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UK organizations release PBC treatment, management guide

The British Society of Gastroenterology and UK-PBC partnered to develop a comprehensive guideline for the treatment and management of primary biliary cholangitis, which the organizations recently published in Gut.

“Primary biliary cholangitis (formerly known as primary biliary cirrhosis, PBC), is a life-long autoimmune cholestatic liver disease that is a rare but important cause of chronic liver disease,” Gideon M. Hirschfield, MD, PhD, from the University of Birmingham, United Kingdom, and colleagues wrote. “These guidelines are targeted predominantly towards those gastroenterologists and hepatologists leading the care of patients with PBC. However, in addition they will be of value to nurses, primary care physicians and those more broadly involved in patient care, as well as patients themselves.”

Regarding diagnosis, Hirschfield and colleagues advised that the presence of antimitochondrial antibodies or highly PBC-specific antinuclear antibodies — in the proper context of cholestatic liver biochemistry and without alternative explanation — is usually sufficient for a diagnosis of PBC.

All patients diagnosed with PBC should be offered structured life-long follow-up to determine disease course and progression. The researchers recommended serum liver tests and imaging for risk assessment and note that men and patients with disease onset before age 45 years have increased disease-related risks.

The recommended treatment for all patients is oral ursodeoxycholic acid at 13 mg/kg to 15 mg/kg per day as first-line pharmacotherapy. Among patients with inadequate response to UDCA, the addition of obeticholic acid has previously correlated with improvements in biochemical surrogates of disease activity reasonably likely to predict improved outcomes.

Fatigue and itch are key symptoms of PBC. However, severity of symptoms does not correlate with disease stage.

While overlap with autoimmune hepatitis is rare, the researchers recommend a liver biopsy if suspected with expert clinicopathological review to provide diagnosis and guide treatment.

Final recommendations include offering patients with PBC direction to support groups and implementing clinical audit tools to document and improve the quality of care delivered to patients.

“Given the heightened awareness of poorer outcomes, attention should be given to managing high-risk, younger and UDCA non-responsive patients in specialist centers,” the researchers wrote. “Deterioration of PBC can be rapid in the end stages (particularly once a patient is jaundiced) and timely referral for consideration of transplantation, which is an effective treatment for end-stage disease, is essential.” – by Talitha Bennett

Disclosure: Hirschfield reports he is on the advisory boards of Falk, GfK, GlaxoSmithKline, Intercept and Novartis; is a consultant for CymaBay; and is a clinical trial investigator for Falk, FF Pharma, Gilead, GlaxoSmithKline, Intercept, Novartis, NGM Bio and Shire. Please see the full study for the other authors’ relevant financial disclosures.