From the Guidelines
The management of primary sclerosing cholangitis (PSC) focuses on symptom control, complication prevention, and liver transplantation for advanced disease, with a strong recommendation against the use of corticosteroids and immunosuppressants for the treatment of classic PSC, as stated in the British Society of Gastroenterology and UK-PSC guidelines 1. The goal of treatment is to alleviate symptoms, prevent complications, and improve quality of life.
- Ursodeoxycholic acid (UDCA) may be used to improve liver biochemistry, although its impact on disease progression remains uncertain, and it is not recommended for the prevention of colorectal cancer or cholangiocarcinoma 1.
- Endoscopic retrograde cholangiopancreatography (ERCP) with balloon dilation and stenting is recommended for dominant strictures causing symptoms or worsening liver function, with a strong recommendation for pathological sampling of suspicious strictures and biliary dilatation preferred to the insertion of biliary stents 1.
- Antibiotics like ciprofloxacin or metronidazole are prescribed for cholangitis episodes, and prophylactic antibiotics are recommended for patients with suspected PSC undergoing ERCP 1.
- Pruritus can be managed with cholestyramine, rifampicin, or naltrexone, and fat-soluble vitamin supplementation and calcium/vitamin D for bone health are important, with a recommendation for all patients with PSC to have a risk assessment for osteoporosis 1. Key aspects of PSC management include:
- Regular screening for complications, including annual colonoscopies for colorectal cancer surveillance and MRCP or ultrasound every 6-12 months for cholangiocarcinoma screening, with a strong recommendation for colonoscopy with biopsies from all colonic segments at the time of PSC diagnosis 1.
- Liver transplantation remains the only definitive treatment for advanced PSC, with 5-year survival rates of 85-90%, although PSC can recur in the transplanted liver, and there is no specific immunosuppressive regimen effective in preventing PSC recurrence 1. The disease pathogenesis involves immune dysregulation, bile acid toxicity, and genetic factors, explaining why immunosuppressive therapies have shown limited efficacy in this progressive condition, and the management of PSC requires a multidisciplinary approach, including hepatologists, biliary endoscopists, and abdominal radiologists, to maximize the benefit and minimize the burden and adverse events of treatments 1.
From the Research
Management of Primary Sclerosing Cholangitis (PSC)
The management of PSC involves a combination of medical and endoscopic therapies. Some key aspects of PSC management include:
- Medical therapy: Ursodeoxycholic acid (UDCA) is the most commonly used medical treatment for PSC, as it has been shown to improve biochemical parameters of cholestasis and liver histology 2, 3.
- Endoscopic therapy: Endoscopic dilation of major duct stenoses is a beneficial treatment for patients with dominant strictures, and can help to relieve cholestasis and improve liver function 2, 4.
- Liver transplantation: Liver transplantation is a successful treatment for end-stage liver disease due to PSC, and can improve survival 5, 6.
Treatment Options
Some treatment options for PSC include:
- Ursodeoxycholic acid (UDCA): UDCA has been shown to improve liver chemistries and histology in PSC patients, but its effect on clinically relevant endpoints is still unclear 2, 3.
- Endoscopic dilation: Endoscopic dilation of major duct stenoses can help to relieve cholestasis and improve liver function 2, 4.
- Liver transplantation: Liver transplantation is a successful treatment for end-stage liver disease due to PSC, and can improve survival 5, 6.
Complications and Prognosis
PSC is associated with several complications, including:
- Cholangiocarcinoma: PSC patients have an increased risk of developing cholangiocarcinoma, a highly malignant tumor 4, 5, 6.
- Liver cirrhosis: PSC can lead to liver cirrhosis, which can result in liver failure and the need for liver transplantation 5, 6.
- The prognosis for PSC patients is variable, with a median survival time of 12-17 years after diagnosis 5, 6.