Multicenter validation of the DiStrict score, a novel classification and prognostic score for individuals with primary sclerosing cholangitis (PSC)
Author Block: A. Grigoriadis1, S. G. Hamma1, G. Kemmerich2, J. S. Nayagam3, K. Horsthuis4, M-C. Londoño5, D. Assis6, S. Charanjeet7, A. Bergquist1; 1Stockholm/SE, 2Oslo/NO, 3London/UK, 4Amsterdam/NL, 5Barcelona/ES, 6Connecticut, CT/US, 7New Haven, CT/US
Purpose: To validate the reproducibility and prognostic value of the DiStrict-score in a multicenter international cohort.
Methods or Background: The DiStrict-score is an MRCP-based classification of the severity of ductal changes (ranging from 1 to 8) and is reproducible and associated with liver-transplantation and liver-related death. However, it has not been validated. For this retrospective multicenter study with participation of eight international PSC-centers, hepatologists from each center retrieved data of consecutive adult PSC individuals (MRCP, demographics, liver-tests, PSC diagnosis date, hepatobiliary cancer development, liver- transplantation, death, and cause of death). Two radiologists from each center applied the DiStrict-score independently to the patients of their center. Cases of disagreement were resolved in consensus. Interreader agreement was assessed for each pair of radiologists with the intraclass correlation coefficient (ICC), with a two-way random-effects model, absolute-agreement, and single-measurement. The association of the DiStrict-score with outcomes (transplant-free survival, development of hepatobiliary malignancy) was assessed with Cox-regression. Survival estimates were calculated with Kaplan-Meier curves and the curves were compared with the log-rank test.
Results or Findings: 415 patients (271 males, 248 with ulcerative colitis) with median diagnosis age of 39 years, were included. During a median follow-up of 84 months 101 patients developed outcomes (liver-transplantation; n=78, liver-related death; n=10, hepatobiliary cancer; n=13). The interreader agreement ranged between 0.61 and 0.91 for the different centers. Patients with high DiStrict-scores (5–8) had a higher risk of developing outcomes compared to patients with low scores (1–4) (log-rank test; p=0.0008) with a hazard ratio of 1,98 (95%CI; 1.32-2.96, p=0.001).
Conclusion: The DiStrict-sore is reproducible with good interreader agreement and is associated with transplant-free survival and development of hepatobiliary malignancy.
Limitations: The limitations of the study are its retrospective design and the non-standardized MRCP acquisition technique.
Funding for this study: Funding was provided by Medical Diagnostics Karolinska.
Has your study been approved by an ethics committee? Yes
Ethics committee - additional information: Each center obtained ethical approval by local ethical committees .