Prevalence and Impact of Chronic Liver Disease in Adult Patients Admitted With Cystic Fibrosis

Cystic fibrosis (CF) is a genetic, multisystem disorder characterized by progressive lung disease driven by mucus obstruction, chronic inflammation, and recurrent infections. Although chronic liver disease (CLD) is a recognized complication of CF, its prevalence and clinical impact among hospitalized adults with CF have not been well characterized using large-scale national databases.

We utilized the National Inpatient Sample (NIS) database to identify adults aged ≥18 years hospitalized between 2015 and 2019 with a diagnosis of CF. Patients were stratified based on the presence or absence of CLD. International Classification of Diseases (ICD) codes were used to identify comorbidities, including congestive heart failure, prior myocardial infarction, atrial fibrillation, obesity, chronic kidney disease, and end-stage renal disease. Multivariable regression analyses were performed to evaluate in-hospital mortality, length of stay, hospital costs, and in-hospital complications, including sepsis, pneumonia, acute kidney injury (AKI), and the need for intubation, tracheostomy, and gastrostomy.

Among an estimated 90,675 hospitalized adults with CF, 11.68% had concomitant CLD. In multivariable analyses, patients with CLD had significantly higher in-hospital mortality compared with those without CLD (2.50% vs. 1.24%, P<0.001). The CLD group also experienced longer hospital stays (10.4 vs. 8.6 days, P<0.001) and higher hospitalization costs ($38,880 vs. $31,246, P=0.019). The coexistence of CF and CLD was associated with a significantly increased risk of in-hospital complications, including sepsis (6.47% vs. 4.20%, P<0.001), AKI (17.13% vs. 11.79%, P<0.001), and malnutrition (42.63% vs. 31.49%, P=0.002). There were no significant differences between groups in the rates of pneumonia (21.00% vs. 20.57%, P=0.596), need for intubation (2.74% vs. 1.92%, P=0.139), tracheostomy (0.69% vs. 0.54%, P=0.072), or gastrostomy (0.89% vs. 0.66%, P=0.499).

CLD is common among adults hospitalized with CF and is independently associated with higher in-hospital mortality, longer length of stay, and increased hospital costs. Additionally, CLD confers a significantly higher risk of sepsis, AKI, and malnutrition. These findings underscore the importance of early recognition and proactive management of liver disease in hospitalized patients with CF.