Efficacy and Safety of Immunosuppressive Therapy for PBC–AIH Overlap Syndrome Accompanied by Decompensated Cirrhosis: A Real-World Study
Aim. To explore the efficacy and safety of immunosuppressive therapy for the treatment of primary biliary cirrhosis-autoimmune hepatitis (PBC-AIH) overlap syndrome accompanied by decompensated cirrhosis. Methods. A cohort study was performed to evaluate the usefulness of immunosuppressive therapy in...
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| Format: | Article |
| Language: | English |
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Wiley
2018-01-01
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| Series: | Canadian Journal of Gastroenterology and Hepatology |
| Online Access: | http://dx.doi.org/10.1155/2018/1965492 |
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| author | Xiaoli Fan Yongjun Zhu Ruoting Men Maoyao Wen Yi Shen Changli Lu Li Yang |
| author_facet | Xiaoli Fan Yongjun Zhu Ruoting Men Maoyao Wen Yi Shen Changli Lu Li Yang |
| author_sort | Xiaoli Fan |
| collection | DOAJ |
| description | Aim. To explore the efficacy and safety of immunosuppressive therapy for the treatment of primary biliary cirrhosis-autoimmune hepatitis (PBC-AIH) overlap syndrome accompanied by decompensated cirrhosis. Methods. A cohort study was performed to evaluate the usefulness of immunosuppressive therapy in this unique group. This cohort study was performed between October 2013 and June 2017 and included 28 biopsy-proven patients diagnosed according to the Paris criteria. The therapies included ursodeoxycholic acid (UDCA) alone (N=14) or in combination with immunosuppression (IS) therapy (N=14). The primary endpoints were biochemical remission, liver-related adverse events, transplant-free survival, and drug side-effects. Results. The frequency of biochemical remission for the AIH features was significantly higher in the UDCA+IS group than in the UDCA-only group (60.0 versus 9.1%, P=0.024) after 12 months of therapy but not after 3 and 6 months (28.6 versus 0%, P=0.165; 35.7 versus 7.1%, P=0.098). The rates of liver-related adverse events were lower in the combined group (2/14 versus 9/14, P=0.018). The Kaplan-Meier estimate showed that the transplant-free survival was distinct between the two groups (P=0.019). In the UDCA+IS group, mild and transient leukopenia occurred in two patients receiving azathioprine (AZA), and an infection was observed in one patient receiving mycophenolate mofetil (MMF). Conclusions. PBC-AIH patients with decompensated cirrhosis receiving a combination of UDCA and immunosuppressors presented with higher biochemical remission rates and experienced fewer liver-related adverse events, implying that the combined treatment might be a better therapeutic option for strictly defined decompensated PBC-AIH overlap syndrome. |
| format | Article |
| id | doaj-art-a20749dddab343d9ac3b43331d66bca1 |
| institution | Kabale University |
| issn | 2291-2789 2291-2797 |
| language | English |
| publishDate | 2018-01-01 |
| publisher | Wiley |
| record_format | Article |
| series | Canadian Journal of Gastroenterology and Hepatology |
| spelling | doaj-art-a20749dddab343d9ac3b43331d66bca12025-02-03T05:57:36ZengWileyCanadian Journal of Gastroenterology and Hepatology2291-27892291-27972018-01-01201810.1155/2018/19654921965492Efficacy and Safety of Immunosuppressive Therapy for PBC–AIH Overlap Syndrome Accompanied by Decompensated Cirrhosis: A Real-World StudyXiaoli Fan0Yongjun Zhu1Ruoting Men2Maoyao Wen3Yi Shen4Changli Lu5Li Yang6Department of Gastroenterology & Hepatology, West China Hospital, Sichuan University, Chengdu, Sichuan 610041, ChinaDepartment of Gastroenterology & Hepatology, West China Hospital, Sichuan University, Chengdu, Sichuan 610041, ChinaDepartment of Gastroenterology & Hepatology, West China Hospital, Sichuan University, Chengdu, Sichuan 610041, ChinaDepartment of Gastroenterology & Hepatology, West China Hospital, Sichuan University, Chengdu, Sichuan 610041, ChinaDepartment of Gastroenterology & Hepatology, West China Hospital, Sichuan University, Chengdu, Sichuan 610041, ChinaDepartment of Pathology, West China Hospital, Sichuan University, Chengdu, Sichuan 610041, ChinaDepartment of Gastroenterology & Hepatology, West China Hospital, Sichuan University, Chengdu, Sichuan 610041, ChinaAim. To explore the efficacy and safety of immunosuppressive therapy for the treatment of primary biliary cirrhosis-autoimmune hepatitis (PBC-AIH) overlap syndrome accompanied by decompensated cirrhosis. Methods. A cohort study was performed to evaluate the usefulness of immunosuppressive therapy in this unique group. This cohort study was performed between October 2013 and June 2017 and included 28 biopsy-proven patients diagnosed according to the Paris criteria. The therapies included ursodeoxycholic acid (UDCA) alone (N=14) or in combination with immunosuppression (IS) therapy (N=14). The primary endpoints were biochemical remission, liver-related adverse events, transplant-free survival, and drug side-effects. Results. The frequency of biochemical remission for the AIH features was significantly higher in the UDCA+IS group than in the UDCA-only group (60.0 versus 9.1%, P=0.024) after 12 months of therapy but not after 3 and 6 months (28.6 versus 0%, P=0.165; 35.7 versus 7.1%, P=0.098). The rates of liver-related adverse events were lower in the combined group (2/14 versus 9/14, P=0.018). The Kaplan-Meier estimate showed that the transplant-free survival was distinct between the two groups (P=0.019). In the UDCA+IS group, mild and transient leukopenia occurred in two patients receiving azathioprine (AZA), and an infection was observed in one patient receiving mycophenolate mofetil (MMF). Conclusions. PBC-AIH patients with decompensated cirrhosis receiving a combination of UDCA and immunosuppressors presented with higher biochemical remission rates and experienced fewer liver-related adverse events, implying that the combined treatment might be a better therapeutic option for strictly defined decompensated PBC-AIH overlap syndrome.http://dx.doi.org/10.1155/2018/1965492 |
| spellingShingle | Xiaoli Fan Yongjun Zhu Ruoting Men Maoyao Wen Yi Shen Changli Lu Li Yang Efficacy and Safety of Immunosuppressive Therapy for PBC–AIH Overlap Syndrome Accompanied by Decompensated Cirrhosis: A Real-World Study Canadian Journal of Gastroenterology and Hepatology |
| title | Efficacy and Safety of Immunosuppressive Therapy for PBC–AIH Overlap Syndrome Accompanied by Decompensated Cirrhosis: A Real-World Study |
| title_full | Efficacy and Safety of Immunosuppressive Therapy for PBC–AIH Overlap Syndrome Accompanied by Decompensated Cirrhosis: A Real-World Study |
| title_fullStr | Efficacy and Safety of Immunosuppressive Therapy for PBC–AIH Overlap Syndrome Accompanied by Decompensated Cirrhosis: A Real-World Study |
| title_full_unstemmed | Efficacy and Safety of Immunosuppressive Therapy for PBC–AIH Overlap Syndrome Accompanied by Decompensated Cirrhosis: A Real-World Study |
| title_short | Efficacy and Safety of Immunosuppressive Therapy for PBC–AIH Overlap Syndrome Accompanied by Decompensated Cirrhosis: A Real-World Study |
| title_sort | efficacy and safety of immunosuppressive therapy for pbc aih overlap syndrome accompanied by decompensated cirrhosis a real world study |
| url | http://dx.doi.org/10.1155/2018/1965492 |
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