Systemic Vasculitides in Portugal and Brazil: Preliminary Results from the Reuma.pt/vasculitis Registry

Mariana de Aguiar¹, Alexandre wagner Silva de Souza², Joana Martinho³, Adriana Carones⁴, Ana Beatriz Bacchiega de Freitas⁵, Ana Catarina Duarte⁶, Ana Filipa Águeda⁷, Camila Souto Oliveira Elias⁸, Catarina Cortesão⁹, Catarina Soares¹⁰, Carla Macieira¹¹, Carlos Eduardo Garcez Teixeira¹², Daniela Peixoto¹³, Duarte Vinha³, Estela Nogueira³, Fabricia Simil¹⁴, Francisca Guimarães¹³, Frederico Rajão Martins¹⁵, Gilda Ferreira¹⁶, Helena Assunção⁴, Heloisa Rulff⁸, Jorge Pestana Lopes⁶, José Costa³, Julia Medeiros¹⁷, Lilian Santos Porto¹⁸, Luiz Felipe Adsuara¹⁷, Manuella Ochtrop⁸, Maria João Gonçalves¹⁹, Mariana Diz Lopes²⁰, Matheus Vieira²¹, Nikita Khmelinskii¹¹, Rita Torres²², Sarah Neaime¹, Vitor Teixeira²³, Zoraida Sachetto¹² and Cristina Ponte²⁴, ¹Universidade Federal de São Paulo (UNIFESP), São Paulo, Brazil, ²Division of Rheumatology, Medical School, Universidade Federal de São Paulo, São Paulo, Brazil, ³Centro Hospitalar Universitario Lisboa Norte, Lisboa, Portugal, ⁴Centro Hospitalar e Universitário de Coimbra, Coimbra, Portugal, ⁵Faculdade de Ciências da Saúde de Barretos, Barretos, Brazil, ⁶Hospital Garcia de Orta, Almada, Portugal, ⁷Centro Hospitalar Universitário Cova da Beira, Covilhã, Portugal, ⁸Universidade Estadual do Rio de Janeiro, Rio de Janeiro, Brazil, ⁹Instituto Português de Reumatologia, Lisboa, Portugal, ¹⁰Centro Unidade Local de Saúde do Alto Minho, Viana do Castelo, Portugal, ¹¹Centro Hospitalar Universitário Lisboa Norte, Lisboa, Portugal, ¹²Universidade Estadual de Campinas, Campinas, Brazil, ¹³Centro Unidade Local de Saúde do Alto Minho, Ponte de Lima, Portugal, ¹⁴Universidade Federal de Minas Gerais, Belo Horizonte, Brazil, ¹⁵Department of Rheumatology, University Hospital Centre of Algarve, Faro, Portugal, ¹⁶Federal University of Minas Gerais, Belo Horizonte, Brazil, ¹⁷Santa Casa de Misericórdia de São Paulo, São Paulo, Brazil, ¹⁸Santa Casa de Misericórdia de Belo Horizonte, Belo Horizonte, Brazil, ¹⁹Centro Hospitalar de Lisboa Ocidental, Lisboa, Portugal, ²⁰Centro Hospitalar Universitário de São João, Porto, Portugal, ²¹Universidade Federal de São Paulo; Universidade Federal do Rio de Janeiro, Rio de Janeiro, Brazil, ²²Hospital Egas Moniz, Lisboa, Portugal, ²³Hospital de Faro, CHUA, Lisbon, Portugal, ²⁴Department of Rheumatology, Centro Hospitalar Universitário Lisboa Norte, Centro Académico de Medicina de Lisboa, Lisbon, Portugal; Rheumatology Research Unit, Instituto de Medicina Molecular, Faculdade de Medicina, Universidade de Lisboa, Centro Académico de Medicina de Lisboa, Lisbon, Portugal

Meeting: ACR Convergence 2023

Keywords: Epidemiology, registry, Vasculitis

Session Information

Date: Sunday, November 12, 2023

Title: (0117–0144) Epidemiology & Public Health Poster I

Session Type: Poster Session A

Session Time: 9:00AM-11:00AM

Background/Purpose: The epidemiology of vasculitis varies widely across different geographic areas of the world which may be due to different ethnic and environmental factors. Brazil has a heterogeneous population with influences from Indigenous, African, Asian, and European countries, while Portugal has a very ethnically homogeneous background. This study aims to assess the main differences in the profile of systemic vasculitides between Portugal and Brazil.

Methods: Collaborative project between the Portuguese and the Brazilian Societies of Rheumatology in which centres from both countries were invited to register data in the vasculitis module of the Rheumatic Diseases Portuguese Register, Reuma.pt/vasculitis. A cross-sectional analysis was performed comparing demographic, ethnic and diagnostic information between Brazilian and Portuguese centres.

Results: A total of 1,955 patients were analysed: 74.2% from 30 Portuguese centres and 25.8% from 7 Brazilian centres. Portuguese patients were predominantly European White (89.2%) and in Brazil the most common ethnic groups with vasculitis were the non-European White (48.3%) and Mestizos (40.1%); 5.4% of all participants were born in other countries. Brazilian patients were younger at the onset of symptoms [35.2 (24.1-46.6) vs.48.1 (27.4-70.1) years; p< 0.05]and diagnosis of vasculitis [37.0 (27.6-48.3) vs. 50.8 (32.7-70.4) years; p< 0.05] than Portuguese patients, respectively. When analysing individual forms of vasculitis, Brazilian patients with giant cell arteritis (GCA), Takayasu arteritis (TAK), polyarteritis nodosa (PAN) and granulomatosis with polyangiitis (GPA) were significantly younger than Portuguese patients at diagnosis (p< 0.05). The proportion of females was higher in Portuguese patients with Behçet’s disease (BD) than in Brazilian patients (p=0.03). No differences regarding the proportion of females were observed for other vasculitides (Table 1). The most common form of vasculitis in both countries was BD followed by GCA in Portugal and by TAK in Brazil. Regarding ANCA-associated vasculitis, GPA was more common in Brazil and microscopic polyangiitis (MPA) in Portugal. Both countries had similar proportions of patients with PAN and eosinophilic granulomatosis with polyangiitis (EGPA) (Figure 1 and Table 2). Time elapsed between the onset of symptoms and the diagnosis of GCA was higher in Brazil than in Portugal (p=0.014) while Portugal had a longer interval between the onset of BD symptoms and its diagnosis compared to Brazil (p< 0.05) (Table 1).

Conclusion: In this large multicentre binational study, Portugal and Brazil had a different profile of systemic vasculitis concerning the proportion of GCA and TAK patients, as well as GPA and MPA patients. In addition, both countries had differences in the age of onset, female gender, and ethnicity of patients with systemic vasculitis.

References

Ponte C, et al. Orphanet J Rare Dis, 2020.
Watts RA, et al. Nat Rev Rheumatol, 2022.

Table 1 – Comparison of features in vasculitis patients between Portugal and Brazil.

Figure 1 – Frequency of systemic vasculitis in Portugal and Brazil. GCA, giant cell arteritis; TAK, Takayasu arteritis; BD, Behçet´s disease; PAN, polyarteritis nodosa; GPA, granulomatosis with polyangiitis; MPA, microscopic polyangiitis; EGPA, eosinophilic granulomatosis with polyangiitis

Table 2 – Frequency of systemic vasculitis in Portugal and Brazil.

Disclosures: M. de Aguiar: None; A. Silva de Souza: None; J. Martinho: None; A. Carones: None; A. Bacchiega de Freitas: None; A. Duarte: None; A. Águeda: None; C. Souto Oliveira Elias: None; C. Cortesão: None; C. Soares: None; C. Macieira: None; C. Garcez Teixeira: None; D. Peixoto: None; D. Vinha: None; E. Nogueira: None; F. Simil: None; F. Guimarães: None; F. Rajão Martins: None; G. Ferreira: None; H. Assunção: None; H. Rulff: None; J. Pestana Lopes: None; J. Costa: None; J. Medeiros: None; L. Santos Porto: None; L. Adsuara: None; M. Ochtrop: None; M. Gonçalves: None; M. Diz Lopes: None; M. Vieira: None; N. Khmelinskii: None; R. Torres: None; S. Neaime: None; V. Teixeira: None; Z. Sachetto: None; C. Ponte: None.

To cite this abstract in AMA style:

de Aguiar M, Silva de Souza A, Martinho J, Carones A, Bacchiega de Freitas A, Duarte A, Águeda A, Souto Oliveira Elias C, Cortesão C, Soares C, Macieira C, Garcez Teixeira C, Peixoto D, Vinha D, Nogueira E, Simil F, Guimarães F, Rajão Martins F, Ferreira G, Assunção H, Rulff H, Pestana Lopes J, Costa J, Medeiros J, Santos Porto L, Adsuara L, Ochtrop M, Gonçalves M, Diz Lopes M, Vieira M, Khmelinskii N, Torres R, Neaime S, Teixeira V, Sachetto Z, Ponte C. Systemic Vasculitides in Portugal and Brazil: Preliminary Results from the Reuma.pt/vasculitis Registry [abstract]. Arthritis Rheumatol. 2023; 75 (suppl 9). https://acrabstracts.org/abstract/systemic-vasculitides-in-portugal-and-brazil-preliminary-results-from-the-reuma-pt-vasculitis-registry/. Accessed .

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