{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,21]],"date-time":"2026-05-21T10:22:03Z","timestamp":1779358923132,"version":"3.51.4"},"reference-count":17,"publisher":"Ovid Technologies (Wolters Kluwer Health)","issue":"3","license":[{"start":{"date-parts":[[2020,1,16]],"date-time":"2020-01-16T00:00:00Z","timestamp":1579132800000},"content-version":"unspecified","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Hepatol Commun"],"published-print":{"date-parts":[[2020,3]]},"abstract":"<jats:p>Patients with primary sclerosing cholangitis (PSC) frequently manifest features of autoimmune hepatitis (AIH). We sought to understand factors affecting expert management, with the goal of facilitating uniformity of care. A Survey Monkey questionnaire with four hypothetical cases suggesting a potential AIH\/PSC variant was sent to hepatologists spanning global practices. Eighty responses from clinicians in 23 countries were obtained. Most of the respondents would request a liver biopsy, and stated that the cases presented could not be appropriately managed without a biopsy. Despite the fact that histology did not unequivocally support an AIH\/PSC variant in three of the four cases, this diagnosis was reached by most of the respondents for all cases, except case 1, in which 49% were diagnosed with AIH\/PSC. There was a wide variation of suggested medical treatment. For three cases, the most commonly chosen treatment options did not exceed 35%, indicating a lack management consensus. Most respondents would treat with ursodeoxycholic acid, despite current American Association for the Study of Liver Diseases guidelines, either alone or in combination with immunosuppression. European clinicians recommended ursodeoxycholic acid more frequently than their counterparts in North America (<jats:italic toggle=\"yes\">P\u00a0<\/jats:italic>&lt;\u00a00.05 in three out of four cases), who advocated the use of immunosuppression alone more commonly than Europeans (<jats:italic toggle=\"yes\">P\u00a0<\/jats:italic>=\u00a00.005 in case 2). <jats:italic toggle=\"yes\">Conclusions:<\/jats:italic> We document a wide variation in clinical decision making in the context of managing patients with a potential AIH\/PSC variant. Guidance, likely based on systematic studies arising from prospective registries, is needed to better address this difficult clinician problem.<\/jats:p>","DOI":"10.1002\/hep4.1467","type":"journal-article","created":{"date-parts":[[2020,1,16]],"date-time":"2020-01-16T16:50:50Z","timestamp":1579193450000},"page":"399-408","source":"Crossref","is-referenced-by-count":11,"title":["Primary Sclerosing Cholangitis With Features of Autoimmune Hepatitis: Exploring the Global Variation in Management"],"prefix":"10.1097","volume":"4","author":[{"given":"Piotr","family":"Milkiewicz","sequence":"first","affiliation":[{"name":"Liver and Internal Medicine Unit,Department of General, Transplant and Liver Surgery,Medical University of Warsaw,Warsaw,Poland"},{"name":"Translational Medicine Group,Pomeranian Medical University in Szczecin,Szczecin,Poland"},{"name":"European Reference Network,Medical University of Warsaw Hospital,Warsaw,Poland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Marcin","family":"Krawczyk","sequence":"additional","affiliation":[{"name":"Laboratory of Metabolic Liver Diseases,Department of General, Transplant and Liver Surgery,Medical University of Warsaw,Warsaw,Poland"},{"name":"Department of Medicine II,Saarland University Medical Center,Saarland University,Homburg,Germany"},{"name":"European Reference Network,Saarland University Medical Centre,Homburg,Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ewa","family":"Wunsch","sequence":"additional","affiliation":[{"name":"Translational Medicine Group,Pomeranian Medical University in Szczecin,Szczecin,Poland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Cyriel","family":"Ponsioen","sequence":"additional","affiliation":[{"name":"Amsterdam University Medical Centers,University of Amsterdam,Amsterdam,Netherlands"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Gideon M.","family":"Hirschfield","sequence":"additional","affiliation":[{"name":"Toronto Center for Liver Disease,University of Toronto,Toronto,ON,Canada"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Stefan G.","family":"Hubscher","sequence":"additional","affiliation":[{"name":"Department of Cellular Pathology,Queen Elizabeth Hospital,Birmingham,United Kingdom"},{"name":"Institute of Immunology and Immunotherapy,University of Birmingham,Birmingham,United Kingdom"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"276","published-online":{"date-parts":[[2020,1,16]]},"reference":[{"key":"hep41467-bib-0001-20240114","doi-asserted-by":"crossref","first-page":"1587","DOI":"10.1016\/S0140-6736(13)60096-3","article-title":"Primary sclerosing 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