs) were calculated. The life expectancy of patients with AAV was estimated by standard single-decrement life-table analysis. RESULTS: We identified 236 patients with AAV (female, 58%) with an annual incidence of 8.22 per 1 000 000 adult inhabitants (period 1, 9.83; period 2, 6.88; period 3, 7.95). Increasing incidence was noted for MPA (period 1, 0.9; period 3, 2.49). The incidence of GPA (4.89 per 1 000 000 inhabitants per year) was three times higher than the total incidence of EGPA and MPA. The prevalence of AAV per 1 000 000 adult population increased from 35.92 in 2015 to 69.14 in 2021. 40 deaths were recorded during the study period, with a mean age at death of 65.2 (±13.03). AAV was associated with reduced life expectancy compared with the general population, especially when diagnosed at a young age. The SMRs for the total AAV cohort revealed a decreasing trend: 1.79 in period 1 and 1.67 in period 3. CONCLUSIONS: AAV is associated with increased mortality. During the study period, the incidence of AAV was found to be stable, and the prevalence has increased.
Source: BMJ Open
Language: English
Field: Medicine
Open Access status: gold
Estimated APC paid: $1,839
OpenAlex ID: W4416397000
FWCI: 0.608
Cites: 26
Cited by: 1
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