Background & Aims: Primary sclerosing cholangitis (PSC) has a variable, often progressive course. Magnetic resonance cholangiography (MRC) is used in diagnosis of PSC. Magnetic resonance risk scoring systems, called Anali without and with gadolinium, are used to predict disease progression, determined by radiologic factors. We aimed to assess the prognostic value of Anali scores in patients with PSC and validate our findings in a separate cohort.
Methods: We performed a retrospective study of patients with large-duct PSC (internal cohort, 119 patients in France and external cohort, 119 patients in Canada, Italy, and United Kingdom). All the first-available MRC results were reviewed by 2 radiologists and the Anali scores were calculated: Anali without gadolinium= (1x dilatation of intrahepatic bile ducts) + (2x dysmorphy) + (1x portal hypertension); Anali with gadolinium= (1x dysmorphy) + (1x parenchymal enhancement heterogeneity). The primary endpoint was survival without liver transplantation or cirrhosis decompensation. The prognostic value of Anali scores was assessed by Cox regression modeling.
Results: During a total of 549 patient-years for the internal cohort and 497 patient-years for the external cohort, we recorded 2 and 8 liver transplantations, 4 and 3 liver-related deaths, 26 and 25 cirrhosis decompensations, respectively. In the univariate analysis, factors associated with survival without liver transplantation or cirrhosis decompensation in the internal cohort were: serum levels of bilirubin, aspartate aminotransferase, alanine aminotransferase, gamma-glutamyl transferase, alkaline phosphatase, and albumin and Anali scores. Anali scores without and with gadolinium identified patients survival without liver transplantation or cirrhosis decompensation with a c-statistic of 0.89 (95% CI, 0.84-0.95) and 0.75 (95% CI, 0.64-0.87), respectively. Independent prognostic factors identified by multivariate analysis were Anali scores and bilirubinemia. The prognostic value of Anali scores was confirmed in the external cohort.
Conclusions: In internal and external cohorts, we found that Anali scores, determined from MRC, associate with outcomes of patients with PSCs. These scores might be used as prognostic factors.