From the Guidelines
Inflammation and scarring of bile ducts, known as cholangitis or primary sclerosing cholangitis (PSC), requires prompt medical attention with ursodeoxycholic acid (UDCA) at 15-20 mg/kg/d, as it may improve serum liver tests and surrogate markers of prognosis 1.
Diagnosis and Treatment
The diagnosis of PSC should be made in the presence of typical findings of sclerosing cholangitis on high-quality cholangiography, such as magnetic resonance cholangiopancreatography (MRCP), and after exclusion of secondary causes 1.
- A liver biopsy should be performed in adults suspected of having PSC whose high-quality MRCP is normal, to confirm or exclude small duct PSC 1.
- Risk assessment at the time of diagnosis and sequentially is recommended, based on phenotypic factors and non-invasive tests including standard biochemistry and MRI of the liver with MRCP 1.
Management of Complications
For acute bacterial cholangitis, antibiotics like ciprofloxacin 500 mg twice daily or piperacillin-tazobactam 3.375 g IV every 6 hours for 7-14 days are necessary 1.
- Endoscopic retrograde cholangiopancreatography (ERCP) may be needed to relieve bile duct obstructions, while severe cases might require biliary stenting or balloon dilation 1.
- Pharmacological treatment of moderate to severe pruritus in sclerosing cholangitis with bezafibrate or rifampicin is recommended 1.
Monitoring and Follow-up
Regular liver function tests every 3-6 months are essential for monitoring disease progression 1.
- Non-invasive routine liver surveillance is suggested, based on clinical review and standard serum liver tests, every 6 or 12 months depending on risk stratification 1.
- Liver elastography and/or serum fibrosis tests at least every 2 to 3 years are recommended 1.
From the Research
Inflammation and Scarring of Bile Ducts
- Inflammation and scarring of bile ducts, also known as primary sclerosing cholangitis (PSC), is a chronic liver disease characterized by inflammation and scarring of the bile ducts inside and outside the liver 2.
- The efficacy and safety of ursodeoxycholic acid (UDCA) for the treatment of PSC were evaluated in a prospective, randomized, double-blind, placebo-controlled trial, which showed that patients in the UDCA group improved significantly during 1 year of treatment with respect to serum levels of bilirubin, alkaline phosphatase, gamma-glutamyltransferase, AST, and ALT compared with the placebo group 2.
- However, a meta-analysis of randomized clinical trials comparing UDCA with placebo or no treatment found that UDCA had no significant effect on the incidence of death, liver transplantation, and death and/or liver transplantation, but improved liver biochemistry and had a trend towards improvement in liver histology and cholangiography 3, 4.
Treatment Options
- UDCA is the primary medication for the treatment of PSC, but not all patients respond to UDCA 5.
- For patients who are refractory to UDCA, obeticholic acid or a fibrate may be a reasonable choice as an adjunctive treatment to UDCA 5.
- Other medications such as methotrexate, colchicine, budesonide, mycophenolate mofetil, and azathioprine have shown some benefit, but the findings were not consistent and the benefits were marginal 5.
Bile Acid Changes
- High-dose UDCA treatment in PSC patients results in marked UDCA enrichment and significant expansion of the total serum bile acid pool, including lithocholic acid (LCA) 6.
- Patients who reached clinical endpoints of disease progression tended to have greater increases in their posttreatment total bile acid levels, including LCA 6.