Study	Reason for exclusion
Charatcharoenwitthaya '08	A longitudinal, cohort study performed to determine the clinical features of small‐duct PSC, as well as to determine the influence of IBD and the use of UDCA on the clinical course of the livers disease. Forty‐two patients with small‐duct PSC were followed for up to 24.9 years. UDCA treatment of 13 to 15 mg/kg body weight/day for an average of 40 months showed biochemical improvement (P < 0.001) in UDCA‐treated patients, while no statistically significant change occurred in untreated patients. However, UDCA therapy did not show a statistically significant effect on disease progression. IBD had no impact on survival or transplantation in small‐duct PSC patients.
Chazouilleres 1990	A case series study that prospectively evaluated the effects of UDCA (dose range 750 to 1250 mg/day) in 15 patients with PSC. A clinical and biochemical evaluation was carried out at 3 and 6 months after treatment initiation. Six months of UDCA treatment resulted in a statistically significant improvement of clinical and biochemical variables.
Garioud 2006	A prospective, multicentre observational study which included 174 patients with PSC of at least four years duration. Study objective was to describe the outcome of PSC under treatment with low dose UDCA. The observed survival was compared to the predicted survival by the revised Mayo model. During the study period 10 patients died and 28 were transplanted. Observed survival and predicted survival were similar.
Gilger 2000	A case series study including ten children with primary sclerosing cholangitis. Data were retrospectively analysed to evaluate the effect of UDCA in a mean dose of 17 mg/kg body weight/day (range from 9 to 37 mg/kg body weight/day). One patient was not included in the analysis because of loss to follow‐up. There were no adverse events from the medication recorded in any of the patients. Results showed a significant reduction in activities of serum ALP, ALT, AST, and GGT after 20‐month treatment period.
Harnois 2001	A case series study including thirty patients with PSC treated with high‐dose UDCA (25 to 30 mg/kg body weight/day) for one year. Changes in the Mayo risk score at 1 year of treatment and projected survival at 4 years were analysed in comparison with a placebo group of patient (n = 52) and a group of patients (n = 53) treated with low‐dose UDCA (13 to 15 mg/kg body weight/day). A marked improvement in serum activities of ALP, AST, and the concentration of albumin and total bilirubin occurred at end of treatment.
O'Brien 1991	A case series study to evaluate the effect of UDCA treatment (10 mg/kg body weight/day) on liver enzyme activity, bilirubin, cholesterol and bile acids levels, and symptoms in patients with PSC. Twelve patients with persistently elevated pretreatment ALP and GGT activities were observed for a median of 37 months. Significant reductions in serum total cholesterol levels and enzyme activities during treatment were found. Improvements continued after two years of treatment in ten patients.
Okolicsanyi 2003	A multicentre retrospective study to evaluate the efficacy of low‐dose UDCA treatment in PSC patients. Data of 86 patients from eight different centres were analysed. Sixty‐nine were treated with UDCA (8 to 13 mg/kg body weight/day) and seventeen were treated symptomatically and served as controls. Treatment effect was determined by symptom analysis and standard liver function tests. Results showed a statistically significant improvement of liver function tests and there was no amelioration of symptoms including fatigue, jaundice and body weight loss.
Rudolph 1991	A case series study of fifteen patients on UDCA therapy (8 to 10 mg/kg body weight/day) followed for two years or longer to assess the effect of UDCA administration on survival in patients with PSC. After two years, one patient died due to bile duct carcinoma, and one liver transplantation was necessary. According to the Mayo risk factors the predicted risk of death did not increase.
Schonfeld 1996	A case series study of eleven patients who received 10 mg/kg body weight/day of UDCA for three to six years to assess the effect of UDCA on liver function. UDCA significantly reduced serum activities of ALP, GGT, AST, and ALT. Parameters of synthetic liver function remained constant during the observation period. Quantitative liver function tests showed little change during the treatment.
van de Meeberg 1996	A case series study that included PSC and PBC patients designed to evaluate the efficacy of a single or multiple dose regimen on liver enzyme activity and serum and biliary bile salts composition. Twenty‐seven patients (19 PSC, 8 PBC) received 10 mg/kg body weight/day of UDCA in a single dose at bed time or in three divided doses with meals over three months. Liver biochemical variables improved equally in both groups.
van Hoogstraten 1998	A multicentre randomised trial assessing the effects of 10 mg/kg body weight/day of UDCA, given in either single or multiple daily doses, on symptoms, serum liver tests, cholangiographic and histological findings, and treatment failure rates. Forty‐eight patients with PSC were enrolled. After two years treatment, no beneficial effects of UDCA on symptoms, liver histology, or mortality were observed. Serum biochemical variables including ALP, GGT, and AST improved significantly in both groups.
van Hoogstraten 2000	An eight‐week randomised double blind pilot study assessing the effects of 9 mg of budesonide (n = 6) versus 3 mg of budesonide (n = 6) or 10 mg of prednisone (n = 6) in patients who had been treated with 12 mg/kg body weight/day of UDCA for at least five months without achieving biochemical remission. Pruritus decreased significantly more in the prednisone group, compared to both the 3 mg and 9 mg budesonide groups. ALP decreased in the prednisone group whereas serum bilirubin, GGT, AST, and ALT did not change significantly.
van Milligen 1999	A case series study evaluating the effect of UDCA treatment in patients with PSC by assessing biochemical variables, immunological markers and inflammation indices. Seventeen PSC patients were enrolled for one year of treatment with UDCA (dose range 12 to 15 mg/kg body weight/day). Treatment with UDCA was associated with significant improvements in serum biochemical liver tests, immunoglobulin levels, and blood coagulation factors.
van Thiel 1992	A randomised clinical trial comparing UDCA with colchicine or no treatment. It was not possible to ascertain from the abstract, which is the only published information on the trial, which treatment the participants of the control group received. A total of 16 control group patients and 32 UDCA treated patients with PSC were studied for a mean of 18.1 months, standard deviation 1.0. UDCA was administered at a dose of 300 mg twice a day. Patients in the control group received either colchicine (0.6 mg twice a day) or no treatment. Patients in the UDCA group experienced a significant reduction in serum bilirubin level. No difference in ALT, AST, ALP, or albumin levels was observed between the two groups and there was no percent change in these parameters compared to baseline.

ALP = alkaline phosphatases. 
 ALT = alanine aminotransferase. 
 AST= aspartate aminotransferase. 
 GGT = gamma glutamyltransferase. 
 PBC = primary biliary cirrhosis. 
 PSC = primary sclerosing cholangitis. 
 UDCA = ursodeoxycholic acid.