Rituximab and improved nodular regenerative hyperplasia-associated non-cirrhotic liver disease in common variable immunodeficiency: a case report and literature study

Willem Roosens; Frederik Staels; Sien Van Loo; Stephanie Humblet-Baron; Isabelle Meyts; Isabelle Meyts; Hadewijch De Samblanx; Chris Verslype; Hannah van Malenstein; Hannah van Malenstein; Schalk van der Merwe; Schalk van der Merwe; Wim Laleman; Wim Laleman; Rik Schrijvers

doi:10.3389/fimmu.2023.1264482

Frontiers in Immunology (Sep 2023)

Rituximab and improved nodular regenerative hyperplasia-associated non-cirrhotic liver disease in common variable immunodeficiency: a case report and literature study

Willem Roosens,
Frederik Staels,
Sien Van Loo,
Stephanie Humblet-Baron,
Isabelle Meyts,
Isabelle Meyts,
Hadewijch De Samblanx,
Chris Verslype,
Hannah van Malenstein,
Hannah van Malenstein,
Schalk van der Merwe,
Schalk van der Merwe,
Wim Laleman,
Wim Laleman,
Rik Schrijvers

Affiliations

Willem Roosens: Department of Microbiology, Immunology and Transplantation, Allergy and Clinical Immunology Research Group, KU Leuven, Leuven, Belgium
Frederik Staels: Department of Microbiology, Immunology and Transplantation, Allergy and Clinical Immunology Research Group, KU Leuven, Leuven, Belgium
Sien Van Loo: Center for Human Genetics, University Hospitals Leuven, Leuven, Belgium
Stephanie Humblet-Baron: Department of Microbiology, Immunology and Transplantation, Laboratory of Adaptive Immunology, KU Leuven, Leuven, Belgium
Isabelle Meyts: Department of Microbiology, Immunology and Transplantation Laboratory of Inborn Errors of Immunity, KU Leuven, Leuven, Belgium
Isabelle Meyts: Department of Pediatrics, University Hospitals Leuven, Leuven, Belgium
Hadewijch De Samblanx: Department of Hematology, Ziekenhuis Geel, Geel, Belgium
Chris Verslype: Department of Gastroenterology and Hepatology, Section of Liver and Biliopancreatic disorders, University Hospitals Leuven, Leuven, Belgium
Hannah van Malenstein: Department of Gastroenterology and Hepatology, Section of Liver and Biliopancreatic disorders, University Hospitals Leuven, Leuven, Belgium
Hannah van Malenstein: Department of Chronic Diseases, Metabolism and Aging (CHROMETA), Laboratory of Hepatology, KU Leuven, Leuven, Belgium
Schalk van der Merwe: Department of Gastroenterology and Hepatology, Section of Liver and Biliopancreatic disorders, University Hospitals Leuven, Leuven, Belgium
Schalk van der Merwe: Department of Chronic Diseases, Metabolism and Aging (CHROMETA), Laboratory of Hepatology, KU Leuven, Leuven, Belgium
Wim Laleman: Department of Chronic Diseases, Metabolism and Aging (CHROMETA), Laboratory of Hepatology, KU Leuven, Leuven, Belgium
Wim Laleman: Medizinische Klinik B, Universitätsklinikum Münster, Münster University, Münster, Germany
Rik Schrijvers: Department of Microbiology, Immunology and Transplantation, Allergy and Clinical Immunology Research Group, KU Leuven, Leuven, Belgium

DOI: https://doi.org/10.3389/fimmu.2023.1264482
Journal volume & issue: Vol. 14

Abstract

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Common variable immunodeficiency (CVID) associated liver disease is an underrecognized and poorly studied non-infectious complication that lacks an established treatment. We describe a CVID patient with severe multiorgan complications, including non-cirrhotic portal hypertension secondary to nodular regenerative hyperplasia leading to diuretic-refractory ascites. Remarkably, treatment with rituximab, administered for concomitant immune thrombocytopenia, resulted in the complete and sustained resolution of portal hypertension and ascites. Our case, complemented with a literature review, suggests a beneficial effect of rituximab that warrants further research.

Published in Frontiers in Immunology

ISSN: 1664-3224 (Online)
Publisher: Frontiers Media S.A.
Country of publisher: Switzerland
LCC subjects: Medicine: Internal medicine: Specialties of internal medicine: Immunologic diseases. Allergy
Website: http://journal.frontiersin.org/journal/immunology

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