Primary biliary cholangitis
ICD-10 K74.3ICD-11 DB96.1

Primary Biliary Cholangitis: Management When UDCA Therapy Has Not Achieved Targets

Not all patients with primary biliary cholangitis (PBC) achieve adequate biochemical control on first-line therapy. When the initial treatment fails to bring liver biochemistry within acceptable limits, escalation to a second-line protocol is required.

First-line therapy with oral ursodeoxycholic acid (UDCA), taken continuously for life, did not achieve the required outcomes: transplant-free survival comparable to a healthy population, with ALP below 1.5 × ULN and a normal bilirubin after one year of treatment.

The treatment targets attainment of an ALP value below 1.67 × ULN — with a reduction of at least 15% from baseline — and a normal serum bilirubin level, both assessed at 12 months of therapy.

This protocol introduces a licensed second-line oral agent, used in combination with UDCA or as monotherapy in patients who cannot tolerate UDCA. Off-label adjunctive options in non-responding patients are also covered. Full regimen, sequencing, eligibility criteria, and dose adjustments are available in the complete protocol below.