Risk of severe disease and mortality of COVID-19 in patients with Budd-Chiari syndrome: A population-based matched cohort study.

BACKGROUND & AIMS: Budd-Chiari syndrome (BCS) is a rare and potentially life-threatening disorder characterized by obstruction of the hepatic outflow tract. It is unknown whether patients with BCS represent a high risk for severe disease and mortality from coronavirus disease 2019 (COVID-19). Thus, we aimed to assess hospitalization rates, severe disease, all-cause mortality, intensive care unit (ICU) requirement and acute kidney injury (AKI) from COVID-19 diagnoses.

METHODS & RESULTS: We identified 467 patients with BCS with COVID-19, 96 427 non-chronic liver disease (CLD) and 9652 non-BCS CLD. The BCS and non-CLD cohorts (n = 467 each) and BCS and non-BCS CLD (n = 440 each) were well balanced after propensity matching. When compared to the non-CLD cohort, the BCS group had a higher risk of all-cause mortality (5.1% vs. 2.4%, HR 2.18; 95% CI, 1.08-4.40), severe disease (6.0% vs. 2.4%, HR 2.20; 95% CI, 1.09-4.43), hospitalization (24.6% vs. 13.1%, HR 1.77; 95% CI, 1.30-2.42) and AKI (7.9% vs. 2.8%, HR 2.57; 95% CI, 1.37-4.85), but no significant differences in ICU requirements (2.4% vs. 2.1%, HR 0.75; 95% CI, 0.27-2.08) at 60-days time points. When compared to the non-BCS CLD cohort, the BCS group had a higher risk of all-cause mortality (3.6% vs. 2.5%, HR 3.94; 95% CI, 1.31-11.79), hospitalization (29.8% vs. 21.6%, HR 1.43; 95% CI, 1.09-1.86), but differences in ICU requirements (HR 0.90 (0.38-2.12)), AKI (HR 1.41 (0.86-2.30)) or severe disease (HR 1.92 (0.99-3.71)) did not reach statistical significance at 60-day follow up.

CONCLUSION: In conclusion, COVID-19 infection in patients with BCS is associated with poor outcomes. Patients with BCS infected with COVID-19 carry a significantly higher risk of hospitalization and all-cause mortality and a possible effect on severe disease and AKI compared with COVID-19 patients without CLD or with non-BCS-CLD.