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

common variable immune deficiency (CVID) inborn errors of immunity nodular regenerative hyperplasia non-cirrhotic portal hypertension non-infectious complications primary immunodeficiency rituximab

Journal

Frontiers in immunology
ISSN: 1664-3224
Titre abrégé: Front Immunol
Pays: Switzerland
ID NLM: 101560960

Informations de publication

Date de publication:
2023
Historique:
received: 20 07 2023
accepted: 29 08 2023
medline: 1 11 2023
pubmed: 5 10 2023
entrez: 5 10 2023
Statut: epublish

Résumé

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.

Identifiants

pubmed: 37795099
doi: 10.3389/fimmu.2023.1264482
pmc: PMC10546204
doi:

Substances chimiques

Rituximab 4F4X42SYQ6

Types de publication

Review Case Reports Research Support, Non-U.S. Gov't

Langues

eng

Sous-ensembles de citation

IM

Pagination

1264482

Informations de copyright

Copyright © 2023 Roosens, Staels, Van Loo, Humblet-Baron, Meyts, De Samblanx, Verslype, van Malenstein, van der Merwe, Laleman and Schrijvers.

Déclaration de conflit d'intérêts

The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest. The author(s) declared that they were an editorial board member of Frontiers, at the time of submission. This had no impact on the peer review process and the final decision.

Références

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Auteurs

Willem Roosens (W)

Department of Microbiology, Immunology and Transplantation, Allergy and Clinical Immunology Research Group, KU Leuven, Leuven, Belgium.

Frederik Staels (F)

Department of Microbiology, Immunology and Transplantation, Allergy and Clinical Immunology Research Group, KU Leuven, Leuven, Belgium.

Sien Van Loo (S)

Center for Human Genetics, University Hospitals Leuven, Leuven, Belgium.

Stephanie Humblet-Baron (S)

Department of Microbiology, Immunology and Transplantation, Laboratory of Adaptive Immunology, KU Leuven, Leuven, Belgium.

Isabelle Meyts (I)

Department of Microbiology, Immunology and Transplantation Laboratory of Inborn Errors of Immunity, KU Leuven, Leuven, Belgium.
Department of Pediatrics, University Hospitals Leuven, Leuven, Belgium.

Hadewijch De Samblanx (H)

Department of Hematology, Ziekenhuis Geel, Geel, Belgium.

Chris Verslype (C)

Department of Gastroenterology and Hepatology, Section of Liver and Biliopancreatic disorders, University Hospitals Leuven, Leuven, Belgium.

Hannah van Malenstein (H)

Department of Gastroenterology and Hepatology, Section of Liver and Biliopancreatic disorders, University Hospitals Leuven, Leuven, Belgium.
Department of Chronic Diseases, Metabolism and Aging (CHROMETA), Laboratory of Hepatology, KU Leuven, Leuven, Belgium.

Schalk van der Merwe (S)

Department of Gastroenterology and Hepatology, Section of Liver and Biliopancreatic disorders, University Hospitals Leuven, Leuven, Belgium.
Department of Chronic Diseases, Metabolism and Aging (CHROMETA), Laboratory of Hepatology, KU Leuven, Leuven, Belgium.

Wim Laleman (W)

Department of Chronic Diseases, Metabolism and Aging (CHROMETA), Laboratory of Hepatology, KU Leuven, Leuven, Belgium.
Medizinische Klinik B, Universitätsklinikum Münster, Münster University, Münster, Germany.

Rik Schrijvers (R)

Department of Microbiology, Immunology and Transplantation, Allergy and Clinical Immunology Research Group, KU Leuven, Leuven, Belgium.

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