| AIMS:To analyze the clinical features and therapeutic effects of patients with Autoimmune hepatitis (AIH), primary biliray cirrhosis (PBC) and AIH-PBC overlap syndrome (OS) and determine the expression of immune inflammatory factors like interleukin (IL)-12, IL-17, and interferon-gamma (IFN-γ) in the liver of AIH, PBC, and AIH-PBC and investigate the correlation between them, in order to explore the clinical characteristics and pathological mechanism of those diseases.METHODS:A retrospective study was made in21patients with AIH,21patients with PBC and9patients with AIH-PBC OS who all received liver biopsy in Tianjin Medical University General Hospital from Jan,2009to Jan,2012. The clinical features and therapeutic effects of AIH, PBC, and AIH-PBC were compared. The expression of IL-12, IL-17, and IFN-Y in the liver of patients were examined by immunohistochemistry, correlations between those immune inflammatory factors were analyzed.RESULTS:①Female preponderance were seen among the patients with AIH, PBC and AIH-PBC, the percentage of female patients was95.20%(20/21),95.20%(20/21), and100%(9/9), respectively.②Clinical manifestations:33.33%(7/21) patients with AIH presented with weak and inappetence etc,38.10%(8/21) patients with PBC presented with pruritus,33.33%(3/9) patients with AIH-PBC presented with asymptomatic features like weak and inappetence,33.33%(3/9) presented with pruritus.③Lab examinations:Patients with AIH exhibited higher levels of serum alanine aminotransferase (ALT), aspartate aminotransferase (AST), and globulin (GLO) and the concentration of IgG in patients with AIH were significantly higher than those with PBC (P=0.002) and AIH-PBC (P=0.046); The concentration of serum alkaline phosphatase (ALP), and gamma-glutamyl transferase (GGT) were higher in PBC patients, but the level of GLO was significantly lower than AIH (P=0.016) and the level of IgM in patients with PBC were significantly higher than those with AIH (P=0.000) and AIH-PBC (P=0.013); The levels of ALT, AST, ALP, GGT, IgG and IgM were all elevated in patients with AIH-PBC.④Histologic features:interface hepatitis occurred in95.23%(20/21) patients with AIH,42.86%(9/21) patients showed rosettes, all those portal lesions spare the bile ducts;57.14%(12/21) PBC had florid bile duct lesions,38.09%(8/21) had interface hepatitis, no resettes occurred;100%(9/9) AIH-PBC patients had interface hepatitis,22.22%(2/9) showed rosettes,44.44%(4/9) had bile duct lesions.⑤Therapeutic effects:the remisson rate was85.71%(18/21),71.42%(15/21), and66.67%(6/9), respectively for patients with AIH, PBC, and AIH-PBC;9.52%(2/21),19.05%(4/21), and22.22%(2/9) achieved an incomplete response, respectively;4.76%(1/21),9.52%(2/21), and11.11%(1/9) met the criteria for treatment failure, respectively.⑥The expression of inflammatory factors and correlations:the rate of positive expression of IL-12in AIH, PBC and AIH-PBC were19.05%,9.52%and11.11%, respectively, significantly lower than control groupC(P=0.000); those of IL-17were71.43%,76.19%and77.78%, those of IFN-γ were66.67%,85.71%and77.78%, all significantly higher than control group(P=0.002and P=0.000). Negative correlation was found between IL-12and IL-17in the liver of AIH and PBC (Pearson were-0.499and-0.58; P=0.021and0.006).CONCLUSIONS:①AIH, PBC and AIH-PBC have female predominance, clinical features are asymptomatic.②The serological examinations of AIH, PBC and AIH-PBC are specific. Patients with PBC display high level of ALP, GGT and/or high titer of AMA/AMA-M2or elevated IgM, if high level of AST, ALT and/or high titer of ANA/SMA and IgG occurred, AIH-PBC OS may exit.③Interface hepatitis and liver cell rosettes are the features of AIH, bile duct lesions are the features of PBC, AIH-PBC has the features of both AIH and PBC.④AIH patients including those with cirrhosis should receive immunosuppressive therapy;71.42%patients with PBC respond to UDCA well; Patients with AIH-PBC need UDCA combined immunosuppressive therapy.⑤The expression of IL-12are reduced in autoimmune liver disease, especially in PBC, at the same time the expression of IL-17and IFN-γ are increased. Indicted that IL-12-IL17inflammatory pathway plays an important role in pathology. |
