From the Guidelines
Primary sclerosing cholangitis (PSC) should be diagnosed through a combination of clinical presentation, laboratory tests, and imaging studies, with magnetic resonance cholangiopancreatography (MRCP) as the preferred initial imaging study, as recommended by the most recent guidelines 1. The diagnostic process typically begins with laboratory tests showing cholestatic liver enzyme elevations, particularly alkaline phosphatase (ALP) and gamma-glutamyl transferase (GGT), often with mild elevations in aminotransferases (AST, ALT) and bilirubin.
- Key diagnostic features include:
- Cholestatic liver enzyme elevations
- Characteristic multifocal bile duct strictures and dilatations on MRCP
- Exclusion of secondary causes of sclerosing cholangitis
- Additional workup should include:
- Screening for inflammatory bowel disease, particularly ulcerative colitis
- Assessment for complications like cholangiocarcinoma and colorectal cancer in patients with associated inflammatory bowel disease
- The role of endoscopic retrograde cholangiopancreatography (ERCP) is now reserved for cases requiring intervention or when MRCP is inconclusive, due to its procedure-related risks 1.
- Liver biopsy may be necessary in certain cases, such as suspected small duct PSC or overlap variants, but is not always required for diagnosis 1.
- It is essential to note that early diagnosis is crucial for monitoring disease progression and screening for complications, and that patients with PSC should receive lifelong follow-up 1.
From the Research
Diagnosis of Primary Sclerosing Cholangitis
The diagnosis of primary sclerosing cholangitis (PSC) can be confirmed via:
- Cholangiography, either magnetic resonance cholangiography (MRCP) or endoscopic retrograde cholangiography if the former is inconclusive 2
- MRI of the biliary tract is the primary imaging technology for diagnosis 3
- Endoscopic interventions of the bile ducts should be limited to clinically relevant strictures for balloon dilatation, biopsy and brush cytology 3
Key Characteristics for Diagnosis
Key characteristics for diagnosis include:
- A cholestatic picture of liver biochemistries with elevations in serum alkaline phosphatase 4
- Nonspecific autoantibodies such as perinuclear antineutrophilic antibody, antinuclear antibodies and smooth muscle antibodies 4
- Diffuse multifocal biliary strictures, resulting in a 'beaded' appearance on radiographic studies 4
Associated Conditions
PSC is often associated with: