UK_PBC_Risk_Score_guideline v.1

Predicts the risk of liver failure (with transplant need within 5 to 15 years of diagnosis) in patients who suffer from primary biliary cirrhosis (cholangitis) and who are in treatment with the election therapy: Ursodeoxycholic acid (UDCA).

Maryam Razavi

maryam.razavi@cambio.se

© Cambio CDS

To predict the risk of liver failure (with transplant need within 5 to 15 years of diagnosis) in patients who suffer from primary biliary cirrhosis (cholangitis) and who are in treatment with the election therapy: Ursodeoxycholic acid (UDCA).

The variables used in the PBC-UK model are: - 12 month: bilirubin, AST (aspartate aminotransferase) or ALT (alanine aminotransferase) and alkaline phosphatase (considered with upper limit of normal); - Baseline: albumin and platelets (considered with lower limit of normal). The UK-PBC score predicts risk of liver failure at 5, 10 respectively 15 years via: UK-PBC = 1-baseline survival function ^ exp(0.0287854 * (alp12-xuln-1.722136304) - 0.0422873 * (((altast12xuln/10) ^-1) - 8.675729006) + 1.4199 * (ln (bil12xuln/10) + 2.709607778) - 1.960303 * (albxlln-1.17673001) - 0.4161954 * (pltxlln - 1.873564875)) Where: Baseline survivor function is: - 0.982 (at 5 years); - 0.941 (at 10 years); - 0.893 (at 15 years). And: - alp12-xuln is ratio of Alkalin phosphatase measurement at 12 month and its upper limit of normal; - altast12xuln is ratio of either ALT or AST measurement at 12 month and its upper limit of normal; - bil12xuln is ratio of bilirubin measurement at 12 month and its upper limit of normal; - albxlln is ratio of baseline albumin measurement and its lower limit of normal; - pltxlln is ratio of baseline platelet measurement and its lower limit of normal.

Carbone M, Sharp SJ, Flack S, Paximadas D, Spiess K, Adgey C, Griffiths L, Lim R, Trembling P, Williamson K, Wareham NJ, Aldersley M, Bathgate A, Burroughs AK, Heneghan MA, Neuberger JM, Thorburn D, Hirschfield GM, Cordell HJ, Alexander GJ, Jones DE, Sandford RN, Mells GF; UK-PBC Consortium. The UK-PBC risk scores: Derivation and validation of a scoring system for long-term prediction of end-stage liver disease in primary biliary cholangitis. Hepatology. 2016 Mar;63(3):930-50. doi: 10.1002/hep.28017. Epub 2015 Oct 20. PMID: 26223498; PMCID: PMC6984963.

OBSERVATION.lab_test-bilirubin.v1, OBSERVATION.uk_pbc_risk.v0, OBSERVATION.lab_test-liver_function.v1, OBSERVATION.lab_test-full_blood_count.v1