Autoantibodies in Primary Biliary Cholangitis: From Classical Markers to Emerging Targets

Mimura, Shima; Morishita, Asahiro; Oura, Kyoko; Yano, Rie; Nakahara, Mai; Tadokoro, Tomoko; Fujita, Koji; Tani, Joji; Tatsuta, Miwa; Himoto, Takashi; Kobara, Hideki

doi:10.3390/jcm14238503

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Autoantibodies in Primary Biliary Cholangitis: From Classical Markers to Emerging Targets

by

Shima Mimura

^1,*

,

Asahiro Morishita

¹

,

Kyoko Oura

¹

,

Rie Yano

¹,

Mai Nakahara

¹

,

Tomoko Tadokoro

¹

,

Koji Fujita

¹,

Joji Tani

¹,

Miwa Tatsuta

^1,2

,

Takashi Himoto

³ and

Hideki Kobara

¹

Departments of Gastroenterology and Neurology, Faculty of Medicine, Kagawa University, Kagawa 761-0793, Japan

²

Department of Gastroenterology, KKR Takamatsu Hospital, 4-18 Tenjinmae, Kagawa 760-0018, Japan

³

Department of Medical Technology, Kagawa Prefectural University of Health Sciences, Kagawa 761-0123, Japan

^*

Author to whom correspondence should be addressed.

J. Clin. Med. 2025, 14(23), 8503; https://doi.org/10.3390/jcm14238503 (registering DOI)

Submission received: 24 October 2025 / Revised: 25 November 2025 / Accepted: 26 November 2025 / Published: 30 November 2025

(This article belongs to the Special Issue Current Trends and Future Prospects in Autoantibodies in Liver Diseases)

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Abstract

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 (90–95% of cases), AMA-negative PBC (5–10%), 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.

Keywords: primary biliary cholangitis (PBC); autoantibodies; anti-mitochondrial antibody (AMA); antinuclear antibody (ANA); molecular mimicry

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MDPI and ACS Style

Mimura, S.; Morishita, A.; Oura, K.; Yano, R.; Nakahara, M.; Tadokoro, T.; Fujita, K.; Tani, J.; Tatsuta, M.; Himoto, T.; et al. Autoantibodies in Primary Biliary Cholangitis: From Classical Markers to Emerging Targets. J. Clin. Med. 2025, 14, 8503. https://doi.org/10.3390/jcm14238503

AMA Style

Mimura S, Morishita A, Oura K, Yano R, Nakahara M, Tadokoro T, Fujita K, Tani J, Tatsuta M, Himoto T, et al. Autoantibodies in Primary Biliary Cholangitis: From Classical Markers to Emerging Targets. Journal of Clinical Medicine. 2025; 14(23):8503. https://doi.org/10.3390/jcm14238503

Chicago/Turabian Style

Mimura, Shima, Asahiro Morishita, Kyoko Oura, Rie Yano, Mai Nakahara, Tomoko Tadokoro, Koji Fujita, Joji Tani, Miwa Tatsuta, Takashi Himoto, and et al. 2025. "Autoantibodies in Primary Biliary Cholangitis: From Classical Markers to Emerging Targets" Journal of Clinical Medicine 14, no. 23: 8503. https://doi.org/10.3390/jcm14238503

APA Style

Mimura, S., Morishita, A., Oura, K., Yano, R., Nakahara, M., Tadokoro, T., Fujita, K., Tani, J., Tatsuta, M., Himoto, T., & Kobara, H. (2025). Autoantibodies in Primary Biliary Cholangitis: From Classical Markers to Emerging Targets. Journal of Clinical Medicine, 14(23), 8503. https://doi.org/10.3390/jcm14238503

Note that from the first issue of 2016, this journal uses article numbers instead of page numbers. See further details here.

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Autoantibodies in Primary Biliary Cholangitis: From Classical Markers to Emerging Targets

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