title: Risk factors for hepatic decompensation in patients with primary biliary cirrhosis.
abstract: OBJECTIVE
  To examine the clinical features and analyze prognostic factors in a prospective study of primary biliary cirrhosis (PBC) patients.
  
  
  METHODS
  From 1995 to 2010, PBC patients without hepatic decompensation seen at the Peking Union Medical College Hospital were enrolled. Clinical signs and manifestations (pruritus, persistent fatigue, jaundice and pain in the right hypochondrium), laboratory parameters (auto-antibodies for autoimmune hepatic disease, biliary and hepatic enzymes, immunoglobulin, bilirubin, and albumin) and imaging findings were recorded at entry and at specific time points during follow-up. Cox regression and Kaplan-Meier analyses, respectively, assessed the risk factors for hepatic decompensation and survival.
  
  
  RESULTS
  Two hundred and sixty-two PBC patients were enrolled with a median follow-up of 75.2 mo (range, 21-201 mo). The 240 patients were aged 51.5 ± 10.2 years at diagnosis and 91.6% were female. Two hundred and forty-five (93.5%) were seropositive for anti-mitochondrial antibodies. At presentation, 170 patients (64.9%) were symptomatic, while 96 patients (36.6%) had extra-hepatic autoimmune disease. During the follow-up period, 62 (23.7%) patients developed hepatic decompensation of whom four underwent 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 [P < 0.001; hazard risk (HR) 95%CI = 2.423-7.541], anti-centromere antibodies (ACA) positivity (P < 0.001; HR 95%CI = 2.516-7.137), alanine aminotransferase ratio (AAR) elevations (P < 0.001; HR 95%CI = 1.357-2.678), and histological advanced liver disease (P = 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.
  
  
  CONCLUSIONS
  Incomplete UDCA response or inconsistent treatment, ACA positivity, AAR elevations, and advanced histological stage are predictors of decompensation.
authors: Department of Rheumatology, Key Laboratory of Rheumatology and Clinical Immunology, Peking Union Medical College Hospital, Chinese Academy of Medical Science, Beijing 100032, China.
authors: Tian-Yan
authors: TY
authors: Shi
authors: Li-Na
authors: LN
authors: Zhang
authors: Hua
authors: H
authors: Chen
authors: Li
authors: L
authors: Wang
authors: Min
authors: M
authors: Shen
authors: Xuan
authors: X
authors: Zhang
authors: Feng-Chun
authors: FC
authors: Zhang
mesh_terms: D000328:Adult; D015415:Biomarkers / Q000097:blood; D016009:Chi-Square Distribution; D002681:China; D002756:Cholagogues and Choleretics / Q000627:therapeutic use; D018450:Disease Progression; D005260:Female; D006801:Humans; D053208:Kaplan-Meier Estimate; D008105:Liver Cirrhosis, Biliary / Q000097:blood / Q000150:complications* / Q000175:diagnosis / Q000276:immunology / Q000401:mortality / Q000628:therapy; D017093:Liver Failure / Q000097:blood / Q000175:diagnosis / Q000209:etiology* / Q000276:immunology / Q000401:mortality / Q000628:therapy; D016031:Liver Transplantation / Q000009:adverse effects / Q000401:mortality; D008297:Male; D008875:Middle Aged; D016016:Proportional Hazards Models; D011446:Prospective Studies; D012307:Risk Factors; D013997:Time Factors; D016896:Treatment Outcome; D014580:Ursodeoxycholic Acid / Q000627:therapeutic use
chemical_list: D015415:Biomarkers; D002756:Cholagogues and Choleretics; D014580:Ursodeoxycholic Acid
keywords: Anti-centromere antibodies; Hepatic decompensation; Histological stage; Primary biliary cirrhosis; Risk factor; Survival; Ursodeoxycholic acid response