Autoimmune Hepatitis in a Patient With Common Variable Immunodeficiency

ABSTRACT 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 wi...

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Bibliographic Details
Published inACG case reports journal Vol. 8; no. 3; p. e00547
Main Authors Myneedu, Kanchana, Chavez, Luis O., Sussman, Norman L., Michael, Majd, Padilla, Angelica, Zuckerman, Marc J.
Format Journal Article
LanguageEnglish
Published Maryland, MD Wolters Kluwer 01.03.2021
Subjects
Case Report
Liver
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Summary:ABSTRACT 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.
ISSN:2326-3253
2326-3253
DOI:10.14309/crj.0000000000000547