Liver disease is common in patients with common variable immunodeficiency and predicts mortality in the presence of cirrhosis or portal hypertension.

Common variable immunodeficiency (CVID) is characterized by impaired B-cell function with hypogammaglobulinemia, without profound T-cell dysfunction. CVID affects 1 of 25,000 to 50,000 of the population with equal sex preponderance. CVID affects multiple organ systems and is associated with recurrent infections, autoimmunity, granulomas, and malignancy1 (summarized in Table I). Abnormal liver function tests (LFTs) are associated with worse outcomes in CVID,2 but there is limited knowledge about which liver-related factors affect outcome; hence, we present the largest characterization of liver pathology in CVID, detailing aspects of liver disease associated with mortality.