In addition, we enrolled 19 (7.3%) patients with early PBC. liver transplantation and 17 died. The cumulative survival rate and median survival time were 83.9% and 181.7 mo, respectively. Cox regression analysis revealed that an incomplete ursodeoxycholic acid (UDCA) response or inconsistent treatment [< 0.001; hazard risk (HR) 95%CI = 2.423-7.541], anti-centromere antibodies (ACA) positivity (< 0.001; HR 95%CI = 2.516-7.137), alanine aminotransferase ratio (AAR) elevations (< 0.001; HR 95%CI = 1.357-2.678), and histological advanced liver disease (= 0.006; HR 95%CI = 1.481-10.847) were predictors of hepatic decompensation. The clinical features and survival of PBC in China are consistent with those described in Western countries. CONCLUSION: Incomplete UDCA response or inconsistent treatment, ACA positivity, AAR elevations, and advanced histological stage are predictors of decompensation. test. Variables CP544326 (Taprenepag) that were significant in univariate analysis were submitted to Cox regression analysis to predict the impartial risk factors. Hazard risk (HR) and 95%CI were presented. Kaplan-Meier analysis was used to assess survival. Patients who were lost to follow-up or died of causes unrelated to liver failure were censored. RESULTS Clinical profiles of the study group at the entry In this study, 262 patients with a mean age of 51.5 10.2 years, including 240 (91.6%) women, were enrolled. The median duration of follow-up was 75.2 mo (range, 21-201 mo). The demographic and clinical features are shown in Tables ?Tables11 and ?and22. Table 1 Demographic and clinical laboratory characteristics of primary biliary cirrhosis patients at CP544326 (Taprenepag) the time of entry (= 262) (%) Age (yr), mean SD51.5 10.2Gender (female/male), = 262) ANA181(69.1)AMA245 (93.5)ACA73 (27.9)ALT (IU/L)91.0 (9-598)AST (IU/L)86.2 (15-428)AST/ALT1.10 (0.22-4.85)ALP (IU/L)348.5 (21-1486)GGT (IU/L)428.2 (9-2614)ALB (g/L)40.7 (25-69)TBil (mol/L)28.05 (4.90-334.40)DBil (mol/L)14.39 (0.34-237.40)IgG (g/L)16.81 (5.12-56.60)IgA (g/L)3.15 (0.19-8.84)IgM (g/L)4.66 (0.41-22.30) Open in a separate window Continuous data were expressed as (%) or median (range). ANA: Anti-nuclear antibody; AMA: Anti-mitochondrial antibody; ACA: Anti-centromere antibody; ALT: Alanine aminotransferase; AST: Aspartate aminotransferase; ALP: Alkaline phosphatase; GGT: -glutamyl-transferase; ALB: Albumin; TBil: Total bilirubin; DBil: Direct bilirubin; Ig: Immunoglobulin. On entry into the study, 170 (64.9%) patients presented with manifestations of liver disease, and 96 (36.6%) patients had extra-hepatic autoimmune disease. In addition, we enrolled 19 (7.3%) patients with early PBC. Two hundred and forty-five (93.5%) patients were seropositive for AMA. ANA and ACA were detected in 181 (69.1%) and 73 (27.9%) patients, respectively. Among 110 patients who underwent needle liver biopsies, 97 (88.2%) were at stages?I-II, while 13 (11.8%) were at stages III-IV. One hundred and ninety (72.5%) patients received UDCA regularly and responded, while 46 patients (17.6%) took UDCA consistently, but failed to respond. Of the latter, eight patients terminated treatment on their own. Others (9.9%) did not take UDCA consistently, as prescribed. Incidence of outcomes and survival During the study period, 62 (23.7%) patients developed adverse events, and the median duration from diagnosis to hepatic decompensation was CP544326 (Taprenepag) 57.4 mo (range, 7-151 mo). Among these patients, four (1.5%) patients underwent liver transplantation at the end of follow-up or before death, and 17 (6.5%) patients died of liver failure. The median duration from diagnosis to death was 63.6 mo (range, 29-112 mo). According to Kaplan-Meier analysis, the cumulative survival rate and estimated BTF2 median survival time were 83.9% and 181.7 mo, respectively (Determine ?(Physique22 and Table ?Table33). Open in a separate window Physique 2 Survival curve of all patients in this cohort. Table 3 Complications of chronic liver disease in the primary biliary cirrhosis cohort (= 262) (%) Hepatic decompensation62 (23.7)Portal hypertension62 (23.7)Hypersplenism44 (16.8)Esophageal varices47 (17.9)Variceal bleeding35 (13.4)Ascites43 (16.4)Encephalopathy19 (7.3)Coagulant function abnormality12 (4.6)Hypoproteinemia35 (13.4)Spontaneous bacterial peritonitis3 (1.1)Liver transplantation4 (1.5)Death-liver related17 (6.5)1Censor6 (2.3)Lost to follow-up4 (1.5)Death from causes unrelated to liver failure2 (0.8) Open in a separate window 1One patient who underwent liver transplantation died. Risk factors for hepatic decompensation Possible risk factors were first identified by univariate analysis. Subsequently, all variables, except histological stage, that showed statistical differences in univariate analysis were introduced into the Cox regression model. Incomplete UDCA response or inconsistent UDCA treatment (< 0.001; HR 95%CI = 2.423-7.541), ACA positivity (< 0.001; HR 95%CI = 2.516-7.137), alanine aminotransferase ratio (AAR) elevations (< 0.001; HR 95%CI = 1.357-2.678), TBil (= 0.004; HR.