Clinical Analysis Of Overlap Syndrome In Autoimmune Liver Disease

BackgroundAutoimmune liver disease(AILD) was mediated by the abnormal autoimmune liver inflammatory injury, mainly includes autoimmune hepatitis(AIH), primary biliary cirrhosis(PBC), primary sclerosing cholangitis(PSC) and more than two or three kinds of overlap syndrome(OS) of liver disease. AILD is a new hotspot in the research of the domestic field of liver disease, OS in AILD is a special type of autoimmune disease,there were many studies on AIH-PBC OS previously. Recently we found some triple overlap syndrome(AIH-PBC-PSC OS)patients in clinical,who had characteristics of AIH, PBC, PSC at the same time, so we still need to further explore the standard of diagnosis and treatment of such diseases. All data of 7 patients diagnosed with AIH-PBC-PSC OS,including clinical characteristics and biochemistry indexes, autoantibodies, imaging findings,liver histology and therapy were analyzed, to enhance the understanding of AIH-PBC-PSC OS in AILD. ObjectiveTo enhance comprehension skills of trigeminy overlap syndrome(AIH-PBC-PSC OS), explore the diagnosis and treatment of AIH-PBC-PSC OS in AILD. MethodsSeven patients with AIH-PBC-PSC OS and forty-five patients with AIH-PBC OS by liver biopsy, who admitted to the First Affiliated Hospital of Zhengzhou University between January 2012 and October 2015, were enrolled in this study. Immunostaining was performed on liver specimens from these patients. All data of 52 patients, including clinical characteristics and biochemistry indexes, autoantibodies, imaging findings and liver histology were analyzed. Ursodeoxycholic acid(UDCA)[13~15 mg/(kg·d)],prednisone(30mg/d) and diminished(5mg/qw, until 10mg/d) and immunosuppressants were given to these patients. Results1. There were no significant difference in gender, fatigue, anorexia, itching,but there were significant differences in age, jaundice, total bilirubin between the AIH-PBC group with AIH-PBC-PSC group.2. In autoantibodies detection, the detection rate of p-ANCA had significant difference between the two groups.3. All patients underwent liver biopsy, pathological results showed the “hepatic rosette formation”, the "onion skin" appearance change, the level of fibrosis were significantly higher in the livers of AIH-PBC-PSC OS patients as compared with those patients with AIH-PBC OS.4.The survival curves was different between AIH-PBC group and AIH-PBC-PSC group,the latter survival rate was lower. Conclusion1. In autoimmune liver disease, AIH,PBC,PSC can simultaneously or sequential progress to AIH-PBC-PSC OS.2. When patients with AIH-PBC OS with severe jaundice or total bilirubin increased obviously or p-ANCA positive or liver pathology appeared "onion skin" appearance change, which need to consider whether these patients suffering from AIH-PBC-PSC OS.3.The efficacy of AIH-PBC-PSC OS patients was poor, immunosuppressive therapy may be effective to alleviate the patients' clinical symptoms, but their short-term survival rate was low.