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

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

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...

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Bibliographic Details
Main Authors: Willem Roosens, Frederik Staels, Sien Van Loo, Stephanie Humblet-Baron, Isabelle Meyts, Hadewijch De Samblanx, Chris Verslype, Hannah van Malenstein, Schalk van der Merwe, Wim Laleman, Rik Schrijvers
Format: Article
Language:English
Published: Frontiers Media S.A. 2023-09-01
Series:Frontiers in Immunology
Subjects:
non-infectious complications
nodular regenerative hyperplasia
non-cirrhotic portal hypertension
rituximab
common variable immune deficiency (CVID)
primary immunodeficiency
Online Access:https://www.frontiersin.org/articles/10.3389/fimmu.2023.1264482/full
Description
Summary: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.
ISSN:1664-3224

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