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

Abstract Background Patients with autoimmune hepatitis (AIH) may co-present with diagnostic features of primary biliary cholangitis (PBC) or primary sclerosing cholangitis (PSC). Using a national transplant registry, the outcomes of patients with these presentations were compared. Methods The UNOS-STAR registry was used to select a study population of AIH 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 AIH, those with AIH and PBC (AIH-PBC), and those with AIH and PSC (AIH-PSC). Specific endpoints included all-cause mortality, graft failure, and organ-system specific causes of death. Results There were 2048 entries included with 1927 having nonoverlapping AIH, 52 having PSC overlap, and 69 having PBC overlap. Patients with PBC overlap were more likely to have graft failure (aHR 3.53 95% CI 1.73–1.74), mortality secondary to general respiratory causes (aHR 3.55 95% CI 1.22–10.36), mortality secondary to acute respiratory distress syndrome (ARDS) (aHR 18.07 95% CI 3.331–98.74), and recurrent disease (aHR 9.65 95% CI 1.82–51.15). Case incidence rates reflected these findings, expressed in events per 1000 person-years (For the PBC overlap and nonoverlapping AIH cases, respectively. Graft failure: 28.87 events vs. 9.15 events, mortality secondary to general respiratory causes: 12.83 deaths vs. 3.87 deaths, ARDS: 6.42 deaths vs. 0.43 deaths, recurrent disease: 6.42 deaths vs. 1.18 deaths). No increased risks were found in the cohort with PSC overlap. Conclusion Patients with PBC diagnostic overlap may have greater risks for respiratory-induced mortality compared to nonoverlapping AIH. Further investigations are warranted to confirm these results.