Primary sclerosing cholangitis: best practices for diagnosis

ABSTRACT

Introduction: Primary Sclerosing Cholangitis (PSC) is a chronic disorder with cholestatic features, which is characterized by biliary strictures. The etiology is unknown but inflammation around the biliary tree leads to fibrosis which leads to both intrahepatic and in most cases also extrahepatic biliary strictures. The vast majority of patients have concomitant inflammatory bowel disease (IBD), mostly ulcerative colitis (UC) and more rarely Crohns disease (CD)

Areas covered: This paper reviews the history of diagnostic strategies in PSC and the current gold standard for diagnosis of PSC. The role of liver biopsy as a part of diagnostic strategies in PSC is discussed. The differential diagnoses of PSC are reviewed and in particular causes of secondary sclerosing cholangitis are presented.

Expert opinion: Patients with IBD who develop elevated liver tests with a cholestatic pattern should undergo a MRC as the suspicion of PSC is very high in this clinical situation. If the MRC demonstrates biliary strictures there is a very high likelihood that the patient has PSC if secondary sclerosing cholangitis is excluded. If the patient has characteristic biliary strictures a liver biopsy rarely adds additional information in diagnostic purposes.

KEYWORDS:

• Primary sclerosing cholangitis
• diagnosis
• cholangiography
• liver biopsy
• secondary sclerosing cholangitis

Article highlights

• The gold standard for the diagnosis of Primary Sclerosing Cholangitis is cholangiography

• Magnetic resonance cholangiography (MRC) has been shown to be reliable and should be used for diagnostic purposes and endoscopic retrograde cholangiography (ERC) for therapeutic interventions and for the detection of cholangiocarcinoma

• A liver biopsy does not seem to change management in patients with biliary strictures

• A liver biopsy is necessary to diagnose small-duct PSC and should be undertaken in patients with a clinical and biochemical suspicion of PSC but with a normal cholangiography

• A wide range of conditions need to rule out as causes of secondary sclerosing cholangits, mostly by history taking

• IgG4 associated cholangitis is an important differential diagnosis which requires measurement of IgG4 in serum and these biliary strictures are often responsive to corticosteroids.

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Declaration of interest

The author has no relevant affiliations or financial involvement with any organization of entity with a financial interest in or financial conflict with the subject matter of materials discussed in this manuscript. This includes employment, consultancies, honoraria, stock ownership or options, expert testimony, grants or patents, received of pending or royalties.

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Funding

This paper is not funded