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The condition is characterised by multi-organ involvement and unpredictable recurrent disease flares, which lead to organ damage and failure in the absence of early recognition and adequate treatment. It is associated with a 2-fold increase in mortality and higher incidences of malignancies, such as solid-organ tumours and lymphoma. Diagnostic delays result from the lack of awareness and the absence of a single diagnostic test, leading to multiple generalist and specialist visits prior to the initiation of therapy. There have been major advances in the management of IgG4-RD to include a move to more targeted therapies. This includes the novel cluster of differentiation 19 (CD19)-depletion therapy inebilizumab, which has been approved in the United States, Europe and Japan for the treatment of IgG4-RD. This review highlights crucial diagnostic clues and emerging therapeutic advances in the evolving field of the Pancreato-Hepato-Biliary subtype of IgG4-RD.<\/jats:p>","DOI":"10.31083\/bjhm55132","type":"journal-article","created":{"date-parts":[[2026,7,7]],"date-time":"2026-07-07T10:55:28Z","timestamp":1783421728000},"source":"Crossref","is-referenced-by-count":0,"title":["Clinical Updates in IgG4-Related Pancreatic and Hepatobiliary Disease"],"prefix":"10.31083","volume":"87","author":[{"ORCID":"https:\/\/orcid.org\/0009-0001-4475-5080","authenticated-orcid":true,"given":"Hoyun","family":"Lee","sequence":"first","affiliation":[{"name":"Department of Medicine, Royal Prince Alfred Hospital, Camperdown, Sydney, NSW 2050, Australia"},{"name":"Faculty of Medicine & Health, Central Clinical School, The University of Sydney, Sydney, NSW 2050, Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6633-9133","authenticated-orcid":true,"given":"Rodrigo Vieira","family":"Motta","sequence":"additional","affiliation":[{"name":"Translational Gastroenterology Unit and Oxford NIHR Biomedical Research Centre, John Radcliffe Hospital, OX3 9DU Oxford, UK"},{"name":"Nuffield Department of Medicine, University of Oxford, OX3 7BN Oxford, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9644-8392","authenticated-orcid":true,"given":"Emma L","family":"Culver","sequence":"additional","affiliation":[{"name":"Translational Gastroenterology Unit and Oxford NIHR Biomedical Research Centre, John Radcliffe Hospital, OX3 9DU Oxford, UK"},{"name":"Nuffield Department of Medicine, University of Oxford, OX3 7BN Oxford, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"16242","published-online":{"date-parts":[[2026,6,22]]},"reference":[{"key":"ref1","doi-asserted-by":"publisher","unstructured":"Deshpande V, Zen Y, Chan JK, Yi EE, Sato Y, Yoshino T, et al. 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