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The differences in post-liver transplant outcomes of patients with autoimmune hepatitis who present with overlapping autoimmune liver diseases

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Abstract

Background

Patients with autoimmune hepatitis (AIH) may co-present with features of primary biliary cholangitis (PBC) or primary sclerosing cholangitis (PSC). Using a national transplant registry, the outcomes of patients with these autoimmune liver conditions were compared.

Methods

The UNOS-STAR registry was used to select a study population of AIH, PSC, and PBC liver transplant (LT) patients. Living and multi-organ transplant cases were excluded. Using the UNOS-registered diagnoses, the study population was subdivided into those with nonoverlapping autoimmune liver diseases and those with overlapping forms (e.g., AIH–PBC). Outcomes were compared, using endpoints such as all-cause mortality, graft failure, and organ-system specific causes of death.

Results

The main analysis featured 2048 entries, with 1927 entries having nonoverlapping AIH, 52 entries having PSC overlap, and 69 entries having PBC overlap. Patients with PBC overlap were more likely to have graft failure (adjusted hazard ratio [aHR] 3.46 95% CI 1.70–7.05), mortality secondary to respiratory causes (aHR 3.57 95% CI 1.23–10.43), and mortality secondary to recurrent disease (aHR 9.53 95% CI 1.85–49.09). Case incidence rates reflected these findings, expressed in events per 1000 person-years. For patients with PBC overlap and nonoverlapping AIH cases, respectively. Graft failure: 28.87 events vs. 9.42 events, mortality secondary to respiratory causes: 12.83 deaths vs. 3.77 deaths, mortality secondary to recurrent disease: 6.42 deaths vs. 1.26 deaths. Those with AIH–PSC overlap experienced a higher risk of death from graft infection (aHR 10.43 95% CI 1.08–100.37; case-incidence rate: 3.89 vs. 0.31 mortalities per 1000 person-years). Supplementary analysis showed similar findings, in which overlapping autoimmune conditions were associated with higher adverse outcome rates.

Conclusion

Patients with AIH-PBC overlap have higher risk of mortality due to recurrent liver disease and respiratory causes, and patients with AIH-PSC overlap have higher risk of mortality due to graft infection. While further prospective studies are needed to clarify the underlying mechanisms related to these findings, our study characterizes the prognostic implications of AIH overlap on post-LT mortality and graft failure risks.

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Funding

This study was supported by the NIH grant NIDDK T32 DK067872-17 (to David Uihwan Lee MD).

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Authors and Affiliations

  1. Division of Gastroenterology and Hepatology, University of Maryland School of Medicine, 22 S. Greene St N3W50, Baltimore, MD, 21201, USA

    David Uihwan Lee, Samantha Menegas & Nathalie Helen Urrunaga

  2. Department of Medicine, Tufts University School of Medicine, Washington St, Boston, MA, 02111, USA

    Reid Ponder, Kijung Lee, Gregory Hongyuan Fan, Harrison Chou, Keeseok Lee & David Jeffrey Hastie

  3. Department of Medicine, University of Missouri–Kansas City School of Medicine, Boston, MA, USA

    Daniel Jung

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  1. David Uihwan Lee
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Correspondence to David Uihwan Lee.

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David Uihwan Lee, Reid Ponder, Kijung Lee, Samantha Menegas, Gregory Hongyuan Fan, Harrison Chou, Daniel Jung, Keeseok Lee, David Jeffrey Hastie, Nathalie Helen Urrunaga of this manuscript certify they share no affiliation or involvement with any organization or entity with any financial interest or non-financial interest in the subject matter or materials discussed in this manuscript.

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Lee, D.U., Ponder, R., Lee, K. et al. The differences in post-liver transplant outcomes of patients with autoimmune hepatitis who present with overlapping autoimmune liver diseases. Hepatol Int 17, 720–734 (2023). https://doi.org/10.1007/s12072-022-10468-8

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  • DOI: https://doi.org/10.1007/s12072-022-10468-8

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