International Clinical Practice Guidelines: 2018 Midyear Review

John Anello; Brian Feinberg; John Heinegg; Yonah Korngold; Richard Lindsey; Cristina Wojdylo; Olivia Wong, DO


July 10, 2018

In This Article

Biliary Cholangitis

UK-PBC and the British Society of Gastroenterology

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

The presence of antimitochondrial antibodies (>1 in 40) or antinuclear antibodies highly specific for primary biliary cirrhosis (PBC), 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) and 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 alkaline phosphatase (ALP) >1.67 x upper limit of normal (ULN) and/or elevated bilirubin <2 x 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 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 x ULN and/or elevated bilirubin <2 x 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.



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