{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,25]],"date-time":"2026-07-25T00:03:32Z","timestamp":1784937812394,"version":"3.55.0"},"reference-count":32,"publisher":"Wiley","issue":"6","license":[{"start":{"date-parts":[[2024,7,15]],"date-time":"2024-07-15T00:00:00Z","timestamp":1721001600000},"content-version":"vor","delay-in-days":44,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["J of Digest Diseases"],"published-print":{"date-parts":[[2024,6]]},"abstract":"<jats:sec><jats:title>Objectives<\/jats:title><jats:p>Patients diagnosed with primary sclerosing cholangitis (PSC) but with characteristics of immunoglobulin G4 (IgG4)\u2010associated cholangitis (IAC) have been described. IAC often presents with biliary IgG4\u2010positive plasma cell (IgG4+ PC) infiltration and responds to corticosteroids. In PSC, the frequencies or implications of biliary IgG4+ PC are unknown. We aimed to characterize the phenomenon of biliary IgG4+ PC in patients with an established PSC diagnosis.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Bile duct biopsies from 191 surveillance or therapeutic endoscopic retrograde cholangiography of 58 PSC patients were retrospectively analyzed for IgG4+ PC infiltration. Patients with \u226510 IgG4+ PC per high\u2010power field (HPF) were identified and characterized by clinical parameters, including serum IgG4 and cholangiographic presentations.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Altogether 39.7% of the PSC patients showed \u226510 IgG4+ PC\/HPF in bile duct biopsies. Patients with biliary IgG4+ PC infiltration were significantly younger at diagnosis of PSC (<jats:italic>P<\/jats:italic>\u2009=\u20090.023). There was no association between biliary IgG4+ PC infiltration and transplant\u2010free survival (<jats:italic>P<\/jats:italic>\u2009=\u20090.618). Patients with IgG4+ PC infiltration in bile duct biopsies showed significantly higher baseline (<jats:italic>P<\/jats:italic>\u2009=\u20090.002) and maximum (<jats:italic>P<\/jats:italic>\u2009=\u20090.001) serum IgG4 compared to those without. Biliary IgG4+ PC infiltration was associated with high\u2010grade bile duct strictures (<jats:italic>P<\/jats:italic>\u2009=\u20090.05). IgG4\u2010positive plasma cell infiltrations were found multifocally in 72.7% of this subgroup of PSC patients.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>IgG4+ PC \u226510\/HPF can be found abundantly in bile duct biopsies in PSC. Histological findings correlated with serum IgG4, age, and high\u2010grade bile duct strictures. IgG4+ PC was located multifocally, hinting at a systemic biliary phenotype.<\/jats:p><\/jats:sec>","DOI":"10.1111\/1751-2980.13295","type":"journal-article","created":{"date-parts":[[2024,7,16]],"date-time":"2024-07-16T03:50:42Z","timestamp":1721101842000},"page":"394-403","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["Primary sclerosing cholangitis with <scp>IgG4\u2010p<\/scp>ositive plasma cells in bile duct biopsies \u2015 Frequency and characterization"],"prefix":"10.1111","volume":"25","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-8043-5818","authenticated-orcid":false,"given":"Taotao","family":"Zhou","sequence":"first","affiliation":[{"name":"Department of Internal Medicine I University of Bonn  Bonn Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Florian","family":"Fronhoffs","sequence":"additional","affiliation":[{"name":"Institute of Pathology University of Bonn  Bonn Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Glen","family":"Kristiansen","sequence":"additional","affiliation":[{"name":"Institute of Pathology University of Bonn  Bonn Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Leona","family":"Dold","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine I University of Bonn  Bonn Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dominik J.","family":"Kaczmarek","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine I University of Bonn  Bonn Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Christian P.","family":"Strassburg","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine I University of Bonn  Bonn Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9736-1483","authenticated-orcid":false,"given":"Tobias J.","family":"Weism\u00fcller","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine I University of Bonn  Bonn Germany"},{"name":"Department of Internal Medicine \u2013 Gastroenterology and Oncology Vivantes Humboldt Hospital  Berlin Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2024,7,15]]},"reference":[{"key":"e_1_2_7_2_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.cgh.2021.08.039"},{"key":"e_1_2_7_3_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jhep.2017.07.022"},{"key":"e_1_2_7_4_1","doi-asserted-by":"publisher","DOI":"10.1053\/j.gastro.2017.02.038"},{"key":"e_1_2_7_5_1","doi-asserted-by":"publisher","DOI":"10.1007\/s12016-014-8430-2"},{"key":"e_1_2_7_6_1","doi-asserted-by":"publisher","DOI":"10.1002\/jhbp.596"},{"key":"e_1_2_7_7_1","doi-asserted-by":"publisher","DOI":"10.1002\/hep.26977"},{"key":"e_1_2_7_8_1","doi-asserted-by":"publisher","DOI":"10.1136\/gut.2006.115246"},{"key":"e_1_2_7_9_1","doi-asserted-by":"publisher","DOI":"10.1136\/gutjnl-2012-302886"},{"key":"e_1_2_7_10_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.cgh.2007.12.020"},{"key":"e_1_2_7_11_1","doi-asserted-by":"publisher","DOI":"10.1177\/2050640620934911"},{"key":"e_1_2_7_12_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.cgh.2016.12.038"},{"key":"e_1_2_7_13_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1572-0241.2000.02267.x"},{"key":"e_1_2_7_14_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1572-0241.2000.02323.x"},{"issue":"11","key":"e_1_2_7_15_1","doi-asserted-by":"crossref","first-page":"1135","DOI":"10.1055\/s-0043-120199","article-title":"Practice guideline autoimmune liver diseases \u2010 AWMF\u2010reg. 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