Hepatic Manifestations in Common Variable Immunodeficiency Associated With Mortality and Worse Hospital Outcomes in Nationwide Analysis Using National Readmission Database

Abstract

Background: Liver disease is associated with increased mortality in patients with common variable immunodeficiency (CVID), yet we lack population-level data on its impact on CVID-associated hospitalizations in the United States.

Objective: To conduct a nationwide analysis evaluating the prevalence, spectrum, clinical correlates, and outcomes of hepatic manifestations among admitted adults with CVID.

Methods: We performed a retrospective cross-sectional analysis using the National Readmission Database, part of the Healthcare Cost and Utilization Project by the Agency for Healthcare Research and Quality. International Classification of Diseases, Tenth Revision codes were used to assess hepatic complications, comorbidities, risk factors, and outcomes in CVID-associated admissions with liver involvement.

Results: Of 181,288 CVID-related index hospitalizations, 10% (18,823) had a codiagnosed hepatic complication, specifically hepatic steatosis (38%), cirrhosis (24%), nonalcoholic fatty liver disease (17%), portal hypertension (15%), and nodular regenerative hyperplasia (14%). CVID-associated admissions with hepatic disease had longer median lengths of stay (7 vs 5 days, P < .0001), higher in-hospital mortality (11% vs 4%, P < .0001), and higher median hospital charges ($68,114 vs $44,757, P < .0001). They also had a higher prevalence of hypertension, chronic kidney disease, diabetes, obesity, and autoimmune cytopenia (P < .0001 for all). Alcohol use, hepatitis C, and hepatitis B were strong predictors of hepatic manifestations in CVID admissions (P < .0001). Patients with CVID with nodular regenerative hyperplasia (hazard ratio, 1.3; 95% CI, 1.2-1.5; P < .0001) and portal hypertension (hazard ratio, 1.4; 95% CI, 1.2-1.5; P < .0001) had lower survival.

Conclusions: CVID-associated admissions with liver disease, particularly those with nodular regenerative hyperplasia and portal hypertension, were longer, more costly, and associated with increased mortality. Further studies are needed to improve early detection and long-term outcomes.

Keywords: Common variable immunodeficiency; Hepatic manifestations; Hospital readmission; In-hospital mortality; Liver disease; Nationwide Readmissions Database; Nodular regenerative hyperplasia; Portal hypertension; Primary immunodeficiency.