Primary immunodeficiency association with systemic lupus erythematosus: review of literature and lessons learned by the rheumatology division of a tertiary university hospital at sao paulo, brazil

Página do item simplificado
dc.contributor.authorErrante, Paolo Ruggero [UNIFESP]
dc.contributor.authorPerazzio, Sandro Felix
dc.contributor.authorFrazao, Josias Brito [UNIFESP]
dc.contributor.authorda Silva, Neusa Pereira
dc.contributor.authorCoelho Andrade, Luis Eduardo [UNIFESP]
dc.date.accessioned2019-01-21T10:30:00Z
dc.date.available2019-01-21T10:30:00Z
dc.date.issued2016
dc.description.abstractPrimary immunodeficiency disorders (PID) represent a heterogeneous group of diseases resulting from inherited defects in the development, maturation and normal function of immune cellsen
dc.description.abstractthus, make individuals susceptible to recurrent infections, allergy, autoimmunity, and malignancies. In this retrospective study, autoimmune diseases (AIDs), in special systemic lupus erythematosus (SLE) which arose associated to the course of PID, are described. Classically, the literature describes three groups of PID associated with SLE: (1) deficiency of Complement pathway components, (2) defects in immunoglobulin synthesis, and (3) chronic granulomatous disease (CGD). Currently, other PID have been described with clinical manifestation of SLE, such as Wiskott-Aldrich syndrome (WAS), autoimmune polyendocrinopathy candidiasis ectodermal dystrophy (APECED), autoimmune lymphoproliferative syndrome (ALPS) and idiopathic CD4(+) lymphocytopenia. Also we present findings from an adult cohort from the outpatient clinic of the Rheumatology Division of Universidade Federal de Sao Paulo. The PID manifestations found by our study group were considered mild in terms of severity of infections and mortality in early life. Thus, it is possible that some immunodeficiency states are compatible with survival regarding infectious susceptibilityen
dc.description.abstracthowever these states might represent a strong predisposing factor for the development of immune disorders like those observed in SLE. (C) 2015 Elsevier Editora Ltda. All rights reserved.en
dc.description.affiliationDepartamento de Imunologia, Instituto de Ciências Biomédicas, Universidade de São Paulo (USP), São Paulo, SP, Brasil
dc.description.affiliationDepartamento de Medicina da Universidade Federal de São Paulo (Unifesp), São Paulo, SP, Brasil
dc.description.affiliationUnifespDepartamento de Medicina da Universidade Federal de São Paulo (Unifesp), São Paulo, SP, Brasil
dc.description.sourceWeb of Science
dc.format.extent58-68
dc.identifierhttps://doi.org/10.1016/j.rbr.2015.03.002
dc.identifier.citationRevista Brasileira De Reumatologia. New york, v. 56, n. 1, p. 58-68, 2016.
dc.identifier.doi10.1016/j.rbr.2015.03.002
dc.identifier.fileS0482-50042016000100058.pdf
dc.identifier.issn0482-5004
dc.identifier.scieloS0482-50042016000100058
dc.identifier.urihttp://repositorio.unifesp.br/handle/11600/49533
dc.identifier.wosWOS:000374895300010
dc.language.isopor
dc.publisherElsevier science inc
dc.relation.ispartofRevista Brasileira De Reumatologia
dc.rightsinfo:eu-repo/semantics/openAccess
dc.subjectAutoimmune Diseaseen
dc.subjectPrimary Immunodeficiencyen
dc.subjectSystemic Lupus Erythematosusen
dc.subjectAntibodies DeficiencyChronic Granulomatous-Diseaseen
dc.subjectAutoimmune Lymphoproliferative Syndromeen
dc.subjectCommon Variable Immunodeficiencyen
dc.subjectSelective Igm Deficiencyen
dc.subjectWiskott-Aldrich-Syndromeen
dc.subjectMannose-Binding Lectinen
dc.subjectCandidiasis-Ectodermal Dystrophyen
dc.subject22q11.2 Deletion Syndromeen
dc.subjectSubclass Deficiencyen
dc.subjectPatienten
dc.titlePrimary immunodeficiency association with systemic lupus erythematosus: review of literature and lessons learned by the rheumatology division of a tertiary university hospital at sao paulo, brazilen
dc.titleAssociação de imunodeficiência primária com lúpus eritematoso sistêmico: revisão da literatura e as lições aprendidas pela Divisão de Reumatologia de um hospital universitário terciário em São Paulopt
dc.typeinfo:eu-repo/semantics/review
Arquivos
Coleções
EPM - Artigos