Highlights
- •Comparison of clinico-radiologic parameters between adult and pediatric subjects with Primary Sclerosing Cholangitis (PSC)
- •PSC subjects diagnosed as adults have higher incidence of biliary complications and higher serum bilirubin at diagnosis.
- •PSC subjects diagnosed as adults have higher incidence of intrahepatic biliary disease and the worse severity of intrahepatic disease at diagnosis.
- •We hypothesize that PSC subjects diagnosed as adults may have more advanced disease severity at the time of diagnosis.
Abstract
Introduction
Adult Primary Sclerosing Cholangitis (PSC) subjects have worse outcomes compared to
pediatric PSC subjects. The reasons for this observation are not completely understood.
Methods
In this single-center, retrospective (2005–17) study we compared clinical information,
laboratory data, and previously published MRCP-based scores between 25 pediatric (0–18 years
at diagnosis) and 45 adult (19 years and above) subjects with large duct PSC at the
time of diagnosis. For each subject, radiologists determined MRCP-based parameters
and scores after reviewing the MRCP images.
Results
The median age at diagnosis for pediatric subjects was 14 years, while that of adult
subjects was 39 years. At the time of diagnosis, adult subjects had a higher incidence
of biliary complications like cholangitis and high-grade biliary stricture (27% vs.
6%, p = 0.003) and higher serum bilirubin (0.8 vs. 0.4 mg/dl, p = 0.01). MRCP analysis showed that adult subjects had a higher incidence of hilar
lymph node enlargement (24.4% vs. 4%, p = 0.03) at diagnosis. Adult subjects had worse sum-IHD score (p = 0.003) and average-IHD score (p = 0.03). Age at diagnosis correlated with higher average-IHD (p = 0.002) and sum-IHD (p = 0.002) scores. Adult subjects had worse Anali score without contrast (p = 0.01) at diagnosis. MRCP-based extrahepatic duct parameters and scores were similar
between groups.
Discussion
Adult PSC subjects may have higher severity of disease at diagnosis compared to pediatric
subjects. Future prospective cohort studies are required to confirm this hypothesis.
Keywords
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References
- Sclerosing cholangitis in children and adolescents.Clin Liver Dis. 2016; 20: 99-111
- Diagnostic and therapeutic challenges in pediatric primary sclerosing cholangitis.Liver Transpl. 2012; 18: 277-281
- Pediatric liver transplantation for primary sclerosing cholangitis.Liver Transpl. 2011; 17: 925-933
- OPTN/SRTR 2020 annual data report: liver.Am J Transplant. 2022; 22: 204-309
- The natural history of primary sclerosing cholangitis in 781 children: a multicenter, international collaboration.Hepatology. 2017; 66: 518-527
- Patient age, sex, and inflammatory bowel disease phenotype associate with course of primary sclerosing cholangitis.Gastroenterology. 2017; 152: 1975-1984 e1978
- Diagnosis of primary sclerosing cholangitis beyond childhood is associated with worse outcomes.J Clin Exp Hepatol. 2022; 12: 110-117
- Risk stratification in primary sclerosing cholangitis: it's time to move on from replicating imperfection and break the glass ceiling.J Hepatol. 2019; 71: 867-870
- The Sclerosing Cholangitis Outcomes in Pediatrics (SCOPE) index: a prognostic tool for children.Hepatology. 2021; 73: 1074-1087
- Magnetic resonance imaging in primary sclerosing cholangitis-current state and future directions.Semin Liver Dis. 2019; 39: 369-380
- Magnetic resonance cholangiopancreatography severity predicts disease outcomes in pediatric primary sclerosing cholangitis: a reliability and validity study.Hepatol Commun. 2020; 4: 208-218
- Radiologic course of primary sclerosing cholangitis: assessment by three-dimensional magnetic resonance cholangiography and predictive features of progression.Hepatology. 2014; 59: 242-250
- Magnetic resonance cholangiopancreatography in primary sclerosing cholangitis in children.Pediatr Radiol. 2002; 32: 413-417
- Primary sclerosing cholangitis: a modified classification of cholangiographic findings.AJR Am J Roentgenol. 1991; 157: 495-497
- Longitudinal outcomes in young patients with Alpha-1-antitrypsin deficiency with native liver reveal that neonatal cholestasis is a poor predictor of future portal hypertension.J Pediatr. 2020; 227e84
- Defining primary sclerosing cholangitis: results from an International Primary Sclerosing Cholangitis study group consensus process.Gastroenterology. 2021; 161: 1764-1775 e1765
- Natural history of primary sclerosing cholangitis and prognostic value of cholangiography in a Dutch population.Gut. 2002; 51: 562-566
- Validation of a cholangiographic prognostic model in primary sclerosing cholangitis.Endoscopy. 2010; 42: 742-747
- Correlation between findings on MRCP and gadolinium-enhanced MR of the liver and a survival model for primary sclerosing cholangitis.Dig Dis Sci. 2007; 52: 3499-3506
- The role of magnetic resonance imaging and endoscopic retrograde cholangiography in the evaluation of disease activity and severity in primary sclerosing cholangitis.Liver Int. 2018; 38: 2329-2339
- Lack of correlation of liver tests with fibrosis stage at diagnosis in pediatric primary sclerosing cholangitis.J Pediatr Gastroenterol Nutr. 2018; 66: 227-233
- Simple magnetic resonance scores associate with outcomes of patients with primary sclerosing cholangitis.Clin Gastroenterol Hepatol. 2019; 17: 2785-2792 e2783
- Reporting standards for primary sclerosing cholangitis using MRI and MR cholangiopancreatography: guidelines from MR Working Group of the International Primary Sclerosing Cholangitis Study Group.Eur Radiol. 2022; 32: 923-937
- Diagnosis and management of primary sclerosing cholangitis.Hepatology. 2010; 51: 660-678
- Assessment of prognostic value and interreader agreement of ANALI scores in patients with primary sclerosing cholangitis.Eur J Radiol. 2021; 142109884
- Primary sclerosing cholangitis.N Engl J Med. 2016; 375: 1161-1170
- Clinical features and MRI progression of small duct primary sclerosing cholangitis (PSC).Eur J Radiol. 2020; 129109101
- Cholangiocarcinoma in patients with primary sclerosing cholangitis.Curr Opin Gastroenterol. 2020; 36: 77-84
- Inter-reader agreement of interpretation of radiological course of bile duct changes between serial follow-up magnetic resonance imaging/3D magnetic resonance cholangiopancreatography of patients with primary sclerosing cholangitis.Scand J Gastroenterol. 2020; 55: 228-235
- Evaluation of quantitative MRCP (MRCP+) for risk stratification of primary sclerosing cholangitis: comparison with morphological MRCP, MR elastography, and biochemical risk scores.Eur Radiol. 2022; 32: 67-77
- Emerging artificial intelligence applications in liver magnetic resonance imaging.World J Gastroenterol. 2021; 27: 6825-6843
Article info
Publication history
Published online: February 03, 2023
Accepted:
January 30,
2023
Received in revised form:
January 10,
2023
Received:
October 7,
2022
Identification
Copyright
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