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Predictors of survival in autoimmune liver disease overlap syndromes

World Journal of Hepatology 2024年第9期16卷 1269-1277页

作者： Dujinthan Jayabalan Yi Huang Luis Calzadilla-Bertot Malik Janjua Bastiaan de Boer John Joseph Wendy Cheng Simon Hazeldine Briohny W Smith Gerry C MacQuillan Michael C Wallace George Garas Leon A Adams Gary P Jeffrey Medical School University of Western AustraliaNed-lands 6009Western AustraliaAustralia Department of Hepatology Sir Charles Gairdner HospitalNedlands 6009Western AustraliaAustralia Department of Anatomical Pathology PathWest Laboratory MedicineNedlands 6009Western AustraliaAustralia Department of Gastroenterology and Hepatology Royal Perth HospitalPerth 6000Western AustraliaAustralia Department of Gastroenterology and Hepatology Fiona Stanley HospitalMurdoch 6150Western AustraliaAustralia Medical School University of Western AustraliaNedl-ands 6009Western AustraliaAustralia

BACKGROUND Survival in patients with autoimmune liver disease overlap syndromes(AILDOS)compared to those with single autoimmune liver disease is *** To investigate the survival of patients with AILDOS and assess the accuracy of non-invasive serum models for predicting liver-related *** 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 ***-related death or transplantation(liver-related mortality)was determined using a population-based data linkage *** 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 *** 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 *** Survival in the rare,AILDOS is *** current study supports the LOS as a predictor of liver-related mortality in AIH-PBC *** trials investigating predictors of survival in AILDOS are required.

关键词： Autoimmune liver disease overlap syndromes Liver outcome score Liver-related death hepascore Autoimmune hepatitis Primary biliary cholangitis Primary sclerosing cholangitis

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