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
{"title":"自身免疫性肝病重叠综合征的生存预测因素。","authors":"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","doi":"10.4254/wjh.v16.i9.1269","DOIUrl":null,"url":null,"abstract":"<p><strong>Background: </strong>Survival in patients with autoimmune liver disease overlap syndromes (AILDOS) compared to those with single autoimmune liver disease is unclear.</p><p><strong>Aim: </strong>To investigate the survival of patients with AILDOS and assess the accuracy of non-invasive serum models for predicting liver-related death.</p><p><strong>Methods: </strong>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].</p><p><strong>Results: </strong>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 (<i>P</i> < 0.05) in patients with AIH-PBC (<i>n</i> = 14) but not AIH-PSC (<i>n</i> = 8). A LOS cut-point of 6 discriminated liver-related mortality in AIH-PBC patients (<i>P</i> = 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.</p><p><strong>Conclusion: </strong>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.</p>","PeriodicalId":23687,"journal":{"name":"World Journal of Hepatology","volume":null,"pages":null},"PeriodicalIF":2.5000,"publicationDate":"2024-09-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11438591/pdf/","citationCount":"0","resultStr":"{\"title\":\"Predictors of survival in autoimmune liver disease overlap syndromes.\",\"authors\":\"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\",\"doi\":\"10.4254/wjh.v16.i9.1269\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<p><strong>Background: </strong>Survival in patients with autoimmune liver disease overlap syndromes (AILDOS) compared to those with single autoimmune liver disease is unclear.</p><p><strong>Aim: </strong>To investigate the survival of patients with AILDOS and assess the accuracy of non-invasive serum models for predicting liver-related death.</p><p><strong>Methods: </strong>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].</p><p><strong>Results: </strong>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 (<i>P</i> < 0.05) in patients with AIH-PBC (<i>n</i> = 14) but not AIH-PSC (<i>n</i> = 8). A LOS cut-point of 6 discriminated liver-related mortality in AIH-PBC patients (<i>P</i> = 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.</p><p><strong>Conclusion: </strong>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.</p>\",\"PeriodicalId\":23687,\"journal\":{\"name\":\"World Journal of Hepatology\",\"volume\":null,\"pages\":null},\"PeriodicalIF\":2.5000,\"publicationDate\":\"2024-09-27\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11438591/pdf/\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"World Journal of Hepatology\",\"FirstCategoryId\":\"1085\",\"ListUrlMain\":\"https://doi.org/10.4254/wjh.v16.i9.1269\",\"RegionNum\":0,\"RegionCategory\":null,\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"Q2\",\"JCRName\":\"GASTROENTEROLOGY & HEPATOLOGY\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"World Journal of Hepatology","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.4254/wjh.v16.i9.1269","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q2","JCRName":"GASTROENTEROLOGY & HEPATOLOGY","Score":null,"Total":0}
Predictors of survival in autoimmune liver disease overlap syndromes.
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.
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