The key features of the algorithm include new guidance-informed suggestions for staging PBC using noninvasive testing (NIT), earlier assessment of lower thresholds to gauge ursodeoxycholic acid (UDCA) response after initiation of therapy, possible earlier initiation of second-line therapy with obeticholic acid (OCA) at lower levels of alkaline phosphatase (ALP) or bilirubin, avoidance of OCA in patients with cirrhosis complicated by portal hypertension or liver decompensation, and the safety and durability of response with long-term OCA therapy and off-label use of fibrates.
The algorithm is intended for use patients:
All patients that quality, per the criteria above steps, should be started on UDCA 13-15 mg/kg/day for:
Following this treatment, patients should be monitored for response to therapy and UDCA intolerance by following the steps in the algorithm.
Patients with PBC with an inadequate response to or intolerance of UDCA should be considered for second-line therapy with OCA. Second-line therapy consists of OCA, as reviewed in detail above, or off-label bezafibrate or fenofibrate in patients with an inadequate response and if there are no signs of decompensated liver disease or clinically significant portal hypertension.
If the patient has not responded to the first second-line option (OCA or fibrate) after 3-6 months of therapy or the patient is unable to tolerate the selected second-line treatment, then the other second-line option should be considered.
Participation in clinical trials should be discussed and encouraged for appropriate patients who may need additional therapies beyond UDCA, OCA, and off-label fibrates
A referral for liver transplantation evaluation may be appropriate for patients with decompensated cirrhosis, portal hypertension, or significantly affected due to severe pruritus or fatigue even if the Model for End-Stage Liver Disease score is relatively low (< 15). Living donor liver transplants may be an option for some patients.
Kowdley KV, Bowlus CL, Levy C, Mayo MJ, Pratt DS, Vuppalanchi R, Younossi ZM. Application of the Latest Advances in Evidence-Based Medicine in Primary Biliary Cholangitis. Am J Gastroenterol. 2023 Feb 1;118(2):232-242. doi: 10.14309/ajg.0000000000002070. Epub 2022 Sep 21.