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

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

Title: 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;
Associação 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 Paulo
Author Errante, Paolo Ruggero Autor UNIFESP Google Scholar
Perazzio, Sandro Felix Google Scholar
Frazao, Josias Brito Autor UNIFESP Google Scholar
da Silva, Neusa Pereira Google Scholar
Coelho Andrade, Luis Eduardo Autor UNIFESP Google Scholar
Abstract Primary immunodeficiency disorders (PID) represent a heterogeneous group of diseases resulting from inherited defects in the development, maturation and normal function of immune cells

thus, 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 susceptibility

however 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.
Keywords Autoimmune Disease
Primary Immunodeficiency
Systemic Lupus Erythematosus
Antibodies DeficiencyChronic Granulomatous-Disease
Autoimmune Lymphoproliferative Syndrome
Common Variable Immunodeficiency
Selective Igm Deficiency
Wiskott-Aldrich-Syndrome
Mannose-Binding Lectin
Candidiasis-Ectodermal Dystrophy
22q11.2 Deletion Syndrome
Subclass Deficiency
Patient
Language Portuguese
Date 2016
Published in Revista Brasileira De Reumatologia. New york, v. 56, n. 1, p. 58-68, 2016.
ISSN 0482-5004 (Sherpa/Romeo, impact factor)
Publisher Elsevier science inc
Extent 58-68
Origin https://doi.org/10.1016/j.rbr.2015.03.002
Access rights Open access Open Access
Type Revisão
Web of Science ID WOS:000374895300010
SciELO ID S0482-50042016000100058 (statistics in SciELO)
URI http://repositorio.unifesp.br/handle/11600/49533

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