Clinical and laboratory characteristics of Brazilian versus non-Brazilian primary antiphospholipid syndrome patients in AntiPhospholipid Syndrome Alliance for Clinical Trials and InternatiOnal Networking (APS ACTION) clinical database and repository
Abstract Background Antiphospholipid syndrome (APS) is characterized by episodes of thrombosis, obstetric morbidity or both, associated with persistently positive antiphospholipid antibodies (aPL). Studying the profile of a rare disease in an admixed population is important as it can provide new ins...
| Main Authors: | , , , , , , , , , , , , , , , , , , , , , , , , , |
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| Format: | Article |
| Language: | English |
| Published: |
BMC
2021-10-01
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| Series: | Advances in Rheumatology |
| Subjects: | |
| Online Access: | https://doi.org/10.1186/s42358-021-00222-3 |
| _version_ | 1819011180580044800 |
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| author | Erivelton de Azevedo Lopes Gustavo Guimarães Moreira Balbi Maria G. Tektonidou Vittorio Pengo Savino Sciascia Amaia Ugarte H. Michael Belmont Maria Gerosa Paul R. Fortin Chary Lopez-Pedrera Lanlan Ji Hannah Cohen Guilherme Ramires de Jesús D. Ware Branch Cecilia Nalli Michelle Petri Esther Rodriguez Nina Kello Roberto Ríos-Garcés Jason S. Knight Tatsuya Atsumi Rohan Willis Maria Laura Bertolaccini Doruk Erkan Danieli Andrade APS ACTION |
| author_facet | Erivelton de Azevedo Lopes Gustavo Guimarães Moreira Balbi Maria G. Tektonidou Vittorio Pengo Savino Sciascia Amaia Ugarte H. Michael Belmont Maria Gerosa Paul R. Fortin Chary Lopez-Pedrera Lanlan Ji Hannah Cohen Guilherme Ramires de Jesús D. Ware Branch Cecilia Nalli Michelle Petri Esther Rodriguez Nina Kello Roberto Ríos-Garcés Jason S. Knight Tatsuya Atsumi Rohan Willis Maria Laura Bertolaccini Doruk Erkan Danieli Andrade APS ACTION |
| author_sort | Erivelton de Azevedo Lopes |
| collection | DOAJ |
| description | Abstract Background Antiphospholipid syndrome (APS) is characterized by episodes of thrombosis, obstetric morbidity or both, associated with persistently positive antiphospholipid antibodies (aPL). Studying the profile of a rare disease in an admixed population is important as it can provide new insights for understanding an autoimmune disease. In this sense of miscegenation, Brazil is characterized by one of the most heterogeneous populations in the world, which is the result of five centuries of interethnic crosses of people from three continents. The objective of this study was to compare the clinical and laboratory characteristics of Brazilian vs. non-Brazilian primary antiphospholipid syndrome (PAPS) patients. Methods We classified PAPS patients into 2 groups: Brazilian PAPS patients (BPAPS) and PAPS patients from other countries (non-BPAPS). They were compared regarding demographic characteristics, criteria and non-criteria APS manifestations, antiphospholipid antibody (aPL) profile, and the adjusted Global Antiphospholipid Syndrome Score (aGAPSS). Results We included 415 PAPS patients (88 [21%] BPAPS and 327 [79%] non-BPAPS). Brazilian patients were significantly younger, more frequently female, sedentary, obese, non-white, and had a higher frequency of livedo (25% vs. 10%, p < 0.001), cognitive dysfunction (21% vs. 8%, p = 0.001) and seizures (16% vs. 7%, p = 0.007), and a lower frequency of thrombocytopenia (9% vs. 18%, p = 0.037). Additionally, they were more frequently positive for lupus anticoagulant (87.5% vs. 74.6%, p = 0.01), and less frequently positive to anticardiolipin (46.6% vs. 73.7%, p < 0.001) and anti-ß2-glycoprotein-I (13.6% vs. 62.7%, p < 0.001) antibodies. Triple aPL positivity was also less frequent (8% vs. 41.6%, p < 0.001) in Brazilian patients. Median aGAPSS was lower in the Brazilian group (8 vs. 10, p < 0.0001). In the multivariate analysis, BPAPS patients still presented more frequently with livedo, cognitive dysfunction and sedentary lifestyle, and less frequently with thrombocytopenia and triple positivity to aPL. They were also less often white. Conclusions Our study suggests a specific profile of PAPS in Brazil with higher frequency of selected non-criteria manifestations and lupus anticoagulant positivity. Lupus anticoagulant (not triple positivity) was the major aPL predictor of a classification criteria event. |
| first_indexed | 2024-12-21T01:24:04Z |
| format | Article |
| id | doaj.art-715cecf8601841039c95b619557f5261 |
| institution | Directory Open Access Journal |
| issn | 2523-3106 |
| language | English |
| last_indexed | 2024-12-21T01:24:04Z |
| publishDate | 2021-10-01 |
| publisher | BMC |
| record_format | Article |
| series | Advances in Rheumatology |
| spelling | doaj.art-715cecf8601841039c95b619557f52612022-12-21T19:20:34ZengBMCAdvances in Rheumatology2523-31062021-10-016111610.1186/s42358-021-00222-3Clinical and laboratory characteristics of Brazilian versus non-Brazilian primary antiphospholipid syndrome patients in AntiPhospholipid Syndrome Alliance for Clinical Trials and InternatiOnal Networking (APS ACTION) clinical database and repositoryErivelton de Azevedo Lopes0Gustavo Guimarães Moreira Balbi1Maria G. Tektonidou2Vittorio Pengo3Savino Sciascia4Amaia Ugarte5H. Michael Belmont6Maria Gerosa7Paul R. Fortin8Chary Lopez-Pedrera9Lanlan Ji10Hannah Cohen11Guilherme Ramires de Jesús12D. Ware Branch13Cecilia Nalli14Michelle Petri15Esther Rodriguez16Nina Kello17Roberto Ríos-Garcés18Jason S. Knight19Tatsuya Atsumi20Rohan Willis21Maria Laura Bertolaccini22Doruk Erkan23Danieli Andrade24APS ACTIONUniversity of São PauloUniversity of São PauloNational and Kapodistrian University of AthensUniversity Hospital PadovaCenter of Research of Immunopathology and Rare Diseases, University of TurinHospital Universitario CrucesHospital for Joint Diseases, New York UniversityClinical Immunology and Rheumatology Unit, IRCCS Istituto Auxologico ItalianoCHU de Québec-Université LavalRheumatology Service, IMIBIC/Reina Sofia Hospital, University of CordobaRheumatology and Immunology Department, Peking University First HospitalHaemostasis Research Unit, Department of Haematology, University College LondonUniversidade Do Estado Do Rio de JaneiroUniversity of Utah and Intermountain HealthcareRheumatology and Immunology Unit, ASST Spedali Civili of BresciaJohns Hopkins University School of MedicineHospital UniversitarioNorthwell HealthInstitut d’Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), Hospital ClínicDivision of Rheumatology, University of MichiganHokkaido University HospitalAntiphospholipid Standardization Laboratory, University of Texas Medical BranchAcademic Department of Vascular Surgery, King’s College London British Heart Foundation Centre of Excellence, School of Cardiovascular Medicine and SciencesBarbara Volcker Center for Women and Rheumatic Disease, Hospital for Special Surgery, Weill Cornell MedicineUniversity of São PauloAbstract Background Antiphospholipid syndrome (APS) is characterized by episodes of thrombosis, obstetric morbidity or both, associated with persistently positive antiphospholipid antibodies (aPL). Studying the profile of a rare disease in an admixed population is important as it can provide new insights for understanding an autoimmune disease. In this sense of miscegenation, Brazil is characterized by one of the most heterogeneous populations in the world, which is the result of five centuries of interethnic crosses of people from three continents. The objective of this study was to compare the clinical and laboratory characteristics of Brazilian vs. non-Brazilian primary antiphospholipid syndrome (PAPS) patients. Methods We classified PAPS patients into 2 groups: Brazilian PAPS patients (BPAPS) and PAPS patients from other countries (non-BPAPS). They were compared regarding demographic characteristics, criteria and non-criteria APS manifestations, antiphospholipid antibody (aPL) profile, and the adjusted Global Antiphospholipid Syndrome Score (aGAPSS). Results We included 415 PAPS patients (88 [21%] BPAPS and 327 [79%] non-BPAPS). Brazilian patients were significantly younger, more frequently female, sedentary, obese, non-white, and had a higher frequency of livedo (25% vs. 10%, p < 0.001), cognitive dysfunction (21% vs. 8%, p = 0.001) and seizures (16% vs. 7%, p = 0.007), and a lower frequency of thrombocytopenia (9% vs. 18%, p = 0.037). Additionally, they were more frequently positive for lupus anticoagulant (87.5% vs. 74.6%, p = 0.01), and less frequently positive to anticardiolipin (46.6% vs. 73.7%, p < 0.001) and anti-ß2-glycoprotein-I (13.6% vs. 62.7%, p < 0.001) antibodies. Triple aPL positivity was also less frequent (8% vs. 41.6%, p < 0.001) in Brazilian patients. Median aGAPSS was lower in the Brazilian group (8 vs. 10, p < 0.0001). In the multivariate analysis, BPAPS patients still presented more frequently with livedo, cognitive dysfunction and sedentary lifestyle, and less frequently with thrombocytopenia and triple positivity to aPL. They were also less often white. Conclusions Our study suggests a specific profile of PAPS in Brazil with higher frequency of selected non-criteria manifestations and lupus anticoagulant positivity. Lupus anticoagulant (not triple positivity) was the major aPL predictor of a classification criteria event.https://doi.org/10.1186/s42358-021-00222-3Antiphospholipid syndromePrimary antiphospholipid syndromeAntiphospholipid antibodiesLupus AnticoagulantAnticardiolipin antibodiesAnti-beta-2 glycoprotein I antibodies |
| spellingShingle | Erivelton de Azevedo Lopes Gustavo Guimarães Moreira Balbi Maria G. Tektonidou Vittorio Pengo Savino Sciascia Amaia Ugarte H. Michael Belmont Maria Gerosa Paul R. Fortin Chary Lopez-Pedrera Lanlan Ji Hannah Cohen Guilherme Ramires de Jesús D. Ware Branch Cecilia Nalli Michelle Petri Esther Rodriguez Nina Kello Roberto Ríos-Garcés Jason S. Knight Tatsuya Atsumi Rohan Willis Maria Laura Bertolaccini Doruk Erkan Danieli Andrade APS ACTION Clinical and laboratory characteristics of Brazilian versus non-Brazilian primary antiphospholipid syndrome patients in AntiPhospholipid Syndrome Alliance for Clinical Trials and InternatiOnal Networking (APS ACTION) clinical database and repository Advances in Rheumatology Antiphospholipid syndrome Primary antiphospholipid syndrome Antiphospholipid antibodies Lupus Anticoagulant Anticardiolipin antibodies Anti-beta-2 glycoprotein I antibodies |
| title | Clinical and laboratory characteristics of Brazilian versus non-Brazilian primary antiphospholipid syndrome patients in AntiPhospholipid Syndrome Alliance for Clinical Trials and InternatiOnal Networking (APS ACTION) clinical database and repository |
| title_full | Clinical and laboratory characteristics of Brazilian versus non-Brazilian primary antiphospholipid syndrome patients in AntiPhospholipid Syndrome Alliance for Clinical Trials and InternatiOnal Networking (APS ACTION) clinical database and repository |
| title_fullStr | Clinical and laboratory characteristics of Brazilian versus non-Brazilian primary antiphospholipid syndrome patients in AntiPhospholipid Syndrome Alliance for Clinical Trials and InternatiOnal Networking (APS ACTION) clinical database and repository |
| title_full_unstemmed | Clinical and laboratory characteristics of Brazilian versus non-Brazilian primary antiphospholipid syndrome patients in AntiPhospholipid Syndrome Alliance for Clinical Trials and InternatiOnal Networking (APS ACTION) clinical database and repository |
| title_short | Clinical and laboratory characteristics of Brazilian versus non-Brazilian primary antiphospholipid syndrome patients in AntiPhospholipid Syndrome Alliance for Clinical Trials and InternatiOnal Networking (APS ACTION) clinical database and repository |
| title_sort | clinical and laboratory characteristics of brazilian versus non brazilian primary antiphospholipid syndrome patients in antiphospholipid syndrome alliance for clinical trials and international networking aps action clinical database and repository |
| topic | Antiphospholipid syndrome Primary antiphospholipid syndrome Antiphospholipid antibodies Lupus Anticoagulant Anticardiolipin antibodies Anti-beta-2 glycoprotein I antibodies |
| url | https://doi.org/10.1186/s42358-021-00222-3 |
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