Primary sclerosing cholangitis
ICD-10 K83.0 · ICD-11 DB96.2

Treatment of Primary Sclerosing Cholangitis When Ursodeoxycholic Acid Fails to Normalize ALP

In primary sclerosing cholangitis (PSC), an initial course of ursodeoxycholic acid (UDCA) may be trialled to bring alkaline phosphatase (ALP) — or gamma-glutamyl transferase (GGT) in children — down to acceptable levels. When those biochemical targets are not reached after 12 months, further escalation is required.

Ursodeoxycholic acid (UDCA) did not achieve the required biochemical response within 12 months: a reduction in ALP to below 1.5× ULN, a 40% reduction or normalization of ALP, or — in paediatric patients — a 75% reduction in GGT or a GGT below 50 IU.

For patients in whom prior treatment has not met these biochemical targets, the next step involves a definitive surgical intervention directed at the liver — but the full eligibility criteria, patient selection considerations, and clinical decision pathway are contained in the complete protocol.

References

DOI: 10.1002/hep.32771

Ultimately, LT is recommended for patients with refractory cholangitis and/or decompensated cirrhosis.

LT should be considered in all patients with PSC and complications of end-stage liver disease, recurrent cholangitis, intractable pruritus, or early-stage hepatobiliary cancers.

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