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

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

JOURNAL=Frontiers in Immunology

VOLUME=14

YEAR=2023

URL=https://www.frontiersin.org/journals/immunology/articles/10.3389/fimmu.2023.1264482

DOI=10.3389/fimmu.2023.1264482

ISSN=1664-3224

ABSTRACT=<p>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.</p>