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Epidemiology of anti-neutrophil cytoplasmic antibody-associated vasculitis in Lithuania, 2012-2021: a retrospective
Giedre Dereseviciene1, Gediminas Gumbis2, Dalia Miltiniene2,3
1State Research Institute Center of Innovative Medicine, Vilnius, Vilnius County, Lithuania giedre.dereseviciene@imcentras.lt.
Insights
Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) incidence remained stable, while prevalence increased in Lithuania. AAV is linked to higher mortality and reduced life expectancy, though standardized mortality ratios show a decreasing trend.
Area of Science:
- Rheumatology
- Epidemiology
- Public Health
Background:
- Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) encompasses rare autoimmune diseases.
- Understanding the epidemiological trends of AAV is crucial for healthcare planning and resource allocation.
Purpose of the Study:
- To determine the incidence, prevalence, and mortality rates of AAV in Lithuania.
- To analyze temporal trends in AAV incidence and prevalence.
- To assess the impact of AAV on life expectancy and mortality.
Main Methods:
- A retrospective cohort study utilizing Lithuanian national healthcare and mortality registries from 2012 to 2021.
- Patients were identified based on ICD-10 codes for EGPA, GPA, or MPA, with specific diagnostic and treatment criteria.
- Incidence, prevalence, sex- and age-standardized mortality ratios (SMRs), and life expectancy were calculated.
Main Results:
- A total of 236 AAV patients were identified, with an overall annual incidence of 8.22 per 1,000,000 inhabitants.
- The incidence of microscopic polyangiitis (MPA) showed an increasing trend, while granulomatosis with polyangiitis (GPA) had the highest incidence.
- AAV prevalence increased from 35.92 in 2015 to 69.14 per 1,000,000 in 2021.
- AAV was associated with reduced life expectancy, and SMRs showed a decreasing trend over the study periods.
Conclusions:
- AAV incidence in Lithuania remained stable during the study period, but prevalence significantly increased.
- AAV is associated with increased mortality and reduced life expectancy, particularly when diagnosed at a younger age.
- Despite challenges, a decreasing trend in standardized mortality ratios suggests potential improvements in AAV management or outcomes.
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:
Patients were identified from national healthcare registries in Lithuania. The following inclusion criteria were applied: 1) diagnosis of eosinophilic granulomatosis with polyangiitis (EGPA), granulomatosis with polyangiitis (GPA) or microscopic polyangiitis (MPA) (diagnosis codes M30.1, M31.3 and M31.7, according to International Classification of Diseases 10th 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.
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