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Practice changer · 05 of 05

AASLD–AGA guideline: UDCA first, PPAR agonists second, and linerixibat for itch in PBC

In PBC, start UDCA, assess response and stiffness, and escalate to a PPAR agonist for inadequate response where available.

AASLD and AGA convened a multidisciplinary panel and used GRADE to answer six prioritised questions on primary biliary cholangitis in adults. The six recommendations:

- test PBC-specific antinuclear antibodies when PBC is suspected but antimitochondrial antibody is negative; - use ursodeoxycholic acid as first-line therapy; - use liver stiffness measurement for prognosis; - use peroxisome proliferator-activated receptor (PPAR) agonists for inadequate response to, or intolerance of, UDCA; - use linerixibat for moderate-to-severe cholestatic pruritus; - give UDCA after liver transplantation to reduce recurrence.

Certainty ranged from moderate to very low. The direction is towards risk-based escalation: measure response, stratify with stiffness, and add a second-line agent rather than leaving inadequate responders on UDCA alone. Availability of PPAR agonists and linerixibat in India is limited or unknown, so for many patients the immediately actionable parts are AMA-negative testing, weight-based UDCA, stiffness assessment and post-transplant UDCA.

  • Test PBC-specific antinuclear antibodies when AMA is negative but PBC is suspected
  • Start weight-based UDCA as first-line treatment
  • Use liver stiffness to stratify prognosis
  • Consider a PPAR agonist for inadequate response or intolerance to UDCA where available
  • Give UDCA after transplant to reduce recurrence

Why it matters

It moves PBC care from 'UDCA and wait' to measured, risk-based escalation.

Don't overread it

Several recommendations rest on low or very low certainty evidence.

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