Department of Microbiology, Immunology and Transplantation, Allergy and Clinical Immunology Research Group, KU Leuven, Leuven, Belgium.
Center for Human Genetics, University Hospitals Leuven, Leuven, Belgium.
Front Immunol. 2023 Sep 19;14:1264482. doi: 10.3389/fimmu.2023.1264482. eCollection 2023.
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.
普通可变免疫缺陷（CVID）相关肝病是一种认识不足且研究甚少的非传染性并发症，缺乏既定的治疗方法。我们描述了一例 CVID 患者，其合并有多器官严重并发症，包括由结节性再生性增生引起的非肝硬化性门静脉高压，导致利尿剂难治性腹水。值得注意的是，利妥昔单抗治疗伴随的免疫性血小板减少症，导致门静脉高压和腹水完全和持续缓解。我们的病例，并结合文献复习，提示利妥昔单抗具有有益的效果，值得进一步研究。
