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We aimed to examine the worldwide incidence, prevalence and features of PSC and PSC\u2010inflammatory bowel disease (PSC\u2010IBD).<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>A systematic search of multiple databases was conducted to identify all original, full\u2010text 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\u2010analysis of PSC IR was performed. The study was registered in PROSPERO (CRD42021224550).<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Of the 1003 studies identified, 17 studies spanning three continents were included. PSC IR was 0.60 per 100\u00a0000 person\u2010years (PY) (95% confidence interval: 0.37\u20100.88 per 100\u00a0000 PY). In pooled subgroup analysis for studies conducted in Europe and North America, PSC IR was 0.62 and 0.53 per 100\u00a0000 PY, respectively. PSC prevalence ranged 0\u201031.7 per 100\u00a0000 persons, with notable inter\u2010regional differences. Mean age at PSC diagnosis was bimodally distributed, with relative peaks at 15 and 35\u00a0years. Mean concurrence of IBD with PSC was 50%, with 76% having ulcerative colitis, 17% Crohn's disease and 8% indeterminate\/unspecified IBD.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>While considerable heterogeneity exists in the geoepidemiology of PSC, overall, the classical dogmata of male predilection, bimodal distribution of mean age and high PSC\u2010IBD concurrence appear to hold true. Despite a seemingly stable IR over time, further studies are needed to better understand the geoepidemiology of PSC.<\/jats:p><\/jats:sec>","DOI":"10.1111\/liv.15007","type":"journal-article","created":{"date-parts":[[2021,7,5]],"date-time":"2021-07-05T13:47:38Z","timestamp":1625492858000},"page":"2418-2426","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":62,"title":["Global incidence, prevalence and features of primary sclerosing cholangitis: A systematic review and meta\u2010analysis"],"prefix":"10.1111","volume":"41","author":[{"given":"Tej I.","family":"Mehta","sequence":"first","affiliation":[{"name":"Department of Radiology Johns Hopkins University Hospital Baltimore MD USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0796-6217","authenticated-orcid":false,"given":"Simcha","family":"Weissman","sequence":"additional","affiliation":[{"name":"Department of Medicine Hackensack University\u2010Palisades Medical Center North Bergen NJ USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Brian M.","family":"Fung","sequence":"additional","affiliation":[{"name":"Division of Gastroenterology and Hepatology Department of Internal Medicine University of Arizona College of Medicine\u2010Phoenix Phoenix AZ USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"John","family":"Sotiriadis","sequence":"additional","affiliation":[{"name":"Division of Gastroenterology and Hepatology Hackensack University\u2010Palisades Medical Center North Bergen NJ USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1046-5621","authenticated-orcid":false,"given":"Keith D.","family":"Lindor","sequence":"additional","affiliation":[{"name":"College of Health Solutions Arizona State University Phoenix AZ USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9104-1702","authenticated-orcid":false,"given":"James H.","family":"Tabibian","sequence":"additional","affiliation":[{"name":"Division of Gastroenterology Department of Medicine Olive View\u2010UCLA Medical Center Sylmar CA USA"},{"name":"Vatche and Tamar Manoukian Division of Digestive Diseases, David Geffen School of Medicine at UCLA Los Angeles CA USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2021,7,19]]},"reference":[{"key":"e_1_2_10_2_1","doi-asserted-by":"publisher","DOI":"10.1002\/hep.26565"},{"key":"e_1_2_10_3_1","doi-asserted-by":"publisher","DOI":"10.1002\/hep.24247"},{"key":"e_1_2_10_4_1","doi-asserted-by":"publisher","DOI":"10.1055\/s-2006-933565"},{"key":"e_1_2_10_5_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jhep.2013.03.015"},{"key":"e_1_2_10_6_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.livres.2017.12.002"},{"key":"e_1_2_10_7_1","doi-asserted-by":"publisher","DOI":"10.1111\/apt.12232"},{"key":"e_1_2_10_8_1","doi-asserted-by":"publisher","DOI":"10.1186\/s12874-016-0165-8"},{"key":"e_1_2_10_9_1","doi-asserted-by":"publisher","DOI":"10.1097\/EDE.0b013e3181fe7825"},{"key":"e_1_2_10_10_1","doi-asserted-by":"publisher","DOI":"10.1001\/jama.283.15.2008"},{"key":"e_1_2_10_11_1","doi-asserted-by":"publisher","DOI":"10.1136\/bmj.j4008"},{"key":"e_1_2_10_12_1","unstructured":"MoherD.International prospective register of systematic reviews. 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