Clinical And Pathological Characteristics Of Autoimmune Liver Disease

BackgroudAutoimmune liver diseases is a presumed autoimmune disease of the liver,which predominantly affects liver target organ and is mediated by immunity,including autoimmune hepatitis,primary biliary cirrhosis,primary sclerosing cholangitis and overlap syndrome.The prevalence of the disease is high in Europe and Americans in the past,ALD is considered rare in our nation,but the incidence becomes inereasing recently,More studies of ALD are carried out.the reason is that it is recognized deeply by degrees and the sciences of immunology,molecular biology and immunopathology are developping quickly,in addition,the research of autoantibody is deep.Diagnostic criteria and a scoring system have been proposed to codify and updated by Inter- national Autoimmune Hepatitis Group to hold conferences one after another.The diagnosis and management is deemed the most severe standard with autoimmune dieases.In our country,it was discovered of increasing uninterrupted autoimmunity liver patients in clinical practice work.It is easy to confuse with chronic liver disease. Moreover,it is relative difficulty in clinical diagnose and belong to non-viral disease. Although autoimmunity liver disease(ALD) is mainly appearance liver function abnormity,it is completely different from viral hepatitis in clinical therapeutic principle.ALD is demanded to give hormone and immune depressant,and so on. viral hepatitis is demanded to give Interferon and immune response strengthen medicine.It can lead to worsen if interferon and immune depressant are used impertinently in ALD.For these reasons,it has important significance in differential diagnosis of ALD with other chronic hepatitis.Because of absence of symptoms failing to identify diagnosis and treatment in time,some patients evolve very easily in hepatic cirrhosis.ALD is important reason leading to hepatic cirrhosis,accounting percent 4 to percent 5 transplantation of liver.Now,it is still to advanced identify nosogenesis and clinic and pathology characteristic of ALD.In this article,in order to facilitate recognition of the disease,our study try to discuss the following problem:including to analyze the clinical and histopathological characteristics of autoimmune liver disease.In order to improve its diagnosis and treatment.Objective1.By using descriptive diagnosis standard and scoring system which was newly revised by the Internationai Autoimmune Hepatitis Group Report,to analyze the clinical manifestations and laboratory findings of 25 patients with ALD.2.25 underwen the hepatic biopsy cases of autoimmune liver disease were chosen to analyze the histopathological features by means of Hematoxylin-Eosin(HE) and argyrophil reaction(by Grimeliu's) staining and CD138 immunohistochemical staining.MethodsThe study group is 25 patients with ALD treated in our department from January 2000 to June 2007 were final diagnosis.ALD were retrospectively analyzed,and compared with the control group which is 25 patients with chronic hepatitis B identical inflammation level by stages and grading.To collect clinical data and to carry out immunohistochemistry mark and contrastly to analyze clinical data and pathohistology for hepatic tissue normal position infiltrative plasma cell.Results:1.The number of CD138 positive cells in the ALD group were significantly higher than that in the CHB group with immunohistochemical staining.[(7.1±5.0)/HP and (3.6±2.5)/HP(t=3.098,P=0.003).2.There were 10 cases(40%) of pure AIH patients and 10 case(40%) of pure PBC patients among the 25 cases,the rest 5 cases(20%)were over-lapped synthesis.ALD mainly affects middle and old aged female patients,The female patients with auto -immune hepatopathy was 18 cases,with a mean age of 42.6±13.6 years old,Of all the patients,the onset and clinical features are not typical.Biochemical examination showed elevated levels of alanine aminotransferase,aspartate aminotransferase and total bilirubin.The levels of serum alkaline phosphatase(ALP) and serum gamma glutamy transpeptidase(γ-GT) were also very high;The three group were not significantly in statistics.The positive ratio of autoantibodies in AIH group is 60%,80%in PBC group.The autoantibodies appearance is correlated to age and gender.Biochemical examination showed were not significantly in statistics between the positive group of autoantibodies and the negative group of autoantibodies.3.ALD pathological change reveals heavy inflammation stagings is 17 cases(68%). Hepatic cirrhosis is 8 cases(32%).Inflammation activity is irrelated toγ-GT.The main histopathological change of AIH was characterized of chronic hepatitis,the infiltration of lymphocytes,mononuclear cells and plasma cells in the portal tract or periportal area were found in the liver biopsie.Interface hepatitis is the histologic hall mark of the syndrome and portal plasma cell infiltration,companion with cellular necrosis and fibrosis.Lobular hepatitis,rosette of liver cells,bridging necrosis and nodular regenerative hyperplasia with fibrous septa were observed in serious cases.PBC is characterized by progressive desturetion of small intrahepatic bile duets,granulomatous destruction of the interlobular bile ducts which leads to bile duct obstruction,bile deposition and bile duct disappearance.The consequent cholestasis is generally slowly progressive,and eventual liver fibrosis and cirrhosis occur.The liver biopsy specimens in the AIH-PBC group were characterized by AIH and PBC.Conclusion:1.The increase of plasma cells in liver biopsy is possibly significant for diagnosis of ALD.The number of plasma cells in the ALD group were significantly higher than that in the CHB group[(7.1±5.0)/HP and(3.6±2.5)/HP(t=3.098,P=0.003).2.ALD mainly affects middle and old aged female patients;Biochemical examination showed elevated levels of liver enzyme.Autoantibodies is limited to diagnosis of ALD.Liver biopsy examination is essential to establish the diagnosis and evaluate disease severity to determine the need for treatment.3.ALD is considered the same pathogenesy and similar pathological change that inflammation located in the portal tract or periportal area in earlier period of disease,ended in hepatic cirrhosis in final stage.The main histopathological change was characterized of chronic hepatitis,the infiltration of lymphocytes,mononuclear cells and plasma cells in the portal tract or periportal area were found in the liver biopsie.