Reuma.pt/vasculitis – the Portuguese vasculitis registry
Abstract Background The vasculitides are a group of rare diseases with different manifestations and outcomes. New therapeutic options have led to the need for long-term registries. The Rheumatic Diseases Portuguese Register, Reuma.pt, is a web-based electronic clinical record, created in 2008, which...
| Main Authors: | , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , |
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| Format: | Article |
| Language: | English |
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BMC
2020-05-01
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| Series: | Orphanet Journal of Rare Diseases |
| Subjects: | |
| Online Access: | http://link.springer.com/article/10.1186/s13023-020-01381-0 |
| _version_ | 1818068461286326272 |
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| author | Cristina Ponte Nikita Khmelinskii Vítor Teixeira Karine Luz Daniela Peixoto Marília Rodrigues Mariana Luís Lídia Teixeira Sandra Sousa Nathalie Madeira Joana A. Aleixo Teresa Pedrosa Sofia Serra Raquel Campanilho-Marques Walter Castelão Ana Cordeiro Inês Cordeiro Sílvia Fernandes Carla Macieira Pedro Madureira Armando Malcata Romana Vieira Fernando Martins Graça Sequeira Jaime C. Branco Lúcia Costa José Vaz Patto José Canas da Silva José A. Pereira da Silva Carmo Afonso Helena Canhão Maria J. Santos Raashid A. Luqmani João E. Fonseca |
| author_facet | Cristina Ponte Nikita Khmelinskii Vítor Teixeira Karine Luz Daniela Peixoto Marília Rodrigues Mariana Luís Lídia Teixeira Sandra Sousa Nathalie Madeira Joana A. Aleixo Teresa Pedrosa Sofia Serra Raquel Campanilho-Marques Walter Castelão Ana Cordeiro Inês Cordeiro Sílvia Fernandes Carla Macieira Pedro Madureira Armando Malcata Romana Vieira Fernando Martins Graça Sequeira Jaime C. Branco Lúcia Costa José Vaz Patto José Canas da Silva José A. Pereira da Silva Carmo Afonso Helena Canhão Maria J. Santos Raashid A. Luqmani João E. Fonseca |
| author_sort | Cristina Ponte |
| collection | DOAJ |
| description | Abstract Background The vasculitides are a group of rare diseases with different manifestations and outcomes. New therapeutic options have led to the need for long-term registries. The Rheumatic Diseases Portuguese Register, Reuma.pt, is a web-based electronic clinical record, created in 2008, which currently includes specific modules for 12 diseases and > 20,000 patients registered from 79 rheumatology centres. On October 2014, a dedicated module for vasculitis was created as part of the European Vasculitis Society collaborative network, enabling prospective collection and central storage of encrypted data from patients with this condition. All Portuguese rheumatology centres were invited to participate. Data regarding demographics, diagnosis, classification criteria, assessment tools, and treatment were collected. We aim to describe the structure of Reuma.pt/vasculitis and characterize the patients registered since its development. Results A total of 687 patients, with 1945 visits, from 13 centres were registered; mean age was 53.4 ± 19.3 years at last visit and 68.7% were females. The most common diagnoses were Behçet’s disease (BD) (42.5%) and giant cell arteritis (GCA) (17.8%). Patients with BD met the International Study Group criteria and the International Criteria for BD in 85.3 and 97.2% of cases, respectively. Within the most common small- and medium-vessel vasculitides registered, median [interquartile range] Birmingham Vasculitis Activity Score (BVAS) at first visit was highest in patients with ANCA-associated vasculitis (AAV) (17.0 [12.0]); there were no differences in the proportion of patients with AAV or polyarteritis nodosa who relapsed (BVAS≥1) or had a major relapse (≥1 major BVAS item) during prospective assessment (p = 1.00, p = 0.479). Biologic treatment was prescribed in 0.8% of patients with GCA, 26.7% of patients with AAV, and 7.6% of patients with BD. There were 34 (4.9%) deaths reported. Conclusions Reuma.pt/vasculitis is a bespoke web-based registry adapted for routine care of patients with this form of rare and complex diseases, allowing an efficient data-repository at a national level with the potential to link with other international databases. It facilitates research, trials recruitment, service planning and benchmarking. |
| first_indexed | 2024-12-10T15:39:56Z |
| format | Article |
| id | doaj.art-78c8e017724b4eafbbfce6d717256202 |
| institution | Directory Open Access Journal |
| issn | 1750-1172 |
| language | English |
| last_indexed | 2024-12-10T15:39:56Z |
| publishDate | 2020-05-01 |
| publisher | BMC |
| record_format | Article |
| series | Orphanet Journal of Rare Diseases |
| spelling | doaj.art-78c8e017724b4eafbbfce6d7172562022022-12-22T01:43:08ZengBMCOrphanet Journal of Rare Diseases1750-11722020-05-0115111210.1186/s13023-020-01381-0Reuma.pt/vasculitis – the Portuguese vasculitis registryCristina Ponte0Nikita Khmelinskii1Vítor Teixeira2Karine Luz3Daniela Peixoto4Marília Rodrigues5Mariana Luís6Lídia Teixeira7Sandra Sousa8Nathalie Madeira9Joana A. Aleixo10Teresa Pedrosa11Sofia Serra12Raquel Campanilho-Marques13Walter Castelão14Ana Cordeiro15Inês Cordeiro16Sílvia Fernandes17Carla Macieira18Pedro Madureira19Armando Malcata20Romana Vieira21Fernando Martins22Graça Sequeira23Jaime C. Branco24Lúcia Costa25José Vaz Patto26José Canas da Silva27José A. Pereira da Silva28Carmo Afonso29Helena Canhão30Maria J. Santos31Raashid A. Luqmani32João E. Fonseca33Rheumatology Department, Hospital de Santa Maria - Centro Hospitalar Universitário Lisboa Norte, Centro Académico de Medicina de LisboaRheumatology Department, Hospital de Santa Maria - Centro Hospitalar Universitário Lisboa Norte, Centro Académico de Medicina de LisboaRheumatology Department, Hospital de Santa Maria - Centro Hospitalar Universitário Lisboa Norte, Centro Académico de Medicina de LisboaRheumatology Department, Hospital de Santa Maria - Centro Hospitalar Universitário Lisboa Norte, Centro Académico de Medicina de LisboaRheumatology Department, Unidade Local de Saúde do Alto MinhoRheumatology Department, Centro Hospitalar e Universitário de CoimbraRheumatology Department, Centro Hospitalar e Universitário de CoimbraRheumatology Department, Hospital Garcia de OrtaRheumatology Department, Hospital Garcia de OrtaRheumatology Department, Instituto Português de ReumatologiaRheumatology Department, Centro Hospitalar de São JoãoRheumatology Department, Hospital de Egas Moniz, Centro Hospitalar Lisboa OcidentalRheumatology Department, Hospital de Egas Moniz, Centro Hospitalar Lisboa OcidentalRheumatology Department, Hospital de Santa Maria - Centro Hospitalar Universitário Lisboa Norte, Centro Académico de Medicina de LisboaRheumatology Department, Hospital de Egas Moniz, Centro Hospitalar Lisboa OcidentalRheumatology Department, Hospital Garcia de OrtaRheumatology Department, Hospital de Santa Maria - Centro Hospitalar Universitário Lisboa Norte, Centro Académico de Medicina de LisboaRheumatology Department, Hospital de Santa Maria - Centro Hospitalar Universitário Lisboa Norte, Centro Académico de Medicina de LisboaRheumatology Department, Hospital de Santa Maria - Centro Hospitalar Universitário Lisboa Norte, Centro Académico de Medicina de LisboaRheumatology Department, Centro Hospitalar de São JoãoRheumatology Department, Centro Hospitalar e Universitário de CoimbraRheumatology Department, Centro Hospitalar de São JoãoSociedade Portuguesa de ReumatologiaRheumatology Department, Hospital de Faro, Centro Hospitalar do AlgarveRheumatology Department, Hospital de Egas Moniz, Centro Hospitalar Lisboa OcidentalRheumatology Department, Centro Hospitalar de São JoãoRheumatology Department, Instituto Português de ReumatologiaRheumatology Department, Hospital Garcia de OrtaRheumatology Department, Centro Hospitalar e Universitário de CoimbraRheumatology Department, Unidade Local de Saúde do Alto MinhoSociedade Portuguesa de ReumatologiaUnidade de Investigação em Reumatologia, Instituto de Medicina Molecular, Faculdade de Medicina, Universidade de LisboaNuffield Department of Orthopaedics, Rheumatology and Musculoskeletal Sciences, University of OxfordRheumatology Department, Hospital de Santa Maria - Centro Hospitalar Universitário Lisboa Norte, Centro Académico de Medicina de LisboaAbstract Background The vasculitides are a group of rare diseases with different manifestations and outcomes. New therapeutic options have led to the need for long-term registries. The Rheumatic Diseases Portuguese Register, Reuma.pt, is a web-based electronic clinical record, created in 2008, which currently includes specific modules for 12 diseases and > 20,000 patients registered from 79 rheumatology centres. On October 2014, a dedicated module for vasculitis was created as part of the European Vasculitis Society collaborative network, enabling prospective collection and central storage of encrypted data from patients with this condition. All Portuguese rheumatology centres were invited to participate. Data regarding demographics, diagnosis, classification criteria, assessment tools, and treatment were collected. We aim to describe the structure of Reuma.pt/vasculitis and characterize the patients registered since its development. Results A total of 687 patients, with 1945 visits, from 13 centres were registered; mean age was 53.4 ± 19.3 years at last visit and 68.7% were females. The most common diagnoses were Behçet’s disease (BD) (42.5%) and giant cell arteritis (GCA) (17.8%). Patients with BD met the International Study Group criteria and the International Criteria for BD in 85.3 and 97.2% of cases, respectively. Within the most common small- and medium-vessel vasculitides registered, median [interquartile range] Birmingham Vasculitis Activity Score (BVAS) at first visit was highest in patients with ANCA-associated vasculitis (AAV) (17.0 [12.0]); there were no differences in the proportion of patients with AAV or polyarteritis nodosa who relapsed (BVAS≥1) or had a major relapse (≥1 major BVAS item) during prospective assessment (p = 1.00, p = 0.479). Biologic treatment was prescribed in 0.8% of patients with GCA, 26.7% of patients with AAV, and 7.6% of patients with BD. There were 34 (4.9%) deaths reported. Conclusions Reuma.pt/vasculitis is a bespoke web-based registry adapted for routine care of patients with this form of rare and complex diseases, allowing an efficient data-repository at a national level with the potential to link with other international databases. It facilitates research, trials recruitment, service planning and benchmarking.http://link.springer.com/article/10.1186/s13023-020-01381-0Rare diseasesVasculitisPatient registriesDatabase management systemsPatient reported outcome measures |
| spellingShingle | Cristina Ponte Nikita Khmelinskii Vítor Teixeira Karine Luz Daniela Peixoto Marília Rodrigues Mariana Luís Lídia Teixeira Sandra Sousa Nathalie Madeira Joana A. Aleixo Teresa Pedrosa Sofia Serra Raquel Campanilho-Marques Walter Castelão Ana Cordeiro Inês Cordeiro Sílvia Fernandes Carla Macieira Pedro Madureira Armando Malcata Romana Vieira Fernando Martins Graça Sequeira Jaime C. Branco Lúcia Costa José Vaz Patto José Canas da Silva José A. Pereira da Silva Carmo Afonso Helena Canhão Maria J. Santos Raashid A. Luqmani João E. Fonseca Reuma.pt/vasculitis – the Portuguese vasculitis registry Orphanet Journal of Rare Diseases Rare diseases Vasculitis Patient registries Database management systems Patient reported outcome measures |
| title | Reuma.pt/vasculitis – the Portuguese vasculitis registry |
| title_full | Reuma.pt/vasculitis – the Portuguese vasculitis registry |
| title_fullStr | Reuma.pt/vasculitis – the Portuguese vasculitis registry |
| title_full_unstemmed | Reuma.pt/vasculitis – the Portuguese vasculitis registry |
| title_short | Reuma.pt/vasculitis – the Portuguese vasculitis registry |
| title_sort | reuma pt vasculitis the portuguese vasculitis registry |
| topic | Rare diseases Vasculitis Patient registries Database management systems Patient reported outcome measures |
| url | http://link.springer.com/article/10.1186/s13023-020-01381-0 |
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