Autoimmune Hepatitis in a Patient With Common Variable Immunodeficiency

Common variable immunodeficiency (CVID) is characterized by defective immunoglobulin synthesis because of impaired B-cell function. Liver abnormalities including autoimmune hepatitis (AIH) have been described in up to 10% of patients. We report a 27-year-old woman with CVID who presented with liver...

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Autores principales: Myneedu, Kanchana, Chavez, Luis O., Sussman, Norman L., Michael, Majd, Padilla, Angelica, Zuckerman, Marc J.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Wolters Kluwer 2021
Materias:
Case Report
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8443823/
https://www.ncbi.nlm.nih.gov/pubmed/34549051
http://dx.doi.org/10.14309/crj.0000000000000547
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author Myneedu, Kanchana
Chavez, Luis O.
Sussman, Norman L.
Michael, Majd
Padilla, Angelica
Zuckerman, Marc J.
author_facet Myneedu, Kanchana
Chavez, Luis O.
Sussman, Norman L.
Michael, Majd
Padilla, Angelica
Zuckerman, Marc J.
author_sort Myneedu, Kanchana
collection PubMed
description Common variable immunodeficiency (CVID) is characterized by defective immunoglobulin synthesis because of impaired B-cell function. Liver abnormalities including autoimmune hepatitis (AIH) have been described in up to 10% of patients. We report a 27-year-old woman with CVID who presented with liver dysfunction secondary to AIH. AIH is both uncommon and challenging diagnostically in patients with CVID because they have low IgG levels and often have low or undetectable autoantibody levels. Liver biopsy and response to therapy play an important role in establishing the diagnosis. Corticosteroids are the mainstay of therapy, with or without immune modulators.
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spelling pubmed-84438232021-09-20 Autoimmune Hepatitis in a Patient With Common Variable Immunodeficiency Myneedu, Kanchana Chavez, Luis O. Sussman, Norman L. Michael, Majd Padilla, Angelica Zuckerman, Marc J. ACG Case Rep J Case Report Common variable immunodeficiency (CVID) is characterized by defective immunoglobulin synthesis because of impaired B-cell function. Liver abnormalities including autoimmune hepatitis (AIH) have been described in up to 10% of patients. We report a 27-year-old woman with CVID who presented with liver dysfunction secondary to AIH. AIH is both uncommon and challenging diagnostically in patients with CVID because they have low IgG levels and often have low or undetectable autoantibody levels. Liver biopsy and response to therapy play an important role in establishing the diagnosis. Corticosteroids are the mainstay of therapy, with or without immune modulators. Wolters Kluwer 2021-03-17 /pmc/articles/PMC8443823/ /pubmed/34549051 http://dx.doi.org/10.14309/crj.0000000000000547 Text en © 2021 The Author(s). Published by Wolters Kluwer Health, Inc. on behalf of The American College of Gastroenterology. https://creativecommons.org/licenses/by-nc-nd/4.0/This is an open access article distributed under the terms of the Creative Commons Attribution-Non Commercial-No Derivatives License 4.0 (CCBY-NC-ND) (https://creativecommons.org/licenses/by-nc-nd/4.0/) , where it is permissible to download and share the work provided it is properly cited. The work cannot be changed in any way or used commercially without permission from the journal.
spellingShingle Case Report
Myneedu, Kanchana
Chavez, Luis O.
Sussman, Norman L.
Michael, Majd
Padilla, Angelica
Zuckerman, Marc J.
Autoimmune Hepatitis in a Patient With Common Variable Immunodeficiency
title Autoimmune Hepatitis in a Patient With Common Variable Immunodeficiency
title_full Autoimmune Hepatitis in a Patient With Common Variable Immunodeficiency
title_fullStr Autoimmune Hepatitis in a Patient With Common Variable Immunodeficiency
title_full_unstemmed Autoimmune Hepatitis in a Patient With Common Variable Immunodeficiency
title_short Autoimmune Hepatitis in a Patient With Common Variable Immunodeficiency
title_sort autoimmune hepatitis in a patient with common variable immunodeficiency
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8443823/
https://www.ncbi.nlm.nih.gov/pubmed/34549051
http://dx.doi.org/10.14309/crj.0000000000000547
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