PSC with ‘autoimmune hepatitis (AIH) overlap’, confirmed histologically in those with elevated transaminases and/or immunoglobulin G levels and an abnormal cholangiogram, presents in 5% of patients. ‘Small duct’ PSC that has normal cholangiography but affects only the small intrahepatic bile ducts on liver histology, accounts for 5% of patients. Magnetic resonance imaging/magnetic resonance cholangiopancreatography (MRI/MRCP) is the standard imaging modality to confirm a diagnosis of LD-PSC. It is usually diagnosed on the basis of cholestatic liver biochemistry and characteristic changes in the bile ducts on cholangiography. ‘Classical’ large-duct PSC (LD-PSC), accounting for 90% of patients, involves either the intrahepatic bile ducts, extra hepatic bile ducts or both. Subsequently, four sub-types of PSC were described. A clinical definition for PSC was based upon three landmark papers in the 1980s from the United States, United Kingdom and Norway. The population incidence ranges from 0 to 1.00 persons annually, and the prevalence ranges from 0 to 16.00 persons. It has a male preponderance, with a mean age of diagnosis of 40 years, and a strong association with concomitant inflammatory bowel disease (IBD). Primary sclerosing cholangitis (PSC) is a rare, chronic, immune-mediated liver disease, characterised by intrahepatic and extrahepatic bile duct inflammation, leading to chronic cholestasis, biliary fibrosis and liver cirrhosis with portal hypertension. This review article will discuss how the role of MR techniques have evolved over the last three decades from emerging as an alternative diagnostic tool to endoscopic retrograde cholangiopancreatography, to being instrumental in the ongoing search for imaging biomarker of disease stage, progression and prognosis in PSC. The progression from a qualitative to quantitative approach in MR acquisition techniques and data interpretation, offers the opportunity for the development of objective and reproducible imaging biomarkers that can potentially be incorporated as an additional endpoint in clinical trials. Magnetic resonance (MR) techniques have undergone significant advancement over the last three decades both in MR data acquisition and interpretation. Magnetic resonance imaging/magnetic resonance cholangiopancreatography (MRI/MRCP) continue to play a central role in the diagnosis and follow-up of PSC patients.
In addition to serum and histological biomarkers, there has been much recent interest in developing imaging biomarkers that can predict disease course and clinical outcomes in PSC. Development of non-invasive methods to risk-stratify patients and predict clinical endpoints have been identified as one of the key research priorities in primary sclerosing cholangitis (PSC).
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