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

Detalhes bibliográficos
Autor(a) principal: Perazzio, Sandro F. [UNIFESP]
Data de Publicação: 2016
Outros Autores: Granados, Atila [UNIFESP], Salomao, Reinaldo, Silva, Neusa P. [UNIFESP], Carneiro-Sampaio, Magda, Andrade, Luis Eduardo C.[UNIFESP]
Tipo de documento: Artigo
Idioma: eng
Título da fonte: Repositório Institucional da UNIFESP
dARK ID: ark:/48912/001300000f06b
Texto Completo: http://dx.doi.org/10.1093/rheumatology/kew227
http://repositorio.unifesp.br/handle/11600/51093
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%
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spelling High frequency of immunodeficiency-like states in systemic lupus erythematosus: a cross-sectional study in 300 consecutive patientssystemic lupus erythematosusprimary immunodeficiencyautoimmune diseasesimmunoglobulin deficiencyObjective. 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%P < 0.001), especially low immunoglobulin serum levels. Rigorous testing confirmed only two cases of C2 deficiency carriers among the SLE patients. There were significantly more SLE patients with less than two C4A copies compared with controls. SLE patients had higher frequency of low IgG(2), IgG(3), IgG(4) and IgM levels compared with controls. Patients with low IgG(3) or IgG(4) presented higher frequency of lupus nephropathy. Patients with low IgM had longer disease duration, older age at SLE onset and lower frequency of oral ulcers. Conclusion. An immunodeficiency-like state is present in a sizable fraction of SLE patients. Further studies are warranted to determine the impact of these immunodeficiency states and whether they are a primary condition or are secondary to confounding factors, including SLE itself.Univ Fed Sao Paulo, Escola Paulista Med, Div Rheumatol, Sao Paulo, BrazilUniv Sao Paulo, Escola Paulista Med, Fleury Grp, Div Res & Dev, Sao Paulo, BrazilUniv Sao Paulo, Escola Paulista Med, Div Infect Dis, Sao Paulo, BrazilUniv Sao Paulo, Childrens Hosp, Dept Pediat, Sao Paulo, BrazilUniv Fed Sao Paulo, Escola Paulista Med, Div Rheumatol, Sao Paulo, BrazilWeb of ScienceFAPESP (Fundacao de Amparo a Pesquisa do Estado de Sao Paulo)FAPESP: 2009/53449-0FAPESP: 2008/58238-4Oxford Univ Press2019-07-22T15:46:48Z2019-07-22T15:46:48Z2016info:eu-repo/semantics/articleinfo:eu-repo/semantics/publishedVersion1647-1655http://dx.doi.org/10.1093/rheumatology/kew227Rheumatology. Oxford, v. 55, n. 9, p. 1647-1655, 2016.10.1093/rheumatology/kew227WOS000383849100016.pdf1462-0324http://repositorio.unifesp.br/handle/11600/51093WOS:000383849100016ark:/48912/001300000f06benginfo:eu-repo/semantics/openAccessPerazzio, Sandro F. [UNIFESP]Granados, Atila [UNIFESP]Salomao, ReinaldoSilva, Neusa P. [UNIFESP]Carneiro-Sampaio, MagdaAndrade, Luis Eduardo C.[UNIFESP]reponame:Repositório Institucional da UNIFESPinstname:Universidade Federal de São Paulo (UNIFESP)instacron:UNIFESP2021-10-04T21:26:14Zoai:repositorio.unifesp.br/:11600/51093Repositório InstitucionalPUBhttp://www.repositorio.unifesp.br/oai/requestbiblioteca.csp@unifesp.bropendoar:34652024-12-11T20:14:06.467891Repositório Institucional da UNIFESP - Universidade Federal de São Paulo (UNIFESP)false
dc.title.none.fl_str_mv High frequency of immunodeficiency-like states in systemic lupus erythematosus: a cross-sectional study in 300 consecutive patients
title High frequency of immunodeficiency-like states in systemic lupus erythematosus: a cross-sectional study in 300 consecutive patients
spellingShingle High frequency of immunodeficiency-like states in systemic lupus erythematosus: a cross-sectional study in 300 consecutive patients
Perazzio, Sandro F. [UNIFESP]
systemic lupus erythematosus
primary immunodeficiency
autoimmune diseases
immunoglobulin deficiency
title_short High frequency of immunodeficiency-like states in systemic lupus erythematosus: a cross-sectional study in 300 consecutive patients
title_full High frequency of immunodeficiency-like states in systemic lupus erythematosus: a cross-sectional study in 300 consecutive patients
title_fullStr High frequency of immunodeficiency-like states in systemic lupus erythematosus: a cross-sectional study in 300 consecutive patients
title_full_unstemmed High frequency of immunodeficiency-like states in systemic lupus erythematosus: a cross-sectional study in 300 consecutive patients
title_sort High frequency of immunodeficiency-like states in systemic lupus erythematosus: a cross-sectional study in 300 consecutive patients
author Perazzio, Sandro F. [UNIFESP]
author_facet Perazzio, Sandro F. [UNIFESP]
Granados, Atila [UNIFESP]
Salomao, Reinaldo
Silva, Neusa P. [UNIFESP]
Carneiro-Sampaio, Magda
Andrade, Luis Eduardo C.[UNIFESP]
author_role author
author2 Granados, Atila [UNIFESP]
Salomao, Reinaldo
Silva, Neusa P. [UNIFESP]
Carneiro-Sampaio, Magda
Andrade, Luis Eduardo C.[UNIFESP]
author2_role author
author
author
author
author
dc.contributor.author.fl_str_mv Perazzio, Sandro F. [UNIFESP]
Granados, Atila [UNIFESP]
Salomao, Reinaldo
Silva, Neusa P. [UNIFESP]
Carneiro-Sampaio, Magda
Andrade, Luis Eduardo C.[UNIFESP]
dc.subject.por.fl_str_mv systemic lupus erythematosus
primary immunodeficiency
autoimmune diseases
immunoglobulin deficiency
topic systemic lupus erythematosus
primary immunodeficiency
autoimmune diseases
immunoglobulin deficiency
description 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%
publishDate 2016
dc.date.none.fl_str_mv 2016
2019-07-22T15:46:48Z
2019-07-22T15:46:48Z
dc.type.driver.fl_str_mv info:eu-repo/semantics/article
dc.type.status.fl_str_mv info:eu-repo/semantics/publishedVersion
format article
status_str publishedVersion
dc.identifier.uri.fl_str_mv http://dx.doi.org/10.1093/rheumatology/kew227
Rheumatology. Oxford, v. 55, n. 9, p. 1647-1655, 2016.
10.1093/rheumatology/kew227
WOS000383849100016.pdf
1462-0324
http://repositorio.unifesp.br/handle/11600/51093
WOS:000383849100016
dc.identifier.dark.fl_str_mv ark:/48912/001300000f06b
url http://dx.doi.org/10.1093/rheumatology/kew227
http://repositorio.unifesp.br/handle/11600/51093
identifier_str_mv Rheumatology. Oxford, v. 55, n. 9, p. 1647-1655, 2016.
10.1093/rheumatology/kew227
WOS000383849100016.pdf
1462-0324
WOS:000383849100016
ark:/48912/001300000f06b
dc.language.iso.fl_str_mv eng
language eng
dc.rights.driver.fl_str_mv info:eu-repo/semantics/openAccess
eu_rights_str_mv openAccess
dc.format.none.fl_str_mv 1647-1655
dc.publisher.none.fl_str_mv Oxford Univ Press
publisher.none.fl_str_mv Oxford Univ Press
dc.source.none.fl_str_mv reponame:Repositório Institucional da UNIFESP
instname:Universidade Federal de São Paulo (UNIFESP)
instacron:UNIFESP
instname_str Universidade Federal de São Paulo (UNIFESP)
instacron_str UNIFESP
institution UNIFESP
reponame_str Repositório Institucional da UNIFESP
collection Repositório Institucional da UNIFESP
repository.name.fl_str_mv Repositório Institucional da UNIFESP - Universidade Federal de São Paulo (UNIFESP)
repository.mail.fl_str_mv biblioteca.csp@unifesp.br
_version_ 1818602452704821248

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