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

Frontiers in Immunology

Immunology

Sien Van Loo¹  Wim Laleman²  Chris Verslype³  Hannah van Malenstein⁴  Schalk van der Merwe⁴  Hadewijch De Samblanx⁵  Isabelle Meyts⁶  Willem Roosens⁷  Rik Schrijvers⁷  Frederik Staels⁷  Stephanie Humblet-Baron⁸ 

[1] Center for Human Genetics, University Hospitals Leuven, Leuven, Belgium;Department of Chronic Diseases, Metabolism and Aging (CHROMETA), Laboratory of Hepatology, KU Leuven, Leuven, Belgium;Medizinische Klinik B, Universitätsklinikum Münster, Münster University, Münster, Germany;Department of Gastroenterology and Hepatology, Section of Liver and Biliopancreatic disorders, University Hospitals Leuven, Leuven, Belgium;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;Department of Hematology, Ziekenhuis Geel, Geel, Belgium;Department of Microbiology, Immunology and Transplantation Laboratory of Inborn Errors of Immunity, KU Leuven, Leuven, Belgium;Department of Pediatrics, University Hospitals Leuven, Leuven, Belgium;Department of Microbiology, Immunology and Transplantation, Allergy and Clinical Immunology Research Group, KU Leuven, Leuven, Belgium;Department of Microbiology, Immunology and Transplantation, Laboratory of Adaptive Immunology, KU Leuven, Leuven, Belgium;

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

DOI  :  10.3389/fimmu.2023.1264482

 received in 2023-07-20, accepted in 2023-08-29,  发布年份 2023

来源: Frontiers

【 摘 要 】

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.

【 授权许可】

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