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Doctoral Thesis
DOI
Document
Author
Full name
Cristiane Mendes da Silva
E-mail
Institute/School/College
Knowledge Area
Date of Defense
Published
São Paulo, 2019
Supervisor
Committee
Shinjo, Samuel Katsuyuki (President)
Andrade, Danieli Castro Oliveira de
Kayser, Cristiane
Rodrigues, Maria Carolina de Oliveira
Title in Portuguese
Características clínicas e laboratoriais de pacientes afro-brasileiros com esclerose sistêmica
Keywords in Portuguese
Autoanticorpos
Escleroderma sistêmico
Esclerodermia difusa
Esclerodermia limitada
Grupo com ancestrais do continente africano
Grupo com ancestrais do continente europeu
Grupos étnicos
Abstract in Portuguese
Introdução: Os afro-brasileiros compreendem um grupo de negros e pardos com uma ancestralidade diversa. Como as diferenças raciais podem estar associadas a apresentações distintas, torna-se relevante realizar uma análise de associações clínicas e sorológicas de pacientes afro-brasileiros com esclerose sistêmica (ES). Objetivos: Analisar a apresentação clínica e laboratorial, características demográficas e sobrevida dos afro-brasileiros com ES. Métodos: Foram avaliados soros de 260 pacientes adultos com ES (57 afro-brasileiros e 203 brancos). Pacientes com síndromes de sobreposição foram excluídos. Dados clínicos e demográficos foram obtidos de um banco de dados de registros eletrônicos. A análise laboratorial incluiu anti-CENP-A/ CENP-B, Scl70, RNA polimerase III, Ku, fibrilarina, Th/To, PM/Scl75 e PM/Scl100 por line imunoblotting e anticorpos antinucleares por imunofluorescência indireta em células HEp-2. Resultados: Em relação aos brancos, os pacientes afro-brasileiros com ES apresentaram menor tempo de doença (9,0±5,4 anos vs. 11,3±7,5 anos, P=0,001), maior frequência do padrão nucleolar (28% vs. 13%, P=0,008) e menores frequências do padrão centromérico (14% vs. 29%, P=0,026) e CENP-B (18% vs. 34%, P=0,017). Comparações adicionais entre grupos étnicos de acordo com a forma clínica revelaram que pacientes afro-brasileiros com ES difusa apresentaram maior frequência de hipertensão pulmonar (P=0,017), envolvimento cardíaco (P=0,037), padrão nucleolar (P=0,036) e anticorpos antifibrilarina (P=0,037). De maneira diferente foi observado para a forma limitada apenas uma menor frequência de envolvimento esofágico (P=0,050) e padrão centromérico (P=0,049). A análise de sobrevida mostrou que os afro-brasileiros apresentaram maior mortalidade quando ajustados para sexo e forma clínica (risco relativo 3,65; intervalo de confiança de 95% 1,05-12,62, P=0,041). Conclusão: Os pacientes afro-brasileiros com ES, apesar de sua forte impressão de ancestralidade europeia, apresentam características distintas de acordo com a forma clínica e uma doença mais grave do que os brancos, com um padrão muito semelhante ao descrito para negros de outros países
Title in English
Clinical and laboratory features of African-Brazilian patients with systemic sclerosis
Keywords in English
African Continental ancestry group
Autoantibodies
Ethnic groups
European Continental ancestry group
Scleroderma diffuse
Scleroderma limited
Scleroderma systemic
Abstract in English
Introduction: African-Brazilians comprise a group of blacks and "pardos" with a diverse ancestry. As racial differences can be associated with distinct presentations, it becomes relevant to assess the clinical and serological associations of African-Brazilians with systemic sclerosis (SSc). Objectives: To analyze clinical and laboratory presentation, demographic features and survival of Afro-Brazilians with SSc. Methods: Sera from 260 adult SSc patients (57 African-Brazilians and 203 whites) were evaluated. Patients with overlap syndromes were excluded. Clinical and demographic data were obtained from an electronic register database. Laboratory analysis included: anti-CENPA/CENP-B, Scl70, RNA polymerase III, Ku, fibrillarin, Th/To, PM/Scl75 and PM/Scl100 by line immunoassay and antinuclear antibodies by indirect immunofluorescence on HEp-2 cells. Results: In relation to whites, African-Brazilian patients with SSc presented shorter disease duration (9.0±5.4 years vs. 11.3±7.5 years, P=0.001), higher frequency of nucleolar pattern (28% vs. 13%, P=0.008) and lower frequencies of centromeric pattern (14% vs. 29%, P=0.026) and CENP-B (18% vs. 34%, P=0.017). Further comparison of ethnic groups according to subsets revealed that African-Brazilian patients with diffuse SSc presented higher frequency of pulmonary hypertension (P=0.017), heart involvement (P=0.037), nucleolar pattern (P=0.036) and anti-fibrillarin antibodies (P=0.037). A different pattern was observed for the limited subset with solely a lower frequency of esophageal involvement (P=0.050) and centromeric pattern (P=0.049). Survival analysis showed that African-Brazilians had a higher mortality, when adjusted for gender and clinical subset (relative risk 3.65, confidence interval 95% 1.05-12.62, P=0.041). Conclusion: African-Brazilian patients with SSc, despite their strong imprint of European ancestry, have distinct characteristics according to clinical subset and an overall more severe disease than whites, with a pattern very similar to the described for blacks from other countries
 
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Publishing Date
2019-06-04
 
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