{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,21]],"date-time":"2026-08-21T12:48:27Z","timestamp":1787316507114,"version":"build-2736575974"},"reference-count":117,"publisher":"MDPI AG","issue":"23","license":[{"start":{"date-parts":[[2025,11,30]],"date-time":"2025-11-30T00:00:00Z","timestamp":1764460800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["JCM"],"abstract":"<jats:p>Primary biliary cholangitis (PBC) is a chronic autoimmune liver disease characterized by progressive destruction of intrahepatic bile ducts. PBC encompasses several clinical subtypes, including classical AMA-positive PBC (90\u201395% of cases), AMA-negative PBC (5\u201310%), and overlap syndromes such as AIH-PBC. These subtypes exhibit distinct serological profiles, with AMA-negative cases often presenting PBC-specific antinuclear antibodies (anti-gp210, anti-sp100) and overlap syndromes demonstrating combined autoantibody patterns characteristic of both conditions. Autoantibodies serve as central biomarkers for diagnosis, prognosis, and understanding disease pathogenesis. This review provides a comprehensive overview of classical and emerging autoantibodies associated with PBC, including AMA-M2, anti-gp210, anti-sp100, anti-KLHL12, and anti-RPL30. We discuss their diagnostic significance across PBC subtypes, pathogenic implications, and potential utility in patient stratification and therapeutic monitoring. Recent evidence suggests that bile acid-induced neoantigen formation, rather than classical loss of immune tolerance, may drive AMA production. Advances in autoantibody profiling, including subclass-specific analysis and multi-marker panels, may pave the way for personalized medicine and improved outcomes in PBC.<\/jats:p>","DOI":"10.3390\/jcm14238503","type":"journal-article","created":{"date-parts":[[2025,12,8]],"date-time":"2025-12-08T16:20:29Z","timestamp":1765210829000},"page":"8503","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":3,"title":["Autoantibodies in Primary Biliary Cholangitis: From Classical Markers to Emerging Targets"],"prefix":"10.3390","volume":"14","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-9574-0479","authenticated-orcid":false,"given":"Shima","family":"Mimura","sequence":"first","affiliation":[{"name":"Departments of Gastroenterology and Neurology, Faculty of Medicine, Kagawa University, Takamatsu 761-0793, Kagawa, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0760-3045","authenticated-orcid":false,"given":"Asahiro","family":"Morishita","sequence":"additional","affiliation":[{"name":"Departments of Gastroenterology and Neurology, Faculty of Medicine, Kagawa University, Takamatsu 761-0793, Kagawa, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9531-7240","authenticated-orcid":false,"given":"Kyoko","family":"Oura","sequence":"additional","affiliation":[{"name":"Departments of Gastroenterology and Neurology, Faculty of Medicine, Kagawa University, Takamatsu 761-0793, Kagawa, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rie","family":"Yano","sequence":"additional","affiliation":[{"name":"Departments of Gastroenterology and Neurology, Faculty of Medicine, Kagawa University, Takamatsu 761-0793, Kagawa, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3227-0801","authenticated-orcid":false,"given":"Mai","family":"Nakahara","sequence":"additional","affiliation":[{"name":"Departments of Gastroenterology and Neurology, Faculty of Medicine, Kagawa University, Takamatsu 761-0793, Kagawa, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9975-386X","authenticated-orcid":false,"given":"Tomoko","family":"Tadokoro","sequence":"additional","affiliation":[{"name":"Departments of Gastroenterology and Neurology, Faculty of Medicine, Kagawa University, Takamatsu 761-0793, Kagawa, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Koji","family":"Fujita","sequence":"additional","affiliation":[{"name":"Departments of Gastroenterology and Neurology, Faculty of Medicine, Kagawa University, Takamatsu 761-0793, Kagawa, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Joji","family":"Tani","sequence":"additional","affiliation":[{"name":"Departments of Gastroenterology and Neurology, Faculty of Medicine, Kagawa University, Takamatsu 761-0793, Kagawa, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5726-6905","authenticated-orcid":false,"given":"Miwa","family":"Tatsuta","sequence":"additional","affiliation":[{"name":"Departments of Gastroenterology and Neurology, Faculty of Medicine, Kagawa University, Takamatsu 761-0793, Kagawa, Japan"},{"name":"Department of Gastroenterology, KKR Takamatsu Hospital, 4-18 Tenjinmae, Takamatsu 760-0018, Kagawa, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Takashi","family":"Himoto","sequence":"additional","affiliation":[{"name":"Department of Medical Technology, Kagawa Prefectural University of Health Sciences, Takamatsu 761-0123, Kagawa, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hideki","family":"Kobara","sequence":"additional","affiliation":[{"name":"Departments of Gastroenterology and Neurology, Faculty of Medicine, Kagawa University, Takamatsu 761-0793, Kagawa, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2025,11,30]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"145","DOI":"10.1016\/j.jhep.2017.03.022","article-title":"EASL Clinical Practice Guidelines: The Diagnosis and Management of Patients with Primary Biliary Cholangitis","volume":"67","author":"Hirschfield","year":"2017","journal-title":"J. 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