A Cochrane review [Abstract] 1 included two studies with a total of 35 subjects. One trial compared biliary lavage with hydrocortisone versus saline in 17 patients. A significant number of adverse events (pancreatitis; cholangitis with septicaemia; paranoid ideas; fluid retention) and no cholangiographic improvement led to termination of the trial. The other trial compared budesonide versus prednisone in 18 patients. Patients had significantly higher serum bilirubin concentration after treatment with prednisone compared with budesonide (WMD 10.4 µmol/litre, 95% CI 1.16 to 19.64 µmol/litre). No other significant effects on clinical or biochemical outcomes were identified for any of the evaluated interventions.
Another Cochrane review [Abstract] 2 included one study comparing D-penicillamine with placebo, with a total of 70 subjects. The trial was of low methodological quality. D-penicillamine therapy had no significant effect on mortality (RR 1.14, 95% CI 0.49 to 2.64), liver transplantation (RR 1.11, 95% CI 0.39 to 3.17), hepatic histologic progression (RR 1.17, 95% CI 0.79 to 1.74), or cholangiographic deterioration (RR 0.87, 95% CI 0.43 to 1.79). D-penicillamine led to a significant improvement in the serum aspartate aminotransferase (WMD -23.00 U/L; 95% CI -30.66 to -15.34), but not in serum bilirubin level (WMD 0.40 mg/l; 95% CI -0.19 to 0.99) and serum alkaline phosphatases activity (WMD 44.00 U/l; 95% CI -37.89 to 125.89). There were significantly more adverse events in patients receiving D-penicillamine (p=0.013).
Comment: The quality of evidence is downgraded by imprecise results (few patients and wide confidence intervals), by limitations in study quality and by inconsistency (heterogeneity in interventions and outcomes, variability in results across studies).
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