Abstract
ANCA-ASSOCIATED VASCULITIS INCIDENCE IN A NORTHERN SPANISH HEALTH REGION, 1994-2022: A POPULATION-BASED STUDY
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S. Al Fazazi, A. Herrero-Morant, V. Calvo-Río, M. Renuncio García, C. Escagedo Cagigas, M. Rodriguez Vidriales, R. BlancoHospital Universitario Puerta del Mar, Rheumatology, Cádiz, Spain
Marqués de Valdecilla University Hospital, Rheumatology, Santander, Spain
Marqués de Valdecilla University Hospital, Immunology, Santander, Spain
Marqués de Valdecilla University Hospital, Nephrology, Santander, Spain
Background Anti-neutrophil cytoplasmic antibodies (ANCA)-associated vasculitis (AAV) includes granulomatosis with polyangiitis (GPA), eosinophilic granulomatosis with polyangiitis (EGPA) or microscopic polyarteritis (MPA). Precise estimation of the incidence of AAV has been difficult due to the absence of reliable diagnostic criteria.
Objectives To estimate the incidence in a Northern Spanish population-based cohort.
Methods Population-based study of 132 patients diagnosed with small vessel vasculitis between 1994 and 2022 in a tertiary hospital. Finally, 98 patients were included as AAV according to ACR/EULAR 2022 criteria [1]. Incidence was estimated by gender, age, and year of diagnosis.
Results AAV was diagnosed in 98 (49 women/ 49 men) patients: GPA (n=47, 48%) MPA (n=37,37.8%) and EGPA (n=14, 14.3%).
Annual incidences were estimated in AAV (Figure 1). GPA annual incidence in our population area in the 1994-2022 period was 2.81 per 1,000,000 people, 95% CI: 3.77-1.85 (2.82 [4.35-1.29] in males and 2.80 [4.50-1.09] in females). An upward trend in annual incidence over time was observed with rates ranging from 1.88 in 1994 to 6.84 in 2022 (weak correlation; r 2=0.2729). On the other hand, MPA annual incidence was 2.23 per 1,000,000 people (1.92 [3.55-0.27] in males and 2.46 [4.07-0.85] in females). Rates ranged from 1.88 in 1994 to 1.71 in 2022 with an upward trend over time (very weak correlation; r 2=0.0746). In the case of GEPA, annual incidence was 0.83 per 1,000,000 people, 95% CI: 1.72-0.06 (1.22 [2.74-0.30] in males and 0.46 [2.29-1.37] in females). As in the other types of AAV, there was an upward trend over time with variations of from 1.88 in 1999 to 3.42 in 2022 (weak correlation; r 2=0.3335).
A comparison between different geographical areas is summarized in Table 1. Wide varia-tions in annual incidence per million were observed in all AAV (GPA 2.1-34; MPA 2.23-10.4; EGPA 0.64-2.7). The highest annual incidence of all AAV was observed in nordic countries and central Europe while the lowest in Southern Europe.
Conclusion There seems to be a progressive increase in incidence of AAV over the years in the studied population. Annual incidence in our population was similar to that of other south-ern European countries.
Reference [1]Yazici, Hasan et al Current opinion in rheumatology vol. 35,1 (2023): 1-5.
Image/graph:Figure 1. Annual incidence of AAV in 1994-2022.
Table 1. AAV incidence cases reported in the literature.
Study, year
Country, data source
Time period
Incident cases per million population GPA
Incident cases per million population MPA
Incident cases per million population EGPA
Pearce, F. A. et al. 2016
Nottingham, UK, population register
1656-1663
8.2
13.4
1.5
Nilsen, A. T. et al. 2020
Tromso, Norway, population register
1999-2013
15.6
10.4
2.7
Mohammad, A. J et al. 2009
Lund, South Sweden, population register
1997-2006
9.8
10.1
0.9
Takala, J. H et al. 2008
Finland, analysis national discharge data
1996-2000
9.3
NA
NA
Hellmich, B. et al. 2021
Germany, analysis insurance claims database
2013-2016
34
13
NA
Dadoniene, J. et al 2005
Vilnius, Lithuania, analysis hospital database
1990-1999
2.1
3
1.3
Panagiotakis, S. H. et al. 2009
Crete, Greece, analysis hospital database
1995-2003
6.6
10.2
NA
Romero-Gómez, C. et al. 2004
Malaga, Spain, retrospectiva population.
1994-2010
2.1
3.4
0.64
Catanaso, M. et al. 2014
Reggio Emilia, Italia, analysis hospital discharge database
2004-2009
3.4
NA
NA
Vinit, J. et al. 2006
Burgundy, France, analysis hospital discharge database
1998-2008
NA
NA
1.2
Kanecki, K. et al. 2018
Poland, analysis hospital discharge database
2008-2013
NA
NA
1.5
Kanecki, K. et al. 2018
Poland, analysis hospital discharge database
2011-2015
7.7
NA
NA
Present study, 2023
Northern Spain.
1999-2022
2.81
2.23
0.83
Acknowledgements: NIL.
Disclosure of Interests Salma Al Fazazi: None declared, Alba Herrero-Morant: None declared, Vanesa Calvo-Río: None declared, Mónica Renuncio García: None declared, Clara Escagedo Cagigas: None declared, Maria Rodriguez Vidriales: None declared, Ricardo Blanco Speakers bureau: Abbvie, Lilly, pfizer, Roche, Bristol-Myers, Janssen, Galapagos and MSD., Consultant of: Abbvie, Lilly, pfizer, Roche, Bristol-Myers, Janssen and MSD., Grant/research support from: Abbvie, MSD, Novartis and Roche.
Keywords: Descriptive studies, Vasculitis, Epidemiology
DOI: 10.1136/annrheumdis-2023-eular.3838Citation: , volume 82, supplement 1, year 2023, page 798Session: Epidemiology, risk factors for disease or disease progression
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