Is autoimmune cholangitis-anti-mitochondrial antibody-negative primary biliary cholangitis still an independent disease? / 中华肝脏病杂志

Autoimmune cholangitis (AIC) was first reported in 1987 as a chronic cholestatic disease that occurs predominantly in middle-aged women and has a common clinical manifestations, biochemical abnormalities and pathological changes with primary biliary cholangitis (PBC). However, serum anti-mitochondrial antibodies (AMA) are negative, and ANA and/or smooth muscle antibody positive rates are higher. The treatment response and prognosis with ursodeoxycholic acid and steroids is poor, thus it needs to be treated with immunosuppressive agents. Presently, the exact pathological mechanism of AIC is still unclear, and there is no unified assertion that classifies it as a new autoimmune liver disease or AMA-negative PBC. This article reviews the worldwide published work on AIC and compares them with PBC.

Value of serum autoantibodies in the diagnosis of anti-mitochondrial antibody-negative primary biliary cirrhosis / 中华消化杂志

Objective To compare clinical biochemical and pathological characters among antimitochondrial antibody-negative primary biliary cirrhosis (AMA PBC),AMA positive PBC (AMA+ PBC)and autoimmune hepatitis (AIH),and try to find serum biomarkers in the diagnosis of AMA-PBC.Methods From January 2005 to May 2013,23 patients with AMA-PBC,102 patients with AMA+ PBC and 55 patients with AIH were collected.Clinical features of patients were observed.Biochemical indexes (total bilirubin (TBil),direct bilirubin (DBil),alanine aminotransferase (ALT),aspartate transaminase (AST),alkaline phosphatase (ALP),γ-glutamyltransferase (GGT) and globulin) were tested.Immunological indexes immunoglobulin ((Ig)G,IgM and IgA) were also measured.Antinuclear amibody,anti smooth muscle antibody (SMA) and AMA were detected by indirect immunofluorescence.Soluble acidic phosphorylation nucleoprotein antibody and anti-nuclear membrane glycoprotein antibody were detected by Western blotting.Non parameter test was for non normal distributed measurement data and grade data comparison.The cut off value,sensitivity and specificity of IgM in the diagnosis of PBC were calculated with receiver operating characteristic (ROC) curve.The sensitivity and specificity of soluble acidic phosphorylation nucleoprotein antibody and anti-nuclear membrane glycoprotein antibody in the diagnosis of PBC were analyzed with fourfold table.Results The rates of fatigue and jaundice of AMA-PBC group (39.1％,9/23 ; 43.5 ％,10/23) were both higher than those of AIH group (16.4 ％,9/55; 14.5 ％,8/55),and the differences were statistically significant (x2 =4.735 and 7.648,both P＜ 0.05).The levels of ALP and GGT of AMA PBC group were higher than those of AIH group,and the levels of ALT and AST were lower than those of AIH group (Z=-4.577,-4.257,-2.820 and -2.055,all P＜0.05).The level of IgM of AMA PBC group (417(270,610) mg/L) was higher than that of AIH group (97(69,195) mg/L),however lower than that of AMA+ PBC group (546(419,704) mg/L),and the differences were statistically significant (Z=-4.362 and-0.210,both P＜0.05).The level of IgG of AMA PBC group was lower than that of AIH group (Z=-2.202,P＜0.05).The positive rates of antinuclear antibody and SMA of AMA PBC group (95.7％ (22/23),8.7％ (2/23)) were both higher than those of AMA+ PBC group (33.3％(34/102),1.0％(1/102)),and the differences were statistically significant (x2 =29.474 and 4.769,both P＜0.05).The positive rates of soluble acidic phosphorylation nucleoprotein antibody and anti nuclear membrane glycoprotein antibody of AMA PBC group (60.9％,14/23; 30.4％,7/23) were both higher than those of AIH group (2.0％,1/55; 0),and the differences were statistically significant (x2=36.409 and 24.329,both P＜0.05).The karyotype of antinuclear antibody of AMA PBC and AMA+ PBC group mainly was granules pattern (60.9 ％,14/23;75.5％,77/102),however of AIH group was mainly homogeneous pattern (38.2％,21/55).If the cut off value of IgM was 277 mg/L,the sensitivity and specificity of IgM in the diagnosis of PBC was 72.2％ and 94.4 ％.The sensitivity and specificity of soluble acidic phosphorylation nucleoprotein antibody in the diagnosis of PBC was 24.1％ and 97.2％.The sensitivity and specificity of anti-nuclear membrane glycoprotein antibody was 35.2 ％ and 100.0 ％.The sensitivity and specificity of any one of anti-nuclear membrane glycoprotein antibody positive,soluble acidic phosphorylation nucleoprotein antibody positive or IgM＞277 mg/L in the diagnosis of PBC was 88.9％ and 94.4％.Conclusions AMA-PBC and AMA+ PBC have similar clinical manifestations and serum biochemical test results.Antinuclear antibody and/or SMA positive is the feature of AMA-PBC and which should be distinguished with AIH.The sensitivity and specificity of any one of the followiags:anti nuclear membrane glycoprotein antibody positive,soluble acidic phosphorylation nucleoprotein antibody positive or IgM＞277 mg/L is high in the diagnosis of PBC.

Significance of serum anti-gp210 antibody detection in diagnosis of primary biliary cirrhosis / 临床肝胆病杂志

Primary biliary cirrhosis (PBC)is a chronic disease characterized by progressive destruction of intrahepatic small bile ducts, which may progress to liver cirrhosis.Anti-mitochondrial antibodies,especially anti-M2 antibody,have a high diagnostic value for PBC, but they are unrelated to the severity and prognosis of the disease and are negative in some patients.There have been reports from around the world that anti-nuclear antibodies,especially anti-gp210 antibody,are closely associated with PBC.It showed that anti-gp210 antibody has high specificity and sensitivity for the diagnosis of PBC,especially for the patients with negative anti-M2 antibody tests;in addition,it has a high predictive value for the prognosis and development model of the disease.Anti -gp210 antibody has a high diagnostic value for PBC,with great clinical significance,so its detection holds promise for clinical application.

The comparison of two different detection methods for anti-gp210 antibody / 中华风湿病学杂志

Objective To attempt using the synthetic poly-peptides in the carboxyl terminal of gp210 antigen as the substrate for anti-gp210 antibody detection and search for a simple assay method of detecting anti-gp210 antibody. Methods The enzyme linked immunosorbent assay (ELISA) method for anti-gp210 antibody detection was set-up by chessboard test. Anti-gp210 antibody was tested in the serum of both patients with primary biliary cirrhosis (PBC) and the control group by ELISA and immuno-blotting. Results The working concentration of gp210's antigen was 5 μg/ml. The optical density higher than 0.61 (x+3s) was designated as the positive cutoff of anti-gp210 antibody. There was no statistical difference between the two assays (P=0.617). And there was very strongly positive correlation between them (P=0.000, r=0.868). Conclusion The sensitivity and specificity are basically consistent between the assays using synthetic poly-peptides of gp210 antigen and native antigen as the detecting substrates. The former substrate is preferred to be used for the testing of anti-gp210 antibody in clinical laboratories.