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New PSC risk scores predict mortality, transplant outcomes up to 10 years

Researchers developed and validated new risk scores for primary sclerosing cholangitis that predicted either 2-year or 10-year outcomes and out-performed both Mayo and Aspartate Aminotransferase-to-Platelet Ratio Index scores, according to recently published data.

“Primary sclerosing cholangitis (PSC) is a chronic fibrosing cholestatic liver disease frequently associated with inflammatory bowel disease (IBD),” Elizabeth C. Goode, MD, from Norfolk and Norwich University Hospital in the United Kingdom, and colleagues wrote. “Clinical course is variable and efforts to individualize risk prediction are important for patients, clinicians and trials of experimental agents.”

The researchers recruited 1,001 patients to the UK-PSC cohort study. Over a cumulative follow-up period of 7,904 years, 35.7% of patients reached a primary endpoint of liver transplantation or mortality.

Results showed that elevated serum alkaline phosphatase correlated with an increased 10-year risk for transplantation or mortality (P .001). Patients with an ALP less than 2.4 times the upper limit of normal had longer median transplant-free survival times than those with a higher ALP (108 vs. 63 months; P < .0001).

The presence of extrahepatic biliary disease also correlated with adverse outcomes (HR = 1.45; 95% CI, 1.09-1.92), as those without extrahepatic biliary disease had improved 10-year event-free survival.

With these data, Goode and colleagues developed the “Short-term UK-PSC Risk Score” for 2-year outcomes that included bilirubin, albumin, hemoglobin and platelet count.

They also developed the “Long-term UK-PSC Risk Score” for 10-year outcomes that included age and presence of extrahepatic biliary disease at diagnosis, and bilirubin, ALP, albumin, platelet count and variceal hemorrhage at 2 years postdiagnosis.

The short-term score (C = 0.81) outperformed the APRI (C = 0.63) and Mayo scores (C = 0.75) for predicting 2-year outcomes. Similarly, the long-term scores (C = 0.8) outperformed the APRI (C = 0.59) and Mayo scores (C = 0.79) for 10-year outcomes.

“Our analyses based on a detailed clinical evaluation of a large representative cohort of participants with PSC has furthered our understanding of clinical risk markers for predicting outcome in patients with PSC,” the researchers concluded. “The risk score analyses best reflect collective real-world clinical practice, with a focus on a spectrum of patients reflective of the disease, and a study design that through large cohort size, accommodates the weaknesses introduced by non-trial based uniform evaluation and data capture.” – by Talitha Bennett

Disclosure: The authors report no relevant financial disclosures.