From the Guidelines
Primary Sclerosing Cholangitis (PSC) should be diagnosed and treated based on the latest clinical practice guidelines, which recommend a multidisciplinary approach for management, including hepatologists, biliary endoscopists, and abdominal radiologists 1.
Diagnosis
The diagnosis of PSC is based on a combination of clinical, laboratory, imaging, and histological findings.
- A diagnostic work-up for PSC should be performed in all patients with inflammatory bowel disease (IBD) and abnormal liver biochemistry test findings, especially elevated alkaline phosphatase (ALP) and gamma glutamyl transferase (GGT) values, as well as in non-IBD patients with elevated cholestatic liver enzymes not otherwise explained 1.
- Contrast-enhanced, cross-sectional imaging is recommended as the initial diagnostic test when cholangiocarcinoma (CCA) is suspected, potentially followed by ERCP with ductal sampling (brush cytology, endobiliary biopsies) for diagnosis and staging of the suspected CCA 1.
Treatment
The treatment of PSC involves managing symptoms, preventing complications, and potentially considering liver transplantation for advanced disease.
- Pharmacological treatment of moderate to severe pruritus in sclerosing cholangitis with bezafibrate or rifampicin is recommended 1.
- Endoscopic procedures, such as balloon dilatation or stenting, may be necessary to treat dominant strictures and relieve obstructive symptoms 1.
- Bacterial cholangitis episodes require prompt antibiotic therapy, typically with broad-spectrum antibiotics, and subsequent biliary decompression if an underlying relevant stricture is present 1.
- Liver transplantation should be considered for people with PSC and decompensated cirrhosis or hepatocellular carcinoma according to standard guidelines 1.
Surveillance and Monitoring
Regular surveillance and monitoring are crucial for patients with PSC to detect potential complications early.
- Annual surveillance (or every 1 to 2 years in individualized patients without inflammatory activity) colonoscopy with biopsies is recommended in all adult PSC-IBD patients regardless of the duration of IBD or liver transplant status 1.
- Patients should be monitored regularly with liver function tests every 3-6 months to assess disease progression and response to treatment 1.
From the Research
Diagnosis of Primary Sclerosing Cholangitis (PSC)
- The diagnosis of PSC is confirmed by magnetic resonance cholangiopancreaticography [ 2 ]
- Endoscopic retrograde cholangiopancreaticography (ERCP) is performed in patients needing therapeutic endoscopy [ 2 ]
- Laboratory testing with liver chemistries and high-quality cross-sectional imaging with MRCP are used in the diagnosis of PSC [ 3 ]
Treatment of Primary Sclerosing Cholangitis (PSC)
- High-dose ursodeoxycholic acid should be recommended for treatment of PSC patients because there is a trend toward increased survival [ 2 ]
- Dominant bile duct stenoses should be treated endoscopically [ 2 ]
- Liver transplantation continues to be the only therapeutic option for patients with advanced disease [ 2, 4, 5, 6 ]
- Endoscopic techniques such as cholangioscopy and/or intraductal ultrasound may be useful diagnostic tools in patients with stenoses suspicious for malignancy [ 2 ]
Management of Complications
- Cholangiocarcinoma is a major complication of PSC, and early diagnosis is crucial for successful treatment [ 2, 3 ]
- Colorectal cancer is also a significant risk in PSC patients, particularly those with inflammatory bowel disease [ 4, 5 ]
- Recurrence of PSC after liver transplantation is observed in up to 20% of patients [ 5 ]