High frequency of immunodeficiency-like states in systemic lupus erythematosus: a cross-sectional study in 300 consecutive patients

Objective. To determine the frequency of immunodeficiency-like states in SLE and related clinical features. Methods. Three hundred and fifteen SLE patients and 301 controls were evaluated for C4A and C4B gene copy number, immunoglobulin isotypes, IgG subclasses, total haemolytic complement (CH50), C...

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Detalhes bibliográficos
Autores: Perazzio, Sandro F. [UNIFESP], Granados, Atila [UNIFESP], Salomao, Reinaldo, Silva, Neusa P. [UNIFESP], Carneiro-Sampaio, Magda, Andrade, Luis Eduardo C.[UNIFESP]
Formato: artículo
Estado:Versión publicada
Fecha de publicación:2016
País:Brasil
Recursos:Universidade Federal de São Paulo (UNIFESP)
Repositorio:Repositório Institucional da UNIFESP
Idioma:inglés
OAI Identifier:oai:repositorio.unifesp.br:11600/51093
Acesso em linha:http://dx.doi.org/10.1093/rheumatology/kew227
http://repositorio.unifesp.br/handle/11600/51093
Access Level:acceso abierto
Palavra-chave:systemic lupus erythematosus
primary immunodeficiency
autoimmune diseases
immunoglobulin deficiency
Descrição
Resumo:Objective. To determine the frequency of immunodeficiency-like states in SLE and related clinical features. Methods. Three hundred and fifteen SLE patients and 301 controls were evaluated for C4A and C4B gene copy number, immunoglobulin isotypes, IgG subclasses, total haemolytic complement (CH50), C2, C3 and neutrophil oxidative burst. C2 and C3 genes were sequenced in cases of low C2 or C3 levels. Those presenting abnormal CH50 with normal C2 and C3 underwent C1q-C9 determination. Patients with active SLE and abnormal results were re-tested after the flare or were excluded if no remission was attained. Fifteen patients were excluded on this basis. Persistent abnormal results characterized an immunodeficiency-like state. Results. A significantly higher percentage of SLE patients presented an immunodeficiency-like state compared with controls (28.7% vs 3.3%