Author information

¹Medical School, University of Western Australia, Nedlands 6009, Western Australia, Australia.

²Department of Hepatology, Sir Charles Gairdner Hospital, Nedlands 6009, Western Australia, Australia. duji.jayabalan@uwa.edu.au.

³Department of Hepatology, Sir Charles Gairdner Hospital, Nedlands 6009, Western Australia, Australia.

⁴Department of Anatomical Pathology, PathWest Laboratory Medicine, Nedlands 6009, Western Australia, Australia.

⁵Department of Gastroenterology and Hepatology, Royal Perth Hospital, Perth 6000, Western Australia, Australia.

⁶Department of Gastroenterology and Hepatology, Fiona Stanley Hospital, Murdoch 6150, Western Australia, Australia.

Abstract

Background: Survival in patients with autoimmune liver disease overlap syndromes (AILDOS) compared to those with single autoimmune liver disease is unclear.

Aim: To investigate the survival of patients with AILDOS and assess the accuracy of non-invasive serum models for predicting liver-related death.

Methods: Patients with AILDOS were defined as either autoimmune hepatitis and primary biliary cholangitis overlap (AIH-PBC) or autoimmune hepatitis and primary sclerosing cholangitis overlap (AIH-PSC) and were identified from three tertiary centres for this cohort study. Liver-related death or transplantation (liver-related mortality) was determined using a population-based data linkage system. Prognostic scores for liver-related death were compared for accuracy [including liver outcome score (LOS), Hepascore, Mayo Score, model for end-stage liver disease (MELD) score and MELD incorporated with serum sodium (MELD-Na) score].

Results: Twenty-two AILDOS patients were followed for a median of 3.1 years (range, 0.35-7.7). Fourteen were female, the median age was 46.7 years (range, 17.8 to 82.1) and median Hepascore was 1 (range, 0.07-1). At five years post enrolment, 57% of patients remained free from liver-related mortality (74% AIH-PBC, 27% AIH-PSC). There was no significant difference in survival between AIH-PBC and AIH-PSC. LOS was a significant predictor of liver-related mortality (P < 0.05) in patients with AIH-PBC (n = 14) but not AIH-PSC (n = 8). A LOS cut-point of 6 discriminated liver-related mortality in AIH-PBC patients (P = 0.012, log-rank test, 100% sensitivity, 77.8% specificity) (Harrell's C-statistic 0.867). The MELD score, MELD-Na score and Mayo Score were not predictive of liver-related mortality in any group.

Conclusion: Survival in the rare, AILDOS is unclear. The current study supports the LOS as a predictor of liver-related mortality in AIH-PBC patients. Further trials investigating predictors of survival in AILDOS are required.