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Untreated, it may run a progressive course to cirrhosis and hepatic decompensation, at which point liver transplantation is the only effective treatment. This review provides discussion of PBC treatment goals in the Australian context. People with PBC often experience a quality of life burden characterized by fatigue and itch. Extra\u2010hepatic associations may include osteoporosis, Sj\u00f6gren's syndrome, and thyroid dysfunction. Established baseline risk factors for progression are male gender, young age at onset (&lt;\u200945\u2009years), ALP &gt;\u20091.5 \u00d7 upper limit of normal (ULN), presence of anti\u2010gp210 antibodies, and significant liver fibrosis at diagnosis. Ursodeoxycholic acid (UDCA) is the mainstay of therapy and improves outcomes including transplant\u2010free survival. Patients with inadequate response to UDCA (about 40%), disease progression, or bothersome symptoms require additional second\u2010line (2L) therapy. Second\u2010line treatments include selective peroxisome proliferator\u2010associated receptor (PPAR) agonists, obeticholic acid (OCA; subject to precautions), and off\u2010label use of fenofibrate. In Australia, reimbursement for 2L therapies relies on ALP \u2265\u20091.67 \u00d7 ULN or total bilirubin between 1 and 2 \u00d7 ULN after 1\u2009year of UDCA therapy. Normalization of ALP is an aspirational treatment target with increasing evidence for improved outcomes. Proactive and diligent monitoring of patients with PBC allows personalized selection and early initiation of effective and well\u2010tolerated 2L therapies with potential for improved outcomes.<\/jats:p>","DOI":"10.1111\/jgh.70616","type":"journal-article","created":{"date-parts":[[2026,8,10]],"date-time":"2026-08-10T03:25:51Z","timestamp":1786332351000},"page":"2623-2638","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Treatment Goals for Primary Biliary Cholangitis, an Australian Perspective"],"prefix":"10.1111","volume":"41","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-6374-1525","authenticated-orcid":false,"given":"Simone\u00a0I.","family":"Strasser","sequence":"first","affiliation":[{"name":"Royal Prince Alfred Hospital  Camperdown New South Wales Australia"},{"name":"University of Sydney  Camperdown New South Wales Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9882-0071","authenticated-orcid":false,"given":"Alexander\u00a0J.","family":"Thompson","sequence":"additional","affiliation":[{"name":"St Vincents Hospital Melbourne  Fitzroy Victoria Australia"},{"name":"University of Melbourne  Melbourne Victoria Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5195-4443","authenticated-orcid":false,"given":"Rohit","family":"Gupta","sequence":"additional","affiliation":[{"name":"Sunshine Coast University Hospital  Birtinya Queensland Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0828-0572","authenticated-orcid":false,"given":"John","family":"Lubel","sequence":"additional","affiliation":[{"name":"Alfred Health Melbourne  Melbourne Victoria Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0630-5916","authenticated-orcid":false,"given":"Janine","family":"French","sequence":"additional","affiliation":[{"name":"The Austin Hospital Melbourne  Heidelberg Victoria Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Beverly","family":"Sacks","sequence":"additional","affiliation":[{"name":"Patient Author  Sydney Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1677-7653","authenticated-orcid":false,"given":"Martin","family":"Weltman","sequence":"additional","affiliation":[{"name":"Nepean Hospital  Penrith New South Wales Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2026,8,9]]},"reference":[{"key":"e_1_2_14_2_1","doi-asserted-by":"publisher","DOI":"10.1002\/hep.30145"},{"key":"e_1_2_14_3_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0140\u20106736(24)01303\u20105"},{"key":"e_1_2_14_4_1","doi-asserted-by":"publisher","DOI":"10.1111\/jgh.14924"},{"key":"e_1_2_14_5_1","doi-asserted-by":"publisher","DOI":"10.1111\/jgh.15329"},{"key":"e_1_2_14_6_1","doi-asserted-by":"publisher","DOI":"10.14309\/ajg.0000000000002070"},{"key":"e_1_2_14_7_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.cgh.2025.03.025"},{"key":"e_1_2_14_8_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0140\u20106736(87)91610\u20102"},{"key":"e_1_2_14_9_1","doi-asserted-by":"publisher","DOI":"10.1136\/gut.32.9.1061"},{"key":"e_1_2_14_10_1","doi-asserted-by":"publisher","DOI":"10.14309\/ajg.0000000000003029"},{"key":"e_1_2_14_11_1","doi-asserted-by":"publisher","DOI":"10.3390\/cells13181580"},{"key":"e_1_2_14_12_1","doi-asserted-by":"publisher","DOI":"10.1080\/17474124.2025.2537192"},{"key":"e_1_2_14_13_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.cgh.2023.02.005"},{"key":"e_1_2_14_14_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jhep.2017.03.022"},{"key":"e_1_2_14_15_1","doi-asserted-by":"publisher","DOI":"10.1038\/s41575\u2010019\u20100226\u20107"},{"key":"e_1_2_14_16_1","doi-asserted-by":"publisher","DOI":"10.1016\/S1089\u20103261(05)70011\u20108"},{"key":"e_1_2_14_17_1","doi-asserted-by":"publisher","DOI":"10.3390\/diagnostics14030265"},{"key":"e_1_2_14_18_1","doi-asserted-by":"publisher","DOI":"10.1055\/s\u20102005\u2010916321"},{"key":"e_1_2_14_19_1","doi-asserted-by":"publisher","DOI":"10.1053\/j.gastro.2012.12.005"},{"key":"e_1_2_14_20_1","doi-asserted-by":"publisher","DOI":"10.1080\/17474124.2021.1945919"},{"issue":"3","key":"e_1_2_14_21_1","first-page":"145","article-title":"A Practical Review of Primary Biliary Cholangitis for the Gastroenterologist","volume":"15","author":"Onofrio F. 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