Universidade Federal de São Paulo São Paulo, So Paulo, Brazil
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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
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.
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.