Cholangitis Guidelines

Updated: Mar 11, 2020
  • Author: Homayoun Shojamanesh, MD; Chief Editor: Vinay K Kapoor, MBBS, MS, FRCSEd, FICS, FAMS  more...
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Biliary Cholangitis

UK-PBC and the British Society of Gastroenterology

In March 2018, the British Society of Gastroenterology/UK-PBC released guidelines for the treatment and management of primary biliary cholangitis. [17]  These guidelines build from previous guidelines from the European Association for the Study of the Liver (EASL) and the American Association for the Study of Liver Diseases (AASLD).

Recommend that any patient with persistently elevated cholestatic liver biochemistry (raised alkaline phosphatase [ALP] or gamma-glutamyl transferase [GGT]) without an alternative cause should have autoantibodies checked for anti-mitochondrial (AMA) and anti-nuclear (ANA) reactivity.

The presence of antimitochondrial antibodies (>1 in 40) or highly primary biliary cirrhosis (PBC)-specific antinuclear antibodies, in the appropriate context of cholestatic liver biochemistry, without alternative explanation, is usually sufficient for confidently reaching the diagnosis of PBC.

All patients with PBC should be offered structured life-long follow-up, recognizing that different patients have different disease courses and may require different intensity of follow-up.

Risk assessment should evaluate disease severity and activity at baseline and on treatment. We recommend a combination of serum liver tests (to identify those with an elevated bilirubin, a platelet count < 150, or biochemical disease activity on treatment), imaging (liver ultrasound to identify overt cirrhosis and splenomegaly; transient elastography (to identify increased liver stiffness), recognition of young age at disease onset (< 45 years), and male sex.

To identify those at greatest risk of disease progression, we recommend that all patients have individualized risk stratification using biochemical response indices following 1 year of ursodeoxycholic acid (UDCA) therapy. We suggest that UDCA-treated patients with an ALP >1.67 × upper limit of normal (ULN) and/or elevated bilirubin < 2 × ULN represent a group of high-risk patients in whom there is randomized controlled trial evidence for the addition of second-line therapy.

Recommend oral UDCA at 13–15 mg/kg/day be used as the first-line pharmacotherapy in all patients with PBC. If tolerated, treatment should usually be life-long.

In patients with inadequate response to UDCA (or UDCA intolerance) as defined by ALP >1.67 × ULN and/or elevated bilirubin < 2 × ULN, the addition of obeticholic acid (OCA) has been associated with improvements in biochemical surrogates of disease activity reasonably likely to predict improved outcomes. We therefore recommend, in keeping with the National Institute for Health and Care Excellence (NICE) evaluation of OCA, that the addition of OCA for patients with an inadequate response to UDCA, or intolerant of UDCA, is considered. We recommend dose adjustment in patients with advanced liver disease as per the drug label.

Recommend that all patients be evaluated for the presence of symptoms, particularly fatigue and itch. Clinicians should recognize that severity of symptoms does not correlate with stage of disease.

True overlap with autoimmune hepatitis is probably rare, and we suggest that, when suspected, liver biopsy with expert clinicopathologic review is needed to make the diagnosis and guide treatment.

Recommend that patients with PBC be offered the chance to seek support from patient support groups.