Adult Primary Sclerosing Cholangitis (PSC) subjects have worse biliary disease at diagnosis compared to pediatric PSC subjects

• Published in: Clinical imaging Vol. 97; pp. 7 - 13
• Main Authors: Kulkarni, Sakil, Bhimaniya, Sudhir, Chi, Lisa, Tica, Stefani, Alghamdi, Saad, Stoll, Janis, Caudill, Karen, Fleckenstein, Jaquelyn
• Format: Journal Article
• Language: English
• Published: United States Elsevier Inc 01.05.2023
• Subjects: Adolescent; Adult; Anali score; Biliary Tract - pathology; Child; Cholangiopancreatography, Magnetic Resonance - methods; Cholangitis, Sclerosing - diagnostic imaging; Gallbladder Diseases; Humans; Intrahepatic bile ducts; Magnetic Resonance Cholangio-pancreatography; Modified Majoie score; Primary Sclerosing Cholangitis; Retrospective Studies; Magnetic Resonance Cholangio-pancreatography; Anali score; Primary Sclerosing Cholangitis; Intrahepatic bile ducts; Modified Majoie score
• ISSN: 0899-7071; 1873-4499; 1873-4499
• DOI: 10.1016/j.clinimag.2023.01.012

Adult Primary Sclerosing Cholangitis (PSC) subjects have worse outcomes compared to pediatric PSC subjects. The reasons for this observation are not completely understood. 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. 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. 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. •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.