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1316. Uncommon Presentations of Common Variable Immunodeficiency

BACKGROUND: Common Variable Immunodeficiency (CVID) is a primary immunodeficiency disorder which affects B lymphocyte function and differentiation causing decreased levels of Immunoglobulin G (IgG), Immunoglobulin A (IgA) and Immunoglobulin M (IgM).(1) The objective of this study is to highlight how...

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Autores principales: Alok, Akankcha, Greene, John, Aslam, Sadaf
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Oxford University Press 2021
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8644467/
http://dx.doi.org/10.1093/ofid/ofab466.1508
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author Alok, Akankcha
Greene, John
Aslam, Sadaf
author_facet Alok, Akankcha
Greene, John
Aslam, Sadaf
author_sort Alok, Akankcha
collection PubMed
description BACKGROUND: Common Variable Immunodeficiency (CVID) is a primary immunodeficiency disorder which affects B lymphocyte function and differentiation causing decreased levels of Immunoglobulin G (IgG), Immunoglobulin A (IgA) and Immunoglobulin M (IgM).(1) The objective of this study is to highlight how hypogammaglobulinemia can lead to respiratory infections with microbes that are lesser known in the background of CVID with the help of a two-case series. METHODS: Medical records of two patients with CVID were reviewed who were found to have mycobacterium avium-complex intracellulare and streptococcus agalactiae lung infections respectively. RESULTS: Decreased IgG in CVID means reduced antibody production, low IgA leads to mucosal inflammation and increased susceptibility to respiratory infections(2) and lower IgM memory B-cells causes infections with encapsulated microorganisms.(3 )Table 1 highlights the various respiratory infections and their etiologies that have been reported with CVID, the most common being encapsulated organisms like Haemophilus influenza, Streptococcus pneumonia, Neisseria meningitidis along with enterovirus. Table 2 demonstrates our findings. In the first case we have reported a patient with mycobacterium avium-complex intracellulare (MAC-I). This could be because of hypogammaglobulinemia, decreased B and T-cell interaction and reduced T-cell signaling caused by CVID.(4 ) Although, mycobacterium tuberculosis, simiae and hominis lung infections and mycobacterium bovis systemic infections have been reported before, MAC-I is relatively rare in CVID.(5) In our second case, the patient developed streptococcus agalactiae or Group-B streptococcus (GBS) empyema. Most cases of GBS have been reported in pregnant women and infants. Infections with other encapsulated organisms have been reported in CVID but GBS empyema is less frequent and can happen due to decreased bacteria-specific CD4 cells, microbial translocation and hypogammaglobulinemia.(6 .) Table 1. Respiratory Infections reported in CVID along with their etiologies. [Image: see text] [Image: see text] [Image: see text] Figure 1. CT image of MAC-I infection. CONCLUSION: We encountered two unique cases of CVID with rare infectious etiologies. The cases are intended to create an awareness and vigilance regarding CVID induced hypogammaglobulinemia which can cause respiratory infections with lesser known pathogens where antibodies may be important. [Image: see text] DISCLOSURES: All Authors: No reported disclosures
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spelling pubmed-86444672021-12-06 1316. Uncommon Presentations of Common Variable Immunodeficiency Alok, Akankcha Greene, John Aslam, Sadaf Open Forum Infect Dis Poster Abstracts BACKGROUND: Common Variable Immunodeficiency (CVID) is a primary immunodeficiency disorder which affects B lymphocyte function and differentiation causing decreased levels of Immunoglobulin G (IgG), Immunoglobulin A (IgA) and Immunoglobulin M (IgM).(1) The objective of this study is to highlight how hypogammaglobulinemia can lead to respiratory infections with microbes that are lesser known in the background of CVID with the help of a two-case series. METHODS: Medical records of two patients with CVID were reviewed who were found to have mycobacterium avium-complex intracellulare and streptococcus agalactiae lung infections respectively. RESULTS: Decreased IgG in CVID means reduced antibody production, low IgA leads to mucosal inflammation and increased susceptibility to respiratory infections(2) and lower IgM memory B-cells causes infections with encapsulated microorganisms.(3 )Table 1 highlights the various respiratory infections and their etiologies that have been reported with CVID, the most common being encapsulated organisms like Haemophilus influenza, Streptococcus pneumonia, Neisseria meningitidis along with enterovirus. Table 2 demonstrates our findings. In the first case we have reported a patient with mycobacterium avium-complex intracellulare (MAC-I). This could be because of hypogammaglobulinemia, decreased B and T-cell interaction and reduced T-cell signaling caused by CVID.(4 ) Although, mycobacterium tuberculosis, simiae and hominis lung infections and mycobacterium bovis systemic infections have been reported before, MAC-I is relatively rare in CVID.(5) In our second case, the patient developed streptococcus agalactiae or Group-B streptococcus (GBS) empyema. Most cases of GBS have been reported in pregnant women and infants. Infections with other encapsulated organisms have been reported in CVID but GBS empyema is less frequent and can happen due to decreased bacteria-specific CD4 cells, microbial translocation and hypogammaglobulinemia.(6 .) Table 1. Respiratory Infections reported in CVID along with their etiologies. [Image: see text] [Image: see text] [Image: see text] Figure 1. CT image of MAC-I infection. CONCLUSION: We encountered two unique cases of CVID with rare infectious etiologies. The cases are intended to create an awareness and vigilance regarding CVID induced hypogammaglobulinemia which can cause respiratory infections with lesser known pathogens where antibodies may be important. [Image: see text] DISCLOSURES: All Authors: No reported disclosures Oxford University Press 2021-12-04 /pmc/articles/PMC8644467/ http://dx.doi.org/10.1093/ofid/ofab466.1508 Text en © The Author(s) 2021. Published by Oxford University Press on behalf of Infectious Diseases Society of America. https://creativecommons.org/licenses/by/4.0/This is an Open Access article distributed under the terms of the Creative Commons Attribution License (https://creativecommons.org/licenses/by/4.0/), which permits unrestricted reuse, distribution, and reproduction in any medium, provided the original work is properly cited.
spellingShingle Poster Abstracts
Alok, Akankcha
Greene, John
Aslam, Sadaf
1316. Uncommon Presentations of Common Variable Immunodeficiency
title 1316. Uncommon Presentations of Common Variable Immunodeficiency
title_full 1316. Uncommon Presentations of Common Variable Immunodeficiency
title_fullStr 1316. Uncommon Presentations of Common Variable Immunodeficiency
title_full_unstemmed 1316. Uncommon Presentations of Common Variable Immunodeficiency
title_short 1316. Uncommon Presentations of Common Variable Immunodeficiency
title_sort 1316. uncommon presentations of common variable immunodeficiency
topic Poster Abstracts
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8644467/
http://dx.doi.org/10.1093/ofid/ofab466.1508
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