Abstract

Background: Outcomes on the liver transplant waitlist can vary by etiology. Our aim is to investigate differences in waitlist mortality of autoimmune hepatitis (AIH), primary biliary cholangitis (PBC), and primary sclerosing cholangitis (PSC) using the United Network for Organ Sharing (UNOS) database. Methods: We identified patients who were listed for liver transplantation from 1987 to 2016 with a primary diagnosis of AIH, PBC, or PSC. We excluded patients with overlap syndromes, acute hepatic necrosis, missing data, and those who were children. The primary outcome was death or removal from the waitlist due to clinical deterioration. We compared waitlist survival using competing risk analysis. Results: Between 1987 and 2016, there were 7412 patients listed for liver transplant due to AIH, 8119 for PBC, and 10,901 for PSC. Patients with AIH were younger, more likely to be diabetic, and had higher listing model for end-stage liver disease (MELD) scores compared to PBC and PSC patients. Patients with PBC and AIH were more likely to be removed from the waitlist due to death or clinical deterioration. On competing risk analysis, AIH patients had a similar risk of being removed from the waitlist compared to those with PBC (subdistribution hazard ratio (SHR) 0.94, 95% CI 0.85–1.03) and higher risk of removal compared to those with PSC (SHR 0.8, 95% CI 0.72 to 0.89). Conclusion: Autoimmune hepatitis carries a similar risk of waitlist removal to PBC and a higher risk than PSC. The etiology of this disparity is not entirely clear and deserves further investigation.

Highlights

  • Autoimmune liver diseases, including autoimmune hepatitis (AIH), primary biliary cholangitis (PBC), and primary sclerosing cholangitis (PSC), account for up to 24% of liver transplants in the United States and Europe [1]

  • Patients listed for PBC have higher waitlist mortality than those with PSC, as shown by one study reviewing the United Network for Organ Sharing (UNOS) database between 2002 and 2013 [4]

  • Another group who reviewed the UNOS database for the years 2002 to 2016 demonstrated that waitlist mortality is lower in patients listed for AIH than those listed for nonalcoholic steatohepatitis (NASH)

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Summary

Introduction

Autoimmune liver diseases, including autoimmune hepatitis (AIH), primary biliary cholangitis (PBC), and primary sclerosing cholangitis (PSC), account for up to 24% of liver transplants in the United States and Europe [1]. Patients listed for PBC have higher waitlist mortality than those with PSC, as shown by one study reviewing the United Network for Organ Sharing (UNOS) database between 2002 and 2013 [4]. Another group who reviewed the UNOS database for the years 2002 to 2016 demonstrated that waitlist mortality is lower in patients listed for AIH than those listed for nonalcoholic steatohepatitis (NASH). Our aim is to investigate differences in waitlist mortality of autoimmune hepatitis (AIH), primary biliary cholangitis (PBC), and primary sclerosing cholangitis (PSC) using the United Network for Organ Sharing (UNOS) database. The etiology of this disparity is not entirely clear and deserves further investigation

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