Autoimmune hemolytic anemia in systemic lupus erythematosus at diagnosis: differences between pediatric and adult patients

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art_GORMEZANO_Autoimmune_hemolytic_anemia_in_systemic_lupus_erythematosus_at_2017.PDF (85.02 KB)
Citações na Scopus
32
Tipo de produção
article
Data de publicação
2017
DOI
Scopus
Web of Science
PubMed
Título da Revista
ISSN da Revista
Título do Volume
Editora
SAGE PUBLICATIONS LTD
Autores
PEREIRA, O. L.
ESTEVES, G. C. X.
Citação
LUPUS, v.26, n.4, p.426-430, 2017
Projetos de Pesquisa
Unidades Organizacionais
Fascículo
Resumo
Objective To determine the overall prevalence of autoimmune hemolytic anemia (AIHA), and to compare clinical and laboratory features in a large population of children and adult lupus patients at diagnosis. Methods This retrospective study evaluated the medical charts of 336 childhood-onset systemic lupus erythematosus (cSLE) and 1830 adult SLE (aSLE) patients followed in the same tertiary hospital. Demographic data, clinical features and disease activity were recorded. AIHA was defined according to the presence of anemia (hemoglobin <10g/dL) and evidence of hemolysis (reticulocytosis and positive direct antiglobulin test (DAT)/Coombs test) at SLE diagnosis. Evans syndrome (ES) was defined by the combination of immune thrombocytopenia (platelet count <100,000/mm(3)) and AIHA. Results The frequency of AIHA at diagnosis was significantly higher in cSLE patients compared to aSLE (49/336 (14%) vs 49/1830 (3%), p=0.0001), with similar frequency of ES (3/336 (0.9%) vs 10/1830 (0.5%), p=0.438). The median of hemoglobin levels was reduced in cSLE vs aSLE patients (8.3 (2.2-10) vs 9.5 (6.6-10) g/dL, p=0.002) with a higher frequency of multiple hemorrhagic manifestations (41% vs 7%, p=0.041) and erythrocyte transfusion due to bleeding (24% vs 5%, p=0.025). cSLE patients also had more often constitutional involvement (84% vs 31%, p<0.001), fever (65% vs 26%, p<0.001), weight loss>2kg (39% vs 6%, p<0.001), reticuloendothelial manifestations (48% vs 8%, p<0.001), hepatomegaly (25% vs 2%, p<0.001) and splenomegaly (21% vs 2%, p=0.004). Other major organ involvements were common but with similar frequencies in cSLE and aSLE (p>0.05). Median systemic lupus erythematosus disease activity index 2000 (SLEDAI-2K) was comparable in cSLE and aSLE (p=0.161). Conclusions We identified that AIHA was not a common condition in cSLE and aSLE, with distinct features characterized by a higher prevalence/severity in children and concomitant constitutional symptoms in the majority of them.
Palavras-chave
Autoimmune hemolytic anemia, childhood, adult, systemic lupus erythematosus
URI
https://observatorio.fm.usp.br/handle/OPI/19924
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Artigos e Materiais de Revistas Científicas - FM/MCM
Artigos e Materiais de Revistas Científicas - FM/MPE
Artigos e Materiais de Revistas Científicas - HC/ICHC
Artigos e Materiais de Revistas Científicas - HC/ICr
Artigos e Materiais de Revistas Científicas - LIM/17
Artigos e Materiais de Revistas Científicas - LIM/36