Primary Biliary Cirrhosis and Primary Sclerosing Cholangitis: an Update on MR Imaging Findings with Recent Developments

Abstract

Primary sclerosing cholangitis (PSC) and primary biliary cirrhosis (PBC) are the most common immune-mediated chronic cholestatic liver diseases leading to cirrhosis and liver failure. Although magnetic resonance imaging (MRI) is not a necessary procedure for the diagnosis of PBC, MRI is recommended for monitoring disease progression and early detection of complications. Even though liver cirrhosis subtypes have similar MR imaging features, there are some findings which could indicate PBC, such as the periportal halo sign. Additionally, MRI using diffusion-weighted imaging with apparent diffusion coefficient measurements provides non-invasive assessment of the stage of liver fibrosis. The role of cholangiography is crucial for the diagnosis of PSC. Since endoscopic retrograde cholangiography is an invasive procedure with occasional post-procedural complications, the latest guidelines suggest magnetic resonance cholangiography as a reference procedure for evaluation of patients suspected with PSC. Characteristic magnetic resonance cholangiography findings include multiple segmental strictures with slightly dilated ducts among them, usually on both intrahepatic and extrahepatic bile ducts. Furthermore, magnetic resonance cholangiography is useful in the follow-up of these patients, allowing for timely diagnosis of complications such as cholangiocellular carcinoma. With the exception of ursodeoxycholic acid, which slows the progression of PBC, the only curative treatment for both PSC and PBC is still liver transplantation. However, recurrent disease occurs in some patients indicating the need for development of new more effective therapies.

Clinical Updates in Primary Biliary Cholangitis: Trends, Epidemiology, Diagnostics, and New Therapeutic Approaches

“…These imaging modalities are not required but can aid in the exclusion of patients with other cholestatic liver diseases, such as primary sclerosing cholangitis (PSC), and to rule out concomitant pancreatic or biliary masses causing biliary obstruction. 2,56 There are also newer imaging modalities, such as transient elastography, that evaluate disease progression, prognosis, and treatment response. Transient elastography-derived liver stiffness measurement has been used as a surrogate marker of liver fibrosis in PBC patients, allowing noninvasive monitoring over time.…”

“…Progression of liver stiffness over time is predictive of poorer outcomes, and patients with response to UDCA showed improvement in liver stiffness scores. 54,56 The authors also purported that transient elastography is superior in determining liver fibrosis compared to using biochemical testing alone, which is important in the prognostication of PBC patients over time.…”

Review article: pathophysiology and management of primary biliary cholangitis

“…Ancillary imaging tests such as ultrasound are sufficient to rule out biliary tree obstruction. Although magnetic resonance cholangiopancreatography (MRCP) will distinguish PBC from sclerosing cholangitis (primary or secondary), it is not needed routinely, and indeed inappropriate MR imaging can cause unnecessary confusion 47 . Whilst histological confirmation may be appealing to clinicians (but not to patients concerned about the small risks of biopsy), the strong association and positive predictive ability of AMA positivity with cholestasis means routine diagnostic liver biopsy is not indicated.…”

Cholangitis: Diagnosis, Treatment and Prognosis

“… 42 Considering the invasive nature of ERCP and its related complications, MRCP is gaining more and more pros as the first line assessment procedure in suspected PSC. 63 MRCP is also an effective method to follow up the patients, and for screening to provide timely diagnosis of complications. 63 …”

“… 63 MRCP is also an effective method to follow up the patients, and for screening to provide timely diagnosis of complications. 63 …”