RESUMO
Objective:To study the correlation between anti-gp210 antibody, anti-sp100 antibody with clinical features and prognosis of patients with PBC.Methods:A total of 992 patients with PBC from 9 medical centers in Yunnan Province from January 1, 2015 to December 31, 2021 were included retrospectively. The demographic data, medical history, UDCA treatment, laboratory and imaging data were collected, and telephone follow-up was conducted. The positive rates of anti-gp210 antibody and anti-sp100 antibody in PBC patients with different clinical characteristics were compared, and the differences of laboratory parameters and prognosis between the anti-gp210 and anti-sp100 antibodies positive and negative groups were compared. T test, rank sum test, variance analysis were used for statistical analysis.Results:The positive rate of anti-gp210 antibody in Han patients was significantly higher than that in minority patients (21.5% vs 9.9%, χ2=6.88, P=0.009), but there was no significant difference in the positive rate of anti-sp100 antibody between the two groups (10.9% vs 6.6%, χ2=1.62, P=0.204).There were no significant differences in the positive rates of anti-gp210 antibody and anti-sp100 antibody among different genders ( χ2=0.50, P=0.478)( Z=-0.41, P=0.682)and ages( χ2=0.01, P=0.951)( Z=-0.60, P=0.549). There was no significant difference in the positive rate of anti-gp210 antibody between AMA M2 antibody positive and negative patients ( χ2=3.45, P=0.063), PBC patients with Sj?gren′s syndrome compared with those without Sj?gren′s syndrome (21.3% vs 20.4%, χ2=0.05, P=0.828), and PBC patients with viral hepatitis compared with those without viral hepatitis(19.6% vs 20.5%, χ2=0.02, P=0.877). The positive rate of anti-gp210 antibody was significantly increased in patients with PBC confirmed by liver biopsy with unknown diagnosis (25.6% vs 18.4%, χ2=6.52, P=0.011), patients with AIH (26.6% vs 18.9%, χ2=5.82, P=0.016), cirrhosis (23.3% vs 11.3%, χ2=16.00, P<0.001), decompensation of cirrhosis (23.9% vs 18.2%, χ2=4.66, P=0.031), jaundice (29.7% vs 17.1%, χ2=18.59, P<0.001) and hyperlipidemia (24.9% vs 18.1%, χ2=6.30, P=0.012). The positive rate of anti-sp100 antibody was significantly increased in patients with negative AMA M2 antibody PBC patients (20.9% vs 7.2%, χ2=36.54, P<0.001)and patients with PBC confirmed by liver biopsy with unknown diagnosis (17.9% vs 7.5%, χ2=23.40, P<0.001), while in patients with AIH (11.1% vs 10.3%, χ2=0.09, P=0.769), Sj?gren′s syndrome (15.7% vs 10.0%, χ2=2.87, P=0.090), viral hepatitis (4.3% vs 10.8%, χ2=1.94, P=0.164), cirrhosis(10.5% vs 10.5%, χ2<0.01, P=0.991), decompensated symptoms of cirrhosis (10.3% vs 10.6%, χ2=0.03, P=0.868), jaundice (12.5% vs 9.7%, χ2=1.62, P=0.203)and hyperlipidemia (8.7% vs 11.5%, χ2=1.86, P=0.172), the positive rate was not significantly increased. The levels of ALT [71.00(48.00, 111.00)U/L vs 58.00 (31.00,112.75)U/L, Z=-2.63, P=0.009], AST [92.00 (54.00, 133.00)U/L vs 76.00(42.00, 128.00)U/L, Z=-2.73, P=0.006], ALP[306.00(176.00, 528.00)U/L vs 204.00(126.25, 350.75)U/L, Z=-4.78, P<0.001], GGT[284.00(131.00, 524.00)U/L vs 165.00(53.63, 389.00)U/L, Z=-4.36, P<0.001], TBIL[33.60(16.60, 82.10)mmol/L vs 23.45 (14.80, 61.13)mmol/L, Z=-3.00, P=0.003], DBIL [20.30 (6.60, 66.40)mmol/L vs 11.60 (5.90, 45.00)mmol/L, Z=-3.13, P=0.002], bile acid[53.40(19.50, 148.00)mmol/L vs 39.30(11.70, 118.58)mmol/L, Z=-2.26, P=0.024], IgM[3.61(2.03,5.26)g/L vs 2.39(1.37, 3.67)g/L, Z=-5.38, P<0.001] and APTT[37.40(33.10, 41.30)s vs 35.70 (31.30, 41.30)s, Z=-3.28, P=0.001])were significantly increased in patients with positive anti-gp210 antibody compared patients with negative anti-gp210 antibody, while the IgG level was significantly increased in patients with positive anti-sp100 antibody compared with patients with negative anti-gp210 antibody( Z=-2.25, P=0.025), but no other indexes were significantly increased. The Mayo risk score[3.48(2.46, 5.01) vs 3.18 (2.20, 4.64), Z=-2.052, P=0.04] and mortality at the end of follow-up (24.6% vs 16.7%, χ2=6.57, P=0.0.038)in patients with positive anti-gp210 antibody were much higher than those in patients with negative anti-gp210 antibody, but there were no significant differences in Mayo risk score [3.16 (2.21, 4.53) vs 3.28 (2.23,4.71), Z=-0.86, P=0.392] and mortality at the end of follow-up (13.5% vs 18.9%, χ2=2.12, P=0.346) between anti-sp100 antibody positive and negative patients. Conclusion:PBC patients with positive anti-gp210 antibody may have more serious liver pathologic damage and extra-hepatic complications, more serious liver function impairment, more obvious cholestasis, and worse prognosis. Anti-sp100 antibody has been shown to have no significant correlation with disease severity and prognosis.
RESUMO
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.