Development of a prognostic MRCP-score (DiStrict) for individuals with large-duct primary sclerosing cholangitis

Aristeidis Grigoriadis; Kristina Imeen Ringe; Johan Bengtsson; Erik Baubeta; Cecilia Forsman; Nafsika Korsavidou-Hult; Fredrik Rorsman; Emma Nilsson; Nikolaos Kartalis; Annika Bergquist

doi:10.1016/j.jhepr.2022.100595

Development of a prognostic MRCP-score (DiStrict) for individuals with large-duct primary sclerosing cholangitis

JHEP Rep. 2022 Sep 22;4(12):100595. doi: 10.1016/j.jhepr.2022.100595. eCollection 2022 Dec.

Authors

Aristeidis Grigoriadis^{1

2}, Kristina Imeen Ringe³, Johan Bengtsson^{4

5}, Erik Baubeta⁶, Cecilia Forsman⁷, Nafsika Korsavidou-Hult⁸, Fredrik Rorsman⁹, Emma Nilsson¹⁰, Nikolaos Kartalis^{2

11}, Annika Bergquist^{1

12}

Affiliations

¹ Unit of Gastroenterology and Rheumatology, Department of Medicine Huddinge (MedH), Karolinska Institutet, Stockholm, Sweden.
² Department of Radiology, Karolinska University Hospital, Stockholm, Sweden.
³ Hannover Medical School, Department of Diagnostic and Interventional Radiology, Hannover, Germany.
⁴ Department of Clinical Sciences/Division of Radiology, Lund University, Lund, Sweden.
⁵ Center for Medical Imaging and Physiology, Skåne University Hospital, Lund, Sweden.
⁶ Diagnostic Radiology, Department of Translational Medicine, Lund University, Skåne University Hospital, Lund, Sweden.
⁷ Medical Imaging Centre, Uppsala University Hospital, Uppsala, Sweden.
⁸ Radiology & Nuclear Medicine, Department of Surgical Sciences, Uppsala University, Uppsala, Sweden.
⁹ Department of Gastroenterology and Hepatology, Uppsala University Hospital, Uppsala, Sweden.
¹⁰ Division of Gastroenterology and Hepatology, Skåne University Hospital, Lund, Sweden.
¹¹ Division of Radiology, Department of Clinical Science, Intervention and Technology (CLINTEC), Karolinska Institutet, Stockholm, Sweden.
¹² Division of Hepatology, Department of Upper GI Disease, Karolinska University Hospital, Stockholm, Sweden.

Abstract

Background & aims: Magnetic resonance cholangiopancreatography (MRCP) is used for the diagnosis and follow-up of individuals with primary sclerosing cholangitis (PSC). The aim of our study is to develop an MRCP-score based on cholangiographic findings previously associated with outcomes and assess its reproducibility and prognostic value in PSC.

Methods: The score (DiStrict score) was developed based on the extent and severity of cholangiographic changes of intrahepatic and extrahepatic bile ducts (range 0-8) on 3D-MRCP. In this retrospective, multicentre study, three pairs of radiologists with different levels of expertise from three tertiary centres applied the score independently. MRCP examinations of 220 consecutive individuals with PSC from a prospectively collected PSC-cohort, with median follow-up of 7.4 years, were reviewed. Inter-reader and intrareader agreements were assessed via intraclass correlation coefficient (ICC). After consensus, the prognostic value of the score was assessed using Cox-regression and outcome-free survival rates were assessed via Kaplan-Meier estimates. Harrell's C-statistic was calculated.

Results: Forty patients developed outcomes (liver transplantation or liver-related death). Inter-reader agreement between experienced radiologists was good (ICC 0.82; 95% CI 0.74-0.87, and ICC 0.81; 95% CI 0.70-0.87, respectively) and better than the agreement for the pair of experienced/less-experienced radiologists (ICC 0.48; 95% CI 0.05-0.72). Agreement between radiologists from the three centres was good (ICC 0.76; 95% CI 0.57-0.89). Intrareader agreement was good to excellent (ICC 0.85-0.93). Harrell's C was 0.78. Patients with a DiStrict score of 5-8 had 8.2-fold higher risk (hazard ratio 8.2; 95% CI 2.97-22.65) of developing outcomes, and significantly worse survival (p <0.001), compared to those with a DiStrict score of 1-4.

Conclusions: The novel DiStrict score is reproducible and strongly associated with outcomes, indicating its prognostic value for individuals with PSC in clinical practice.

Impact and implications: The diagnosis of primary sclerosing cholangitis (PSC) is based on magnetic resonance cholangiopancreatography (MRCP). However, the role of MRCP in the prognostication of PSC is still unclear. We developed a novel, simple, and reproducible risk-score, based on MRCP findings, that showed a strong association with prognosis in individuals with PSC (DiStrict score). This score can be easily used in clinical practice and thus has the potential to be useful in clinical trials and in patient counselling and management.

Keywords: Bile ducts; CBD, common bile duct; CHD, common hepatic duct; Cholangiopancreatography; Cholangitis sclerosing; EHD, extrahepatic ducts; ERCP, endoscopic retrograde cholangiopancreatography; HR, hazard ratio; ICC, intraclass correlation coefficient; IHD, intrahepatic ducts; LHD, left hepatic duct; MRCP, magnetic resonance cholangiopancreatography; Magnetic resonance; PSC, primary sclerosing cholangitis; Prognosis; RHD, right hepatic duct.