From the Guidelines
Primary biliary cholangitis (PBC) and primary sclerosing cholangitis (PSC) are detected through a combination of blood tests, imaging, and sometimes liver biopsy, with magnetic resonance cholangiopancreatography (MRCP) being the preferred diagnostic test for PSC, as recommended by the most recent guidelines 1. To detect PBC and PSC, the following steps can be taken:
- For PBC, diagnosis typically begins with elevated alkaline phosphatase (ALP) and the presence of antimitochondrial antibodies (AMA), which are positive in about 95% of cases.
- For PSC, diagnosis relies on elevated liver enzymes (particularly ALP and GGT) and characteristic findings on cholangiography, typically MRCP, which shows multifocal strictures and dilations of bile ducts creating a "beaded" appearance.
- MRCP is preferred over endoscopic retrograde cholangiopancreatography (ERCP) as the primary diagnostic modality for PSC, due to its non-invasive nature and lower risk of complications 1.
- Liver biopsy may be needed in unclear cases of both conditions, showing bile duct damage and fibrosis.
- PSC patients should undergo colonoscopy to screen for inflammatory bowel disease, which is associated in 50-80% of cases, as mentioned in the guidelines 1. The most recent guidelines from 2022 emphasize the importance of MRCP in diagnosing PSC, with a strong recommendation and high consensus among experts 1.
Key points to consider:
- Elevated serum liver tests, especially serum alkaline phosphatase (ALP), should raise suspicion for PSC and trigger further evaluation 1.
- MRCP can be used as a quick standalone non-contrast test to diagnose PSC, but performing a more complete, high-quality MRI evaluation will also provide information on bile duct thickness and enhancement, the status of hepatic parenchyma, and complications of liver disease 1.
- The classic features of PSC are multifocal strictures and dilatations or ectasias involving the intra- and/or extrahepatic biliary tree, which can be visualized using MRCP 1.
From the Research
Detection of Primary Biliary Sclerosis and Primary Sclerosing Cholangitis
To detect primary biliary sclerosis and primary sclerosing cholangitis, the following methods can be used:
- Magnetic resonance cholangiopancreatography (MRCP) is the recommended initial diagnostic test for primary sclerosing cholangitis (PSC) 2, 3, 4
- MRCP typically shows bile duct wall thickening, focal bile duct dilatation, and saccular dilatation of the intra- and/or extrahepatic bile ducts 2
- Endoscopic retrograde cholangiography (ERCP) can be used if MRCP is inconclusive 4
- Liver biopsy may be necessary in some cases, especially for proper management of specific subgroups or variants of primary biliary cholangitis (PBC) and PSC 5
- Blood tests, such as alkaline phosphatase and bilirubin, can be used to monitor disease progression and predict survival in PBC patients 2
- Immunoglobulin 4-associated cholangitis must be excluded when considering the diagnosis of PSC to allow for proper treatment and monitoring of disease progression 2
Key Characteristics of Primary Biliary Sclerosis and Primary Sclerosing Cholangitis
Some key characteristics of primary biliary sclerosis and primary sclerosing cholangitis include:
- Primary biliary cirrhosis (PBC) and primary sclerosing cholangitis (PSC) are chronic, cholestatic diseases of the liver with common clinical manifestations 2, 6
- PBC and PSC can progress to liver failure and may require liver transplantation 6, 3, 4
- PSC is a pre-malignant condition and close surveillance is indicated to monitor for malignancies, such as cholangiocellular carcinoma 2, 3, 4
- Ursodeoxycholic acid (UDCA) is the recommended treatment for PBC, while no medical therapy has been approved for PSC 2, 6, 3