title: Prognostic indicators in primary biliary cirrhosis: significance of revised IAHG (International Autoimmune Hepatitis Group) score.
abstract: OBJECTIVE
  Primary biliary cirrhosis (PBC) is a slowly progressing autoimmune disease of the liver that is characterized by portal inflammation and immune-mediated destruction of the intrahepatic bile ducts. Serum total bilirubin is one of the various prognostic factors that have been proposed. A recent study found that PBC with accompanying autoimmune hepatitis (AIH) carries a negative prognosis. This study examined the clinical characteristics of PBC and analyzed the factors that affect its prognosis.
  
  
  METHODS
  Patients diagnosed with PBC between January 1998 and December 2010 based on clinical and histopathological findings were compiled and analyzed retrospectively.
  
  
  RESULTS
  Among 27 patients, 24 (1 male and 23 females, ages 50.0±9.3 years) were followed up. The follow-up period was 8.6±0.9 years. Of the 24 patients, 9 patients progressed to liver cirrhosis (LC). Comparison between patients who did and did not progress to LC revealed statistically significant differences in the patients' serum total bilirubin (2.7±1.8 vs. 0.8±0.4, P=0.012), the Mayo risk score (5.1±0.7 vs. 3.9±0.6, P=0.001), the revised IAHG (International Autoimmune Hepatitis Group) score (9.2±2.3 vs. 5.4±3.0, P=0.004) and frequency of AIH overlap (5/9 [55.6%] vs. 0/15 [0%], P=0.001) at the time of diagnosis.
  
  
  CONCLUSIONS
  We propose that serum total bilirubin, the Mayo risk score, and the revised IAHG score at the time of diagnosis are helpful for predicting PBC prognosis. In particular, since all of the patients with accompanying AIH progressed to LC, the presence of overlap syndrome at the time of diagnosis is helpful for predicting PBC prognosis and providing an adequate treatment.
authors: Institute for Digestive Research, Digestive Disease Center, Department of Internal Medicine, Soonchunhyang University College of Medicine, Seoul, Korea.
authors: Ho Eun
authors: HE
authors: Jung
authors: Jae Young
authors: JY
authors: Jang
authors: Soung Won
authors: SW
authors: Jeong
authors: Jin Nyoung
authors: JN
authors: Kim
authors: Hee Yoon
authors: HY
authors: Jang
authors: Yun Ju
authors: YJ
authors: Cho
authors: Sung Ae
authors: SA
authors: Woo
authors: Sae Hwan
authors: SH
authors: Lee
authors: Sang Gyune
authors: SG
authors: Kim
authors: Sang-Woo
authors: SW
authors: Cha
authors: Young Seok
authors: YS
authors: Kim
authors: Young Deok
authors: YD
authors: Cho
authors: Hong Soo
authors: HS
authors: Kim
authors: Boo Sung
authors: BS
authors: Kim
mesh_terms: D000328:Adult; D000368:Aged; D001663:Bilirubin / Q000097:blood; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008105:Liver Cirrhosis, Biliary / Q000175:diagnosis* / Q000473:pathology; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D012189:Retrospective Studies; D012307:Risk Factors; D012720:Severity of Illness Index
chemical_list: D001663:Bilirubin
keywords: Overlap syndrome; Primary biliary cirrhosis; Prognosis