Tej I Mehta1, Simcha Weissman2, Brian M Fung3, John Sotiriadis4, Keith D Lindor5, James H Tabibian6,7. 1. Department of Radiology, Johns Hopkins University Hospital, Baltimore, MD, USA. 2. Department of Medicine, Hackensack University-Palisades Medical Center, North Bergen, NJ, USA. 3. Division of Gastroenterology and Hepatology, Department of Internal Medicine, University of Arizona College of Medicine-Phoenix, Phoenix, AZ, USA. 4. Division of Gastroenterology and Hepatology, Hackensack University-Palisades Medical Center, North Bergen, NJ, USA. 5. College of Health Solutions, Arizona State University, Phoenix, AZ, USA. 6. Division of Gastroenterology, Department of Medicine, Olive View-UCLA Medical Center, Sylmar, CA, USA. 7. Vatche and Tamar Manoukian Division of Digestive Diseases, David Geffen School of Medicine at UCLA, Los Angeles, CA, USA.
Abstract
BACKGROUND & AIMS: Primary sclerosing cholangitis (PSC) is an idiopathic, cholestatic liver disease with a diverse range of clinical manifestations. Inter-regional data on PSC are variable, but its global geoepidemiology has not been well-studied. We aimed to examine the worldwide incidence, prevalence and features of PSC and PSC-inflammatory bowel disease (PSC-IBD). METHODS: A systematic search of multiple databases was conducted to identify all original, full-text studies until December 2020 with data regarding the incidence rate (IR) and/or prevalence of PSC. Outcomes were PSC IR, prevalence, features and IBD concurrence. Additionally, a meta-analysis of PSC IR was performed. The study was registered in PROSPERO (CRD42021224550). RESULTS: Of the 1003 studies identified, 17 studies spanning three continents were included. PSC IR was 0.60 per 100 000 person-years (PY) (95% confidence interval: 0.37-0.88 per 100 000 PY). In pooled subgroup analysis for studies conducted in Europe and North America, PSC IR was 0.62 and 0.53 per 100 000 PY, respectively. PSC prevalence ranged 0-31.7 per 100 000 persons, with notable inter-regional differences. Mean age at PSC diagnosis was bimodally distributed, with relative peaks at 15 and 35 years. Mean concurrence of IBD with PSC was 50%, with 76% having ulcerative colitis, 17% Crohn's disease and 8% indeterminate/unspecified IBD. CONCLUSION: While considerable heterogeneity exists in the geoepidemiology of PSC, overall, the classical dogmata of male predilection, bimodal distribution of mean age and high PSC-IBD concurrence appear to hold true. Despite a seemingly stable IR over time, further studies are needed to better understand the geoepidemiology of PSC.
BACKGROUND & AIMS:Primary sclerosing cholangitis (PSC) is an idiopathic, cholestatic liver disease with a diverse range of clinical manifestations. Inter-regional data on PSC are variable, but its global geoepidemiology has not been well-studied. We aimed to examine the worldwide incidence, prevalence and features of PSC and PSC-inflammatory bowel disease (PSC-IBD). METHODS: A systematic search of multiple databases was conducted to identify all original, full-text studies until December 2020 with data regarding the incidence rate (IR) and/or prevalence of PSC. Outcomes were PSC IR, prevalence, features and IBD concurrence. Additionally, a meta-analysis of PSC IR was performed. The study was registered in PROSPERO (CRD42021224550). RESULTS: Of the 1003 studies identified, 17 studies spanning three continents were included. PSC IR was 0.60 per 100 000 person-years (PY) (95% confidence interval: 0.37-0.88 per 100 000 PY). In pooled subgroup analysis for studies conducted in Europe and North America, PSC IR was 0.62 and 0.53 per 100 000 PY, respectively. PSC prevalence ranged 0-31.7 per 100 000 persons, with notable inter-regional differences. Mean age at PSC diagnosis was bimodally distributed, with relative peaks at 15 and 35 years. Mean concurrence of IBD with PSC was 50%, with 76% having ulcerative colitis, 17% Crohn's disease and 8% indeterminate/unspecifiedIBD. CONCLUSION: While considerable heterogeneity exists in the geoepidemiology of PSC, overall, the classical dogmata of male predilection, bimodal distribution of mean age and high PSC-IBD concurrence appear to hold true. Despite a seemingly stable IR over time, further studies are needed to better understand the geoepidemiology of PSC.
Authors: Matthew A Morgan; Rachita Khot; Karthik M Sundaram; Daniel R Ludwig; Rashmi T Nair; Pardeep K Mittal; Dhakshina M Ganeshan; Sudhakar K Venkatesh Journal: Abdom Radiol (NY) Date: 2022-09-05