title: Impact of inflammatory bowel disease and ursodeoxycholic acid therapy on small-duct primary sclerosing cholangitis.
abstract: A longitudinal, cohort study was performed to characterize the clinical features of patients with small-duct primary sclerosing cholangitis (PSC) occurring with and without inflammatory bowel disease (IBD) and to determine the influence of IBD and the effect of ursodeoxycholic acid (UDCA) therapy on the course of the liver disease. Forty-two patients with small-duct PSC (14 women and 28 men; mean age, 36.7 +/- 13.3 years) were followed for up to 24.9 years. At presentation, prevalence of signs of liver disease (none versus 35%, P = 0.002), gastroesophageal varices (5% versus 30%, P = 0.03), and stage III/IV disease (9% versus 45%, P = 0.008) were lower in those with IBD versus those without IBD. During follow-up, 6 patients underwent liver transplantation, and another died of cirrhosis. Using the Cox proportional hazard analysis, concomitant IBD was not associated with liver death or transplant, whereas the revised Mayo risk score for PSC was the only prognostic factor associated with liver-related outcomes (relative risk, 6.47; 95% confidence interval, 1.75-137.5). UDCA (13-15 mg/kg/day) therapy for an average of 40 months showed biochemical improvement (P < 0.001) in UDCA-treated patients, while no significant change occurred in untreated patients. UDCA therapy had no effect on delaying progression of disease (relative risk, 0.95; 95% confidence interval, 0.38-2.36).
  
  
  CONCLUSIONS
  Small-duct PSC often is recognized at an early stage in patients with IBD; however, IBD has no impact on long-term prognosis. Although UDCA therapy improves liver biochemistries, it may not delay disease progression during the short period of treatment.
authors.affiliation: Division of Gastroenterology and Hepatology, Mayo Clinic and Foundation, Rochester, MN 55905, USA.
authors.forename: Phunchai
authors.initials: P
authors.lastname: Charatcharoenwitthaya
authors.forename: Paul
authors.initials: P
authors.lastname: Angulo
authors.forename: Felicity B
authors.initials: FB
authors.lastname: Enders
authors.forename: Keith D
authors.initials: KD
authors.lastname: Lindor
mesh_terms: D000293:Adolescent; D000328:Adult; D001653:Bile Ducts, Intrahepatic / Q000473:pathology; D002756:Cholagogues and Choleretics / Q000627:therapeutic use*; D002758:Cholangiography; D015209:Cholangitis, Sclerosing / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy* / Q000401:mortality; D005260:Female; D006801:Humans; D015212:Inflammatory Bowel Diseases / Q000150:complications* / Q000473:pathology; D008137:Longitudinal Studies; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D016019:Survival Analysis; D016896:Treatment Outcome; D014580:Ursodeoxycholic Acid / Q000627:therapeutic use*
chemical_list: D002756:Cholagogues and Choleretics; D014580:Ursodeoxycholic Acid