Clinical significance of autoantibody and liver function test in patients with chronic hepatitis B combined with autoimmune liver disease / 中国热带医学

@#Abstract:Objective To detect the expression of hepatic function indices and autoantibodies in patients with chronic hepatitis B patients, patients with autoimmune liver disease and patients with chronic hepatitis B combined with autoimmune liver disease, and to evaluate the clinical significance of autoantibodies and hepatic function indexes in the early diagnosis of chronic hepatitis B combined with autoimmune liver disease.Methods A total of 109 healthy controls (HC), 72 patients with chronic hepatitis B (CHB), 74 patients with autoimmune liver disease (AILD), and 24 patients with chronic hepatitis B combined with autoimmune liver disease (CHB+AILD) in the Fifth People’s Hospital of Suzhou from 2013 to 2021 were enrolled in this study. Basic information and the value of admission hepatic function indexes and autoantibodies were collected for all enrolled samples, while no autoantibody test was performed for healthy volunteers. All data were processed using GraphPad Prism and SPSS software.Results There were no significant differences in age and gender among the four groups. The detection rates of anti-mitochondrial antibody M2 (AMA-M2) and anti-soluble acidic phosphorylated nuclear protein antibody (anti-SP100 antibodies) in CHB+AILD group [29.2%(7/24), 17.4%(4/23)] were significantly higher than those in CHB group [5.1%(3/59), 0(0/59)], suggesting that the detection of these two autoantibodies is helpful to the differential diagnosis of CHB and CHB+AILD. In addition, eight hepatic function indexes displayed significant differences among the four groups. The levels of total bilirubin and direct bilirubin in CHB+AILD group were significantly higher than those in CHB and AILD groups, while the levels of total protein and albumin were significantly lower than those in CHB and AILD groups. Alkaline phosphatase and glutamyltranspeptidase in AILD group and CHB+AILD group were significantly higher than those in CHB group. The logistic regression analysis showed that total bilirubin, direct bilirubin, albumin, alanine aminotransferase and alkaline phosphatase could form a promising prediction model, which was useful for clinicians in the differential diagnosis of CHB and CHB+AILD (area under the curve, AUC=0.902).Conclusion The combination of autoantibody and hepatic function index detection can be helpful for clinicians in the differential diagnosis of CHB, AILD and CHB+AILD, thus contributing to the early and correct diagnosis of CHB+AILD and providing theoretical basis for patients to obtain reasonable treatment and clinical cure earlier.

The role of pathogen infections in the development and progression of autoimmune liver diseases / 临床肝胆病杂志

The etiology and pathogenesis of autoimmune liver diseases has always been a hot area of research. Pathogen infections can elicit an autoimmune response and often become the key pathogenic factor of immune diseases. Based on the literature data and the author's clinical experience, this review will briefly introduce the role and influence of pathogen infections in the development and progression of autoimmune liver diseases from the aspects such as molecular mimicry mechanism, in order to further understand the pathogenesis of autoimmune liver diseases.

Environmental exposure as a risk-modifying factor in liver diseases: Knowns and unknowns

Liver diseases are considered to predominantly possess an inherited or xenobiotic etiology. However, inheritance drives the ability to appropriately adapt to environmental stressors, and disease is the culmination of a maladaptive response. Thus "pure" genetic and "pure" xenobiotic liver diseases are modified by each other and other factors, identified or unknown. The purpose of this review is to highlight the knowledgebase of environmental exposure as a potential risk modifying agent for the development of liver disease by other causes. This exercise is not to argue that all liver diseases have an environmental component, but to challenge the assumption that the current state of our knowledge is sufficient in all cases to conclusively dismiss this as a possibility. This review also discusses key new tools and approaches that will likely be critical to address this question in the future. Taken together, identifying the key gaps in our understanding is critical for the field to move forward, or at the very least to "know what we don't know."

Pairing analysis of clinical prognosis between liver transplantation with autoimmune liver disease and viral hepatitis cirrhosis / 器官移植

Objective To analyze the difference and influential factors of clinical prognosis between liver transplantation with autoimmune liver disease (AILD) and viral hepatitis cirrhosis. Methods Clinical data of 75 recipients undergoing liver transplantation from January 2002 to January 2017 were retrospectively analyzed. All recipients were divided into the AILD group (n=25) and viral hepatitis cirrhosis group (n=50). The intraoperative conditions of the recipients were observed including warm ischemia time, cold ischemia time, operation time, anhepatic phase and blood transfusion volume. Postoperative complications were observed including severe acute kidney injury (AKI), infection, acute rejection, biliary tract-related complications, vascular-related complications and post transplantation diabetes mellitus (PTDM). The follow-up status were monitored after discharge. The prognostic factors of liver transplant recipients were analyzed. Results The warm ischemia time, cold ischemia time, operation time and anhepatic phase did not significantly differ between two groups (all P > 0.05). In the AILD group, the incidence of postoperative acute rejection was remarkably higher, whereas the incidence of postoperative severe AKI was significantly lower than those in the viral hepatitis cirrhosis group (both P < 0.05). The postoperative 1-, 3- and 5-year survival rates in the AILD group was 92%, 87%, and 87%, which did not significantly differ from 88%, 88% and 88% in the viral hepatitis cirrhosis group (all P > 0.05). Univariate analysis showed that age, model for end-stage liver disease (MELD) score, severe AKI, infection and biliary tract-related complications were the influencing factors of clinical prognosis of the recipients (all P < 0.05). Conclusions The overall survival prognosis does not significantly differ between the AILD and viral hepatitis cirrhosis groups. Age, MELD score, severe AKI, infection and biliary tract-related complications are the risk factors affecting the clinical prognosis of liver transplantation recipients.

Analysis of clinical features and prognosis in patients with primary Sjögren's syndrome and autoimmune liver disease / 北京大学学报(医学版)

OBJECTIVE@#To analyze the clinical features and prognosis in patients with primary Sjögren's syndrome (pSS) and autoimmune liver diseases (ALD).@*METHODS@#A retrospective analysis of clinical manifestation and prognosis was performed in patients with ALD or without ALD during the three years (February 2014 to December 2017).@*RESULTS@#Totally, 203 patients with pSS were included in this study, 68 patients had ALD (31 patients with autoimmune hepatitis, 37 patients with primary biliary cholangitis), while 135 patients did not have ALD. There were no differences between the two groups regarding age, gender, clinical manifestations, such as dry mouth, dry eyes, pain, fatigue, lymphadenopathy, glandular swelling, cutaneous involvement, lung involvement, and renal involvement, and the incidence rate of other autoimmune diseases, such as autoimmune thyroid disease, rheumatoid arthritis, and vasculitis. There were also no differences in the titer of antinuclear antibody (ANA), the positive rates of anti-Sjögren's syndrome A antibody (SSA), SSA52, and anti-Sjögren's syndrome B antibody (SSB), and at the levels of erythrocyte sedimentation rate and C-reactive protein between the two groups. Most importantly, the pSS patients with ALD had a shorter disease course, a higher positive rate of anti-mitochondrial M2 antibody (AMA-M2) and anti-centromere antibody, a higher level of IgG and IgM, a lower level of complement 3, and a decreased number of blood cells. They also had a higher level of liver related serum index, such as alanine aminotransferase, aspartate aminotransferase, gamma-glutamyl transferase, alkaline phosphatase and total bilirubin, direct bilirubin, indirect bilirubin, a higher incidence rate of liver cirrhosis, an increased death incident (the mortality was 13.24% in the pSS patients with ALD, while 2.96% in the controls, P=0.013), and a worse prognosis. Binary Logistic regression analysis revealed that liver cirrhosis, the EULAR Sjögren's syndrome disease activity index (ESSDAI) scores and the level of total bilirubin were the prognostic factors of mortality in the pSS patients with ALD. The survival curve was estimated by the Kaplan-Meier method. It demonstrated that the pSS patients with ALD had a lower survival rate when compared with the controls.@*CONCLUSION@#The patients with both pSS and ALD will suffer from a more severe disease and a higher death incident. We should pay more attention to these patients and provide a better symptomatic treatment for them during clinical practice.

Wilsons disease and autoimmune liver disease overlap syndrome: a clinical study

Background: The coexistence of Wilson’s disease and autoimmune liver disease in a same patient is a rare entity. Combined treatment with steroid and D-penicillamine may be effective. Aim of the study was analyse the clinical, histological, laboratory profile for patients with chronic liver disease with aim of finding the etiology of the disease.Methods: It is an observational study. Common clinical presentations were evaluated. Laboratory investigations done include complete blood count, renal and liver function tests, prothrombin time, viral markers for hepatitis A, B, C and E, USG abdomen and pelvis, portal Doppler studies and upper GI endoscopy. Specific tests include ANA, AMA, ASMA, Anti LKM-1Ab, serum ceruloplasmin and 24hrs urinary copper were done. Liver biopsy was done in selected patients.Results: Commonest clinical presentation was abdominal distension (80%), abdominal pain (30%), pedal edema (60%), splenomegaly (40%) and upper GI bleed (40%). Laboratory investigation revealed anemia (50%), thrombocytopenia (70%), prothrombin time prolongation in (60%), normal liver function in 60%, abnormal liver function in (40%). Autoimmune markers revealed ANA strong positivity in (40%), mild positivity in (60%). AMA, ASMA, Anti-LKM-1 were negative in all cases (100%). Liver biopsy showed features of autoimmune liver disease and Periportal copper deposition in 80% of cases.Conclusions: Coexistence of Wilson’s disease and autoimmune liver disease is a rare entity and medical treatment with steroids and D-penicillamine simultaneously to be started in these patients.

Preliminary study on abundance and diversity of microbiota in liver tissues of patients with autoimmune liver disease / 中华消化杂志

Objective To provide clues for the study on the mechanism of autoimmune liver disease (AILD) by exploring the existence of specific bacteria in liver tissues of AILD patients.Methods From August 2017 to August 2018,at Department of Gastroenterology arnd Hepatology,Tianjin Medical University General Hospital,a total of 12 patients diagnosed as AILD (four autoimmune hepatitis (AIH),four primary biliary cirrhosis (PBC) and four PBC-AIH overlap syndrome (OS)) and four patients with hepatic cyst (control group) were enrolled and all the patients underwent liver biopsy.16S rRNA gene sequencing was carried out in the obtained aseptic liver tissues.Linear discriminant analysis effect size was used to find out the specific bacteria.Spearman correlation analysis was performed to analyze the correlation between the liver microbiota and the disease.The metabolic function of the 16S rRNA gene sequences was also predicted.Results Bacteria were detected in the liver tissues of all the 16 patients.At the species level,the abundance of Planococcus rifietoensis of AIH group was 0.100％,which was higher than those of other three groups (0),and the difference was statistically significant (linear discriminant analysis (LDA) =3.31,P =0.034).The abundance of Anoxybacillus flavithermus of PBC group was 0.200％,which was higher than those of other three groups (0.100％),and the difference was statistically significant (LDA =3.34,P =0.014).The abundance of Pseudomonas aeruginosa PAO1,Bacillus firmus,Brevibacillus agri,Acinetobacter baumannii,Sphingomonas zeae and Salmonella enterica were significantly negatively correlated with serum level of γ-glutamyl transferase (r=-0.68,-0.68,-0.67,-0.68,-0.68 and-0.66,all P ＜0.01).Compared with that of the hepatic cyst group,the lipid metabolism of AILD patients decreased.The levels of serum low density lipoprotein and total cholesterol were significantly negatively correlated with the biosynthesis of unsaturated fatty acids (r =-0.55 and-0.65,both P ＜ 0.05).Conclusions There exist specific bacteria in the liver tissues of AIH and PBC groups.The liver microbiota which is closely related with the pathogenesis of AILD might be a potential therapeutic target and diagnostic biomarker.

Characteristics of initial diagnosis in autoimmune liver disease: an 18-year, retrospective single-center study / 中华内科杂志

Objective To analyze the chorological changes of diagnosis in patients with autoimmune liver disease (AILD) and related factors for early diagnosis.Methods A total of 581 patients with age ranged from 16 to 81 were retrospectively analyzed,who were admitted to Tianjin Medical University General Hospital with AILD during January 2000 to December 2017.Age at diagnosis,diagnostic method and cirrhosis at diagnosis were compared in different groups according to admission period as 2000-2005,2006-2011,2012-2017.Results The diagnostic rate of AILD showed an upward trend during the past near two decades.The proportion of AILD patients diagnosed via health examination was increasing year by year mainly by elevated transaminases (P＜0.001).The mean age at diagnosis in our AILD patients were younger at present,especially in men (P=0.044).The proportion of cirrhosis at diagnosis was gradually reduced in three different periods respectively [77.78％(21/27),41.58％ (79/190),25.00％(91/364),P＜0.001],which were coincident in patients with autoimmune hepatitis (AIH) and primary biliary cirrhosis (PBC) (P＜0.001).The shrinking trend of cirrhosis at diagnosis was significantly correlated with the increasing application of health examination (r=-0.549,P＜0.001).Conclusions Extensive application of health examination expands the diagnostic rate of AILD.During the past 18 years,more young patients are diagnosed with AILD.The proportion of severe cases such as cirrhosis at diagnosis is decreasing.Screening of immunological examinations in patients with abnormal transaminases is needed and critical to the early diagnosis of asymptomatic AILD.

Clinical analysis of liver transplantation in end-stage autoimmune liver diseases / 中华危重病急救医学

Objective@#To explore the clinical characteristics and long-term prognosis of recipients with end-stage autoimmune liver diseases (AILD) after liver transplantation.@*Methods@#A retrospective study was conducted. The data of 147 end-stage autoimmune liver diseases recipients who underwent liver transplantation admitted to department of liver transplantation of Tianjin First Center Hospital from January 2004 to August 2018 were collected. Patients were followed till March 1st, 2019. The postoperative survival was analyzed, the cumulative survival rate was estimated by the Kaplan-Meier survival curve, and the cause of death was analyzed. Postoperative rejection, recurrence of AILD, biliary complications, and hepatitis virus infection were also summarized.@*Results@#Among the 147 recipients, 25 were autoimmune hepatitis (AIH), 94 were primary biliary cholangitis (PBC), 14 were AIH overlapping PBC (AIH-PBC), 14 were primary sclerosing cholangitis (PSC). The 147 recipients were consisted of 22 males and 125 females, with a ratio of 1∶5.7, with a mean age of (53.2±10.1) years old. The average model for end-stage liver disease (MELD) score was 14.6±9.0. After a median follow-up of 49.0 months, 21 recipients died, and 126 recipients survived. The survival curve analysis showed that the 1-, 3-, 5- and 10-year cumulative survival rate was 98.6%, 94.0%, 84.6%, and 76.9%, respectively. The main cause of death was graft dysfunction, followed by graft failure and respiratory failure. Among the 147 recipients, 17 (11.56%) rejection occurred, 19 recurrence (12.93%) were diagnosed with liver biopsy, and of which 2 PBC recipients overlapped de novo AIH-PBC. Biliary complications happened in 37 recipients (25.17%). De novo hepatitis virus infection was found in 19 recipients (12.93%), including 14 with hepatitis B virus (HBV) and 5 with hepatitis C virus (HCV).@*Conclusions@#The majority patients of the end-stage of AILD were middle-aged and old women, and PBC was the most common. Liver transplantation is an effective treatment for end-stage autoimmune liver diseases with excellent prognosis. Postoperative immunosuppressant usage and recurrence should be emphasized.

Clinical analysis of liver transplantation in end-stage autoimmune liver diseases / 中华危重病急救医学

To explore the clinical characteristics and long-term prognosis of recipients with end-stage autoimmune liver diseases (AILD) after liver transplantation. Methods A retrospective study was conducted. The data of 147 end-stage autoimmune liver diseases recipients who underwent liver transplantation admitted to department of liver transplantation of Tianjin First Center Hospital from January 2004 to August 2018 were collected. Patients were followed till March 1st, 2019. The postoperative survival was analyzed, the cumulative survival rate was estimated by the Kaplan-Meier survival curve, and the cause of death was analyzed. Postoperative rejection, recurrence of AILD, biliary complications, and hepatitis virus infection were also summarized. Results Among the 147 recipients, 25 were autoimmune hepatitis (AIH), 94 were primary biliary cholangitis (PBC), 14 were AIH overlapping PBC (AIH-PBC), 14 were primary sclerosing cholangitis (PSC). The 147 recipients were consisted of 22 males and 125 females, with a ratio of 1∶5.7, with a mean age of (53.2±10.1) years old. The average model for end-stage liver disease (MELD) score was 14.6±9.0. After a median follow-up of 49.0 months, 21 recipients died, and 126 recipients survived. The survival curve analysis showed that the 1-, 3-, 5- and 10-year cumulative survival rate was 98.6%, 94.0%, 84.6%, and 76.9%, respectively. The main cause of death was graft dysfunction, followed by graft failure and respiratory failure. Among the 147 recipients, 17 (11.56%) rejection occurred, 19 recurrence (12.93%) were diagnosed with liver biopsy, and of which 2 PBC recipients overlapped de novo AIH-PBC. Biliary complications happened in 37 recipients (25.17%). De novo hepatitis virus infection was found in 19 recipients (12.93%), including 14 with hepatitis B virus (HBV) and 5 with hepatitis C virus (HCV). Conclusions The majority patients of the end-stage of AILD were middle-aged and old women, and PBC was the most common. Liver transplantation is an effective treatment for end-stage autoimmune liver diseases with excellent prognosis. Postoperative immunosuppressant usage and recurrence should be emphasized. sclerosing cholangitis; Liver transplantation; Prognosis

Marcadores serológicos de hepatopatías virales y autoinmunes en pacientes celíacos atendidos en el Instituto de Gastroenterología / Serologic markers of viral and autoimmune liver diseases in celiac patients treated at the Institute of Gastroenterology

Introducción: El espectro del compromiso hepático en la enfermedad celíaca es muy amplio e incluye hepatopatías virales y autoinmunes. Objetivo: Caracterizar el comportamiento de los marcadores serológicos de hepatopatías virales y autoinmunes en pacientes celíacos adultos atendidos en el Instituto de Gastroenterología. Materiales y Métodos: Se realizó un estudio observacional descriptivo transversal en el período comprendido entre marzo de 2016 y marzo 2017 en 43 pacientes adultos celíacos atendidos en el Instituto de Gastroenterología de La Habana. A todos, previo consentimiento informado se les realizaron estudios bioquímicos y serológicos para identificar infección por virus de hepatitis B, C y respuesta inmune humoral frente a autoanticuerpos hepáticos y sistémicos. Para el análisis estadístico se emplearon distribuciones de frecuencia y para la comparación entre los grupos se utilizó la probabilidad exacta de Fisher. Se consideró significativo una p < 0,05. Resultados: El 58,1 por ciento de los pacientes presentó hipofosfatasemia y 14 por ciento hipertransaminasemia, sin coincidencias entre los casos registrados. El 100 por ciento de los celíacos fue negativo para el virus de hepatitis C. La positividad de autoanticuerpos hepáticos se registró entre 2,3 por ciento y 14 por ciento de los pacientes evaluados. Existió asociación significativa entre hipertransaminasemia y la presencia de anticuerpos séricos contra la transglutaminasa tisular. Conclusiones: La expresión de marcadores de autoinmunidad hepática en los pacientes celíacos evaluados es frecuente, no así la presencia de marcadores de hepatopatías virales(AU)

Introduction: The spectrum of hepatic involvement in celiac disease is very broad and includes viral and autoimmune hepatopathies. Objective: To characterize the behavior of serologic markers of viral and autoimmune hepatopathies in adult celiac patients treated at the Institute of Gastroenterology. Materials and Methods: A cross-sectional observational study was conducted in 43 adult celiac patients treated at the Institute of Gastroenterology of Havana from March 2016 to March 2017. Prior informed consent, biochemical and serologic studies were carried out in all cases, in order to identify hepatitis B and C virus infections and humoral immune response to liver and systemic autoantibodies. Frequency distributions were used for statistical analysis, and Fisher's exact test was used for the comparison between groups. Results: 58.1 percent of the patients presented hypophosphatasemia and hypertransaminasemia in 14 percent, with no coincidence between the cases recorded. The 100 percent of the celiac patients were negative for hepatitis C virus. The positivity of liver autoantibodies was recorded between 2,3 percent and 14 percent of the patients evaluated. There was a significant association between hypertransaminasemia and the presence of serum antibodies against tissue transglutaminase. Conclusions: The expression markers of liver autoimmunity in celiac patients was frequent, but not the presence of markers of viral hepatopathy(AU)

Clinical significance of liver biopsy in diagnosis of non-viral liver diseases / 中华消化杂志

Objective To observe the clinical characteristics of non-viral liver diseases which were examined by ultrasound guided liver biopsy in order to explore the significance of liver biopsy.Methods From January 2006 to December 2015,patients with non-viral liver diseases who received liver biopsy were retrospectively enrolled.Etiology,pathological diagnosis and clinical diagnosis of the patients were analyzed;the differences in disease types between male and female,among different age (less than 60 years and over 60 years)were compared;and the consistency of two pathologists in the pathological diagnosis was analyzed.Chi-square test was performed for statistical analysis.Results Among 182 patients,there were 73 (40.1％) males and 109 (59.9％) females.The most common etiology were autoimmune liver disease (68 cases,37.4％),non-alcoholic fatty liver disease (NAFLD) (40 cases,22.0％) and drug-induced liver injury (DILI) (29 cases,15.9％).The differences in etiology between male and female,between age less than 60 years and over 60 years were statistically significant (X2 =7.31 and 5.87,both P ＜ 0.05).The consistency of two pathologists in the diagnosis of NAFLD,alcoholic liver disease,hereditary metabolic disease and neoplastic disease was good (Kappa=0.85,0.88,0.75 and 1.00).The consistency rates of two pathologists in the diagnosis of autoimmune disease,NAFLD and DILI were 52.9％ (36/68),75.0％ (30/40) and 48.3％ (14/29),respectively.The consistency rate was highest in NAFLD,and the difference was statistically significant (X2 =7.68,P=0.023).However,there was no significant difference in consistency rates of two pathologists in the diagnosis of autoimmune liver disease and DILI (X2 =0.12,P=0.859).The consistency rates between pathological diagnosis and clinical diagnosis in autoimmune liver disease,NAFLD and DILI were 60.3％ (41/68),85.0％ (34/40) and 55.2％ (16/29),respectively,and the difference was statistically significant (X2 =8.98,P=0.011).The consistency rate in NAFLD was highest.However,there was no significant difference in consistency rates between pathological diagnosis and clinical diagnosis in autoimmune liver disease and DILI (X2 =0.22,P=0.639).Conclusions The liver biopsy may guide the clinical diagnosis of NAFLD.However,to improve the diagnostic rates of autoimmune liver disease and DILI,biopsy results,history and laboratory results should be combined.

A retrospective analysis of liver transplantation in treating endstage autoimmune liver disease / 中华器官移植杂志

Objective Investigate the prognosis of patients undergoing liver transplantation (LT) for end-stage autoirnmune liver disease (ALD).Method The clinical data of 45 patients with endstage ALD undergoing LT from April 2001 to March 2015 in the first affiliated hospital of Zhejiang University were analyzed retrospectively.The postoperative cumulative survival rate of the recipients was calculated,and the causes of death were analyzed.The postoperative rejections,new onset viral hepatitis and ALD recurrence were also analyzed.Result In 45 ALD recipients,33 cases survived and the postoperative 5-year cumulative survival rate was 78.8％.Causes for 12 dead cases were mnultiple organ failure,liver graft failure,respiratory complications,hemorrhage and hepatic artery embolization.In 45 ALD recipients,6 cases suffered rejection after operation with the incidence bing 13.3％.One case suffered new onset hepatitis B infection 8 years after opcration.One recipient suffered primary disease (primary biliary cirrhosis) recurrence 2 years after operation,and 1 recipient with primary disease (primary sclerosing cholangitis) developed into overlap syndrome.They all survived for a long term after active treatments.Conclusion Most LT recipients with endstage ALD can obtain a long term survival.Attentions should be paid to the immunosuppressive regimens in early period after LT,prevention of infection,rejection and postoperative new onset viral hepatitis,and timely diagnosis of primary disease recurrence.

Clinical Significance of Detection Antibody of Liver Antigens in Patients with Autoimmune Hepatic Diseases and Viral Hepatitis / 现代检验医学杂志

Objective To study the positive incidence and clinical significance of auto-antibody of liver antigens in the serum of patients with autoimmune hepatitis diseases and viral hepatitis.Methods The serum samples with hepatic diseases were col-lected in Jiangyin People’s Hospital from 2010 to 2014 year.Patients were divided into three groups according to diseases:autoimmune hepatic disease group including autoimmune hepatitis (AIH)12 cases and primary biliary cirrhosis (PBC)36 case;viral hepatic group including HAV 23 cases,HBV 30 cases,HCV 14 cases and HEV 8 cases;normal control group 30 cases.Auto-antibody of live antigens AMA-M2,LKM-1,LC-1,SLA/LP,GP210 and SP100 were tested respectively by west-ern blotting assay.Results The positive rates of anti-AMA-M2,anti-LKM-1,anti-LC-1,anti-SLA/LP,anti-GP210,anti-SP100 were 16.7%,16.7%,8.3%,25%,0% and 16% respectively in AIH group,83.3%,0%,0%,0%,44.4% and 27.8% respectively in PBC group.Patients with viral hepatitis,one case were anti-AMA-M2 positive,one case were anti-GP210 positive and three case with positive anti-SP100.anti-AMA-M2,anti-GP210 and anti-SP100 were detected more fre-quently in autoimmune liver diseases group than viral hepatitis group (χ2 = 33.9,10.6 and 8.8,P 0.05)compared with nor-mal control group.Conclusion The antibody of liver antigens are useful clinically for diagnosing and classifying autoimmune liver disease.

A Case of Recurrent Uveitis in Autoimmune Liver Disease

PURPOSE: To report a case of recurrent uveitis associated with autoimmune liver disease. CASE SUMMARY: A 50-year-old female with severe fatigue and arthritis visited the ophthalmology department due to decreased visual acuity and discomfort in her right eye for ten days. She had intermittent injection and blurred vision in both eyes for 30 years. Slit lamp examination of her right eye showed keratic precipitates, pigment deposits on the anterior capsule of the lens and anterior chamber cells; fundus examination was normal without any sign of chorioretinitis. Inflammatory reaction was improved after steroid and cycloplegic eye drop treatment. Two months later, her left eye developed anterior uveitis. Inflammation was well controlled with steroid and cycloplegic eye drop treatment. To evaluate the cause of uveitis and associated systemic disease, serological testing was performed, and abnormal elevation of liver enzymes was detected. The patient was referred to the Gastroenterology Department and diagnosed with autoimmune liver disease. Oral ursodeoxycholic acid was prescribed. Liver function profile improved to normal range, and the patient is currently under routine follow-up with no sign of recurrent uveitis. CONCLUSIONS: When a patient with recurrent uveitis presents symptoms such as nausea, fatigue, abdominal pain, jaundice or abnormal liver profile, association with autoimmune liver disease should be considered.

Prognostic analysis of liver transplantation in treating patients with end-stage autoimmune liver disease / 器官移植

Objective To investigate the prognosis of patients underwent liver transplantation (LT) for end-stage autoimmune liver disease (AILD). Methods Clinical data of 48 patients with end-stage AILD undergoing LT from May 1996 to April 2013 in Affiliated Changzheng Hospital of the Second Military Medical University were analyzed retrospectively. The postoperative cumulative survival rates of the recipients were calculated,and the cause of death was analyzed. The postoperative rejections,new-onset viral hepatitis and AILD recurrence were analyzed. Results In 48 AILD recipients,38 cases survived and the postoperative 5-year cumulative survival rate was 76%. Causes of death for the 10 dead cases were multiple organ failure, liver graft failure,sepsis,pulmonary infection,hemorrhage,hepatic artery embolization and renal failure. In 48 AILD recipients,9 cases (19%) suffered acute rejection after operation,3 cases suffered new-onset hepatitis B infection in 1-2 years after operation,2 recipients suffered primary disease (primary biliary cirrhosis)recurrence 2 years after operation and all survived for a long term after positive treatments.Conclusions Most liver transplant recipients with end-stage AILD can obtain a long-term survival. Attentions should be paid on the immunosuppressive regimens in early period after LT,prevention of infection,rejection and postoperative new-onset viral hepatitis,timely diagnosis of primary disease recurrence.

Orthotopic liver transplantation for the treatment of end-stage autoimmune liver disease / 中华普通外科杂志

Objective To evaluate the effect of liver transplantation for end-stage autoimmune liver disease (ESALD) and summarize the clinical experience of liver transplantation in the treatment of ESALD.Methods The clinical data of 11 ESALD cases who underwent liver transplantation from September 2003 to July 2009 were analyzed retrospectively. There were 2 males and 9 females ( median age, 44. 2 ± 8. 7years). The indication of liver transplantation was end stage of primary biliary cirrhrosis (8 cases),autoimmune hepatitis (2 cases), and primary sclerosing cholangitis ( 1 case). In all cases, modified piggyback liver transplantation with venacavaplasty was carried out. Postoperatively all patients were treated with immunosuppressive agents including tacrolimus (or cyclosporine A) and prednisone, some patients were treated additionally with mycophenolate mofetil and ursodeoxycholic acid. Results Postoperatively 2patients of primary biliary cirrhosis died, one of lung infection and multiple organ failure on the 5th postoperative day, the other dying of sepsis and graft dysfunction on the 964th postoperative day. Five cases suffered from episodes of acute cellular rejection within 1 month after transplantation and was successfully reversed by strengthened immunosuppressive therapy. Nine patients recovered satisfactorily and with excellent life quality until now. Patients were followed up from 7 months to 62 months with the median follow-up time of 38 months. The recipient survival rate at 1 year and 3 years was 91% and 82% ,respectively. One patient has now survived for 5 years. No recurrent ALD case was found during follow up.Conclusions Orthotopic liver transplantation is an exclusive treatment for ESALD. Optimum operation timing and effective immunosuppressive treatment are very important for decreasing occurrence of complications.

Clinical investigation of mycophenolate mofetil treatment for refractory autoimmune liver disease / 中华风湿病学杂志

Objective To investigate the efficacy and safety of mycophenolate mofetil (MMF) for refractory autoimmune 1iver disease:Methods Six patients with autoimmune liver disease who had failed MMF treatment and variation of biochemical indexes.adverse effects of the MMF treatment were recorded.Results After MMF treatment.the serum 1evels of alanine aminotransferase (ALT) were decreased to nOrmal level in three of four patients with higher ALT.The serum levels of alkaline phosphatase (ALP) were decreased to more than fifty percent of normal level in four of five patients with higher ALP.The serum levels of γ-glutamyhransferase (GGT) were decreased to more than fifty percent of normal level in all five patients with higher GGT.The serum levels of immunoglobulin G (IgG) and γ-globulin were decreased to normal level in all two patients who had elevated IgG and r-globulin levels.The serum levels of total bilirublin and total bile acid.the count of white blood cell and platelet had no change.There were no adverse effects in a11 six patients.Conclusion MMF treatment for early refactory autoimmune liver disease is effective with few sideeffects.