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Changing incidence and serotype trends in ANCA-associated vasculitis: a 30-year population-based study in Norfolk, UK
Chetan B Mukhtyar1, Karen Ashurst2, Fiona L Coath1
1Rheumatology Department, Norfolk and Norwich University Hospitals NHS Foundation Trust, Norwich, UK.
Objectives:
The ANCA associated vasculitides (AAV) are rare diseases with poor outcomes if untreated. Over a 30-year period we have investigated the changing incidence of AAV and its subtypes-granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), eosinophilic GPA (EGPA), proteinase 3 AAV (PR3 AAV), and myeloperoxidase AAV (MPO AAV).
Methods:
The study population included adults in the county of Norfolk, UK between 1991 and 2020. Diagnosis of AAV was made clinically and supported by EMA classification. Classification by ANCA subtype was possible from 2001. The overall incidence of clinical phenotypes GPA, MPA and EGPA, and serological phenotypes PR3 AAV and MPO AAV were analysed for temporal fluctuations and seasonality.
Results:
From 1991 to 2020, the incidence (95% CI) of AAV, GPA, MPA and EGPA was 25.1 (22.5, 27.9), 13.6 (11.7, 15.8), 8.5 (7.0, 10.2) and 3.0 (2.1, 4.0) per million person-years, respectively. From 2001 to 2020, the incidence (95% CI) of PR3 AAV and MPO AAV was 11.5 (9.4, 13.9) and 11.7 (9.6, 14.1) per million person-years, respectively. The temporal fluctuations in the incidence of EGPA and PR3 AAV were not significant. The incidence of GPA has a statistical periodicity of 9 years. The incidence of MPO AAV demonstrated a linear rise from 2001 to 2020 (R2 = 0.66). Our patients are getting older at diagnosis. Seasonality was not observed.
Conclusion:
This is the longest incidence study of AAV in the world. The incidence of GPA showed a 9-year periodicity. The incidence of MPO AAV rose steadily. Its relationship to older age at diagnosis needs formal exploration.
Insights
This 30-year study reveals trends in ANCA-associated vasculitis (AAV) incidence. Granulomatosis with polyangiitis (GPA) shows a 9-year cycle, while Myeloperoxidase AAV (MPO AAV) incidence steadily increased.
Area of Science:
- Rheumatology
- Epidemiology
- Immunology
Background:
- ANCA-associated vasculitides (AAV) are rare, severe autoimmune diseases.
- Understanding AAV incidence trends is crucial for public health and resource allocation.
Purpose of the Study:
- To analyze the 30-year incidence of AAV and its subtypes in Norfolk, UK.
- To identify temporal fluctuations, seasonality, and age-related changes in AAV incidence.
Main Methods:
- Retrospective analysis of adult AAV cases diagnosed between 1991 and 2020 in Norfolk, UK.
- Clinical diagnosis supported by EMA classification; ANCA subtype classification from 2001.
- Analysis of incidence rates for Granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), eosinophilic GPA (EGPA), Proteinase 3 AAV (PR3 AAV), and Myeloperoxidase AAV (MPO AAV).
Main Results:
- Overall AAV incidence was 25.1 per million person-years.
- GPA incidence exhibited a 9-year periodicity; MPO AAV incidence showed a linear rise from 2001-2020.
- Patients were diagnosed at an older age over time; no seasonality was observed.
Conclusions:
- This is the longest global incidence study of AAV.
- The 9-year periodicity of GPA and rising MPO AAV incidence require further investigation.
- The association between increasing MPO AAV incidence and older age at diagnosis warrants formal exploration.
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