| Objective Autoimmune hepatitis (AIH) was one of main liver diseases which were harmful to middle-aged females. At present, there was not an uniform clinical diagnostic criteria for diagnosis of patients with AIH in china.The clinical and pathological characteristics of AIH need to be clarified, and the mechanisms of hepatic immunopathologic injury and fibrosis of AIH was not clear.This study was to explore the usefulness of the Criteria of International Autoimmune Hepatitis (CIAH) published by IAIHG in 1999 to the diagnosis of AIH patients in China and the pathological characteristic of AIH. Meanwhile, the aim was to explore the mechanisms'of immunopathologic injury and fibrosis of liver in AIH.Methods The serum and bioptic liver specimens and clinical data from 36 cases of patients with AIH were analyzed. DCs were isolated and incubated from peripheral blood of 8 cases of AIH patients, and identified under electron microscopy. The expression of DC surface makers such as CD80, CD86, CD la, HLA-DR and lymphocyte subpopulations were detected by using the flow cytometric analysis(FACS). Lymphocyte subpopulations in the liver tissues from AIH patients were observed with a panel of monoclonal antibodies to lymphocytes by immunohistochemistry. Used synaptophysin as a new marker for hepatic stellate cells (HSCs), HSCs, collagen I, IV, MTrMMPandTIMP-1 and/or their mRNAs on the liver tissues were detected through immunohistochemical and in situ hybridization methods.Results The corresponding ratio between the CIAH scoring system for diagnosis of autoimmune hepatitis and pathological diagnosis was 97.3%. The pathological features of all the AIH liver tissues showed the lesions of chronic active hepatitis with hepatic fibrosis to varying degrees such as moderate- to severe interface hepatitis, obvious lobular inflammation, bridging necrosis, resetting of heaptocytes, central lobular confluent necrosis and predominant plasmacytic infiltration.The HLA-DR expression level on DCs from peripheral blood in AIH group was higher than that in normal control (p<0.05), and the expression levels of CD80, CD86^ CDla and HLA-DR of DCs were much higher than those in HBV group, respectively (p<0.01). The total T lymphocytes, CD8+ and y 5 T subpopulations in peripheral blood were increased compared with the normal control. With the increase of histological active index (HAI, Knodell), T lymphocytes, especially CD8+, CD57+ subpopulations were increased among the infiltrated mononuclear cells (MNCs) on the the AIH liver tissues.The SYN positive HSCs were observerd in the portal tracts, fibrotic septa and lobules of AIH liver tissues where inflammation was active, especially in the interface hepatitis areas. The number of HSCs increased according to the increasing HAI, while the deposition of Col K Col IV was remarkable with the increase of hepatic fibrosis stages (Knodell). MTrMMP and its mRNA were mainlyexpressed in mesenchymal cells which were distributed in the areas of interface hepatitis, bridging necrosis, lobular active inflammation, and also observed in a few hepatocytes. The expression of MTi-MMP was parallel to collagen IV distribution, and increased according to the the increasing HAI and fibrosis stages, and reached the peak in S4-5 stage. In addition, TIMP-lmRNA expression was similar to MTrMMP mRNA.Conclusions CIAH published by IAIHG in 1999 was suitable for diagnosis of AIH patients in China. There were some charateristic pathological features of AM.DCs were isolated and incubated successfully from peripheral blood from patients with AIH. The changes of cellular surface molecule expression on DCs showed that DCs in patient with AIH might have aberrant immunoactivity, which was correlative with the pathogenesis and pathological injury in AIH. Analysis of lymphocyte subpopulations in the liver tissues and in peripheral blood from patients with AIH indicated that cell immune mechanism might play an important role in the immunopathological injury of the livers, but the ADCC cytotoxicity and v 5 T mediated-immunor...
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