#JustReleased: BSG Guidelines For Diagnosis and Management o
UK-PBC and the British Society of Gastroenterology (BSG) have jointly developed a comprehensive guideline to provide recommendations on the best approaches to the management of Primary biliary cholangitis (formerly known as primary biliary cirrhosis, PBC) an autoimmune liver disease in which a cycle of immune mediated biliary epithelial cell injury, cholestasis and progressive fibrosis can culminate over time in an end-stage biliary cirrhosis. The guidance has been published recently in the BMJ journal GUT.

A series of recommendations have been made to ensure that patients are offered timely licensed therapy (ursodeoxycholic acid (UDCA), obeticholic acid (OCA)) in addition to being actively managed for symptoms as well as complications of progressive liver disease.

The key recommendations are:-
• The presence of antimitochondrial antibodies (>1 in 40) or highly 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, recognising 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. The authors 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, all patients have individualised risk stratification using biochemical response indices following 1 year of UDCA therapy. It is suggested 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 randomised controlled trial evidence for the addition of second-line therapy

• 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 OCA has been associated with improvements in biochemical surrogates of disease activity reasonably likely to predict improved outcomes. Addition of OCA for patients with an inadequate response to UDCA, or intolerant of UDCA, should be considered. Dose adjustment in patients with advanced liver disease as per the drug label

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

• True overlap with autoimmune hepatitis is probably rare and when suspected, liver biopsy with expert clinicopathological review is needed to make the diagnosis and guide treatment

• Patients with PBC should be offered the chance to seek support from patient support groups

About BSG
The British Society of Gastroenterology (BSG) is a British professional organisation of gastroenterologists, focused on the promotion of gastroenterology within the United Kingdom. The society publishes the medical journals Gut and Frontline Gastroenterology. It also produces clinical practice guidelines and various other documents relevant to the field of gastroenterology. This area includes diseases of the gastrointestinal tract, liver, pancreas and biliary tract, and the disciplines of gastrointestinal endoscopy, nutrition, pathology and gastrointestinal surgery.

Note: This list is a brief compilation of some of the key recommendations included in the Guidelines and is not exhaustive and does not constitute medical advice. Kindly refer to the original publication here: https://gut.bmj.com/content/67/9/1568
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