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The challenges of primary biliary cholangitis: What is new and what needs to be done
Open accessAbstract
Primary Biliary Cholangitis (PBC) is an uncommon, chronic, cholangiopathy of autoimmune origin and unknown etiology characterized by positive anti-mitochondrial autoantibodies (AMA), female preponderance and progression to cirrhosis if left untreated. The diagnosis is based on AMA- or PBC-specific anti-nuclear antibody (ANA)-positivity in the presence of a cholestatic biochemical profile, histologic confirmation being mandatory only in seronegative cases. First-line treatment is ursodeoxycholic acid (UDCA), which is effective in preventing disease progression in about two thirds of the patients. The only approved second-line treatment is obeticholic acid. This article summarizes the most relevant conclusions of a meeting held in Lugano, Switzerland, from September 23rd-25th 2018, gathering basic and clinical scientists with various background from around the world to discuss the latest advances in PBC research. The meeting was dedicated to Ian Mackay, pioneer in the field of autoimmune liver diseases. The role of liver histology needs to be reconsidered: liver pathology consistent with PBC in AMA-positive individuals without biochemical cholestasis is increasingly reported, raising the question as to whether biochemical cholestasis is a reliable disease marker for both clinical practice and trials. The urgent need for new biomarkers, including more accurate markers of cholestasis, was also widely discussed during the meeting. Moreover, new insights in interactions of bile acids with biliary epithelia in PBC provide solid evidence of a role for impaired epithelial protection against potentially toxic hydrophobic bile acids, raising the fundamental question as to whether this bile acid-induced epithelial damage is the cause or the consequence of the autoimmune attack to the biliary epithelium. Strategies are needed to identify difficult-to-treat patients at an early disease stage, when new therapeutic approaches targeting immunologic pathways, in addition to bile acid-based therapies, may be effective. In conclusion, using interdisciplinary approaches, groundbreaking advances can be expected before long in respect to our understanding of the etiopathogenesis of PBC, with the ultimate aim of improving its treatment.
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Citations
Cites
Fate Mapping Analysis Reveals That Adult Microglia Derive from Primitive Macrophages
Ginhoux, F., Greter, M., Leboeuf, M., Nandi, S., See, P., Gokhan, S., Mehler, M. F., Conway, S. J., Ng, L. G., Stanley, E. R., et al. (2010) · Science · doi:10.1126/science.1194637 · Cited by 5,175
The Human Intestinal Microbiome in Health and Disease
Lynch, S. V., & Pedersen, O. (2016) · New England Journal of Medicine · doi:10.1056/nejmra1600266 · Cited by 3,637
Gut microbiota composition correlates with diet and health in the elderly
Claesson, M. J., Jeffery, I. B., Conde, S., Power, S. E., O’Connor, E. M., Cusack, S., Harris, H. M. B., Coakley, M., Lakshminarayanan, B., O’Sullivan, O., et al. (2012) · Nature · doi:10.1038/nature11319 · Cited by 3,314
Fate Mapping Reveals Origins and Dynamics of Monocytes and Tissue Macrophages under Homeostasis
Yona, S., Kim, K.-W., Wolf, Y., Mildner, A., Varol, D., Breker, M., Strauss-Ayali, D., Viukov, S., Guilliams, M., Misharin, A., et al. (2013) · Immunity · doi:10.1016/j.immuni.2012.12.001 · Cited by 3,245
An Expanded View of Complex Traits: From Polygenic to Omnigenic
Boyle, E. A., Li, Y. I., & Pritchard, J. K. (2017) · Cell · doi:10.1016/j.cell.2017.05.038 · Cited by 2,851
A Lineage of Myeloid Cells Independent of Myb and Hematopoietic Stem Cells
Schulz, C., Perdiguero, E. G., Chorro, L., Szabo-Rogers, H., Cagnard, N., Kierdorf, K., Prinz, M., Wu, B., Jacobsen, S. E. W., Pollard, J. W., et al. (2012) · Science · doi:10.1126/science.1219179 · Cited by 2,578
Batf3 Deficiency Reveals a Critical Role for CD8α + Dendritic Cells in Cytotoxic T Cell Immunity
Hildner, K., Edelson, B. T., Purtha, W. E., Diamond, M., Matsushita, H., Kohyama, M., Calderon, B., Schraml, B. U., Unanue, E. R., Diamond, M. S., et al. (2008) · Science · doi:10.1126/science.1164206 · Cited by 2,081
Kaplan, M. M. (1987) · New England Journal of Medicine · doi:10.1056/nejm198702263160907 · Cited by 2,016
A new class of membrane-bound chemokine with a CX3C motif
Bazan, J. F., Bacon, K. B., Hardiman, G., Wang, W., Soo, K., Rossi, D., Greaves, D. R., Zlotnik, A., & Schall, T. J. (1997) · Nature · doi:10.1038/385640a0 · Cited by 2,013
Kovatcheva-Datchary, P., Nilsson, A., Akrami, R., Lee, Y. S., De Vadder, F., Arora, T., Hallen, A., Martens, E., Björck, I., & Bäckhed, F. (2015) · Cell Metabolism · doi:10.1016/j.cmet.2015.10.001 · Cited by 1,731
Research works printed but not linked: 3
- Addison, 1851, On a certain affection of the skin, Vitiligoidea:(a) Plana,(b) Tuberosa, Guy’s Hosp. Rep., 7, 267
- Hohenester, 2012, A biliary HCO3- umbrella constitutes a protective mechanism against bile acid-induced injury in human cholangiocytes, Hepatology, 55, 173, 10.1002/hep.24691 · DOI · Paper page
- Rojas, 2018, Molecular mimicry and autoimmunity, J. Autoimmun., 95, 100, 10.1016/j.jaut.2018.10.012 · DOI · Paper page
Cited by
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You, H., Ma, X., Efe, C., Wang, G., Jeong, S.-H., Abe, K., Duan, W., Chen, S., Kong, Y., Zhang, D., et al. (2022) · Hepatology International · doi:10.1007/s12072-021-10276-6 · Cited by 133
“However, ASMA positivity is less frequent, and the serum IgG levels are seldom above 2 × ULN, especially in the Asia-Pacific region [188, 189].”
BackgroundSelf-citationAutoimmune biliary diseases: primary biliary cholangitis and primary sclerosing cholangitis
Sarcognato, S., Sacchi, D., Grillo, F., Cazzagon, N., Fabris, L., Cadamuro, M., Cataldo, I., Covelli, C., Mangia, A., & Guido, M. (2021) · Pathologica · doi:10.32074/1591-951x-245 · Cited by 128
Helicobacter Pylori and Autoimmune Diseases: Involving Multiple Systems
Wang, L. J., Cao, Z.-M., Zhang, L., Dai, X.-C., Liu, Z.-J., Zeng, Y.-X., Li, X., Wu, Q., & Lv, W. (2022) · Frontiers in Immunology · doi:10.3389/fimmu.2022.833424 · Cited by 110
Pi, B., Wang, J., Tong, Y., Yang, Q., Lv, F., & Yu, Y. (2021) · European Journal of Gastroenterology & Hepatology · doi:10.1097/meg.0000000000002280 · Cited by 105
The Role of Gut Microbiota in Some Liver Diseases: From an Immunological Perspective
Wang, L., Cao, Z.-M., Zhang, L., Li, J.-M., & Lv, W. (2022) · Frontiers in Immunology · doi:10.3389/fimmu.2022.923599 · Cited by 89
“Evidence suggests that intestinal dysbiosis and excessive toxic BAs play key roles in the nosogenesis of PBC (74, 75).”
BackgroundLi, B., Zhang, J., Chen, Y., Wang, Q., Li, Y., Wang, R., Wei, Y., You, Z., Li, Y., Miao, Q., et al. (2021) · Gut Microbes · doi:10.1080/19490976.2021.1946366 · Cited by 74
“Primary biliary cholangitis (PBC) is an autoimmune liver disease characterized by the presence of antimitochondrial antibodies (AMA) and progressive destruction of interlobular bile ducts.(1) Ursodeoxycholic acid (UDCA) is effective in approximately two-thirds of early-stage PBC patients and improves life expectancy w…”
BackgroundInfluentialSelf-citationAutoimmune Hepatitis: Serum Autoantibodies in Clinical Practice
Beretta‐Piccoli, B. T., Mieli‐Vergani, G., & Vergani, D. (2021) · Clinical Reviews in Allergy & Immunology · doi:10.1007/s12016-021-08888-9 · Cited by 64
Self-citationNUDT1 promotes the accumulation and longevity of CD103+ TRM cells in primary biliary cholangitis
Huang, B., Lyu, Z., Qian, Q., Chen, Y., Zhang, J., Li, B., Li, Y., Liang, J., Liu, Q., Li, Y., et al. (2022) · Journal of Hepatology · doi:10.1016/j.jhep.2022.06.014 · Cited by 60
“5,6 Despite the lack of uniform success with targeted therapies based on autoantigen-reactive CTLs in PBC to date, this approach remains promising.”
Self-citationPrimary biliary cholangitis: pathogenic mechanisms
Prìeto, J., Bañales, J. M., & Medina, J. F. (2020) · Current Opinion in Gastroenterology · doi:10.1097/mog.0000000000000703 · Cited by 48
Trussoni, C. E., O’Hara, S. P., & LaRusso, N. F. (2022) · Seminars in Immunopathology · doi:10.1007/s00281-022-00909-9 · Cited by 44
Immunomodulatory Effects of Microbiota-Derived Short-Chain Fatty Acids in Autoimmune Liver Diseases
Zhang, W., Mackay, C. R., & Gershwin, M. E. (2023) · The Journal of Immunology · doi:10.4049/jimmunol.2300016 · Cited by 44
Self-citationCrosstalk between Gut Microbiota and Bile Acids in Cholestatic Liver Disease
Shi, Q., Yuan, X., Zeng, Y., Wang, J., Zhang, Y., Chen, X., & Li, L. (2023) · Nutrients · doi:10.3390/nu15102411 · Cited by 41
“PBC is an autoimmune liver disease characterized by the presence of antimitochondrial antibodies and the progressive destruction of interlobular bile ducts [98].”
BackgroundTerziroli Beretta-Piccoli, B., Stirnimann, G., Mertens, J., Semela, D., Zen, Y., Mazzucchelli, L., Voreck, A., Kolbus, N., Merlo, E., Di Bartolomeo, C., et al. (2021) · Journal of Autoimmunity · doi:10.1016/j.jaut.2020.102578 · Cited by 39
Self-citationZhao, Y., Wei, S., Chen, L., Zhou, X., & Ma, X. (2023) · Frontiers in Immunology · doi:10.3389/fimmu.2023.1164202 · Cited by 38
Self-citationInflammation: Cause or consequence of chronic cholestatic liver injury
Woolbright, B. L. (2020) · Food and Chemical Toxicology · doi:10.1016/j.fct.2020.111133 · Cited by 38
Matsumoto, K., Ohfuji, S., Abe, M., Komori, A., Takahashi, A., Fujii, H., Kawata, K., Noritake, H., Tadokoro, T., Honda, A., et al. (2022) · Journal of Gastroenterology · doi:10.1007/s00535-021-01836-6 · Cited by 35
“As with other autoimmune diseases, PBC is a multifactorial disease and is considered to be caused by the interaction of both genetic background and environmental triggers [4, 5].”
BackgroundSelf-citationInflammasome and pyroptosis in autoimmune liver diseases
Wang, J., Sun, Z., Xie, J., Ji, W., Cui, Y., Ai, Z., & Liang, G. (2023) · Frontiers in Immunology · doi:10.3389/fimmu.2023.1150879 · Cited by 34
Animal Models of Autoimmune Liver Diseases: a Comprehensive Review
Liu, S.-P., Bian, Z.-H., Zhao, Z.-B., Wang, J., Zhang, W., Leung, P. S. C., Li, L., & Lian, Z. (2020) · Clinical Reviews in Allergy & Immunology · doi:10.1007/s12016-020-08778-6 · Cited by 33
Self-citationMicroRNAs in autoimmune liver diseases: from diagnosis to potential therapeutic targets
Huang, C., Xing, X., Xiang, X., Fan, X., Men, R., Ye, T., & Yang, L. (2020) · Biomedicine & Pharmacotherapy · doi:10.1016/j.biopha.2020.110558 · Cited by 32
Rigopoulou, E. I., & Dalekos, G. N. (2021) · Cancers · doi:10.3390/cancers13051023 · Cited by 32
Self-citationHLA, gut microbiome and hepatic autoimmunity
Beretta‐Piccoli, B. T., Mieli‐Vergani, G., & Vergani, D. (2022) · Frontiers in Immunology · doi:10.3389/fimmu.2022.980768 · Cited by 34
“HLA-DRB1*08 India, Iran AIH-1 Adults (15, 20, 21)”
BackgroundSelf-citationSasaki, M., Sato, Y., & Nakanuma, Y. (2019) · Journal of Autoimmunity · doi:10.1016/j.jaut.2019.102377 · Cited by 31
Clinical features, treatment, and outcome of pembrolizumab induced cholangitis
Fang, W., Sun, W., Fang, W., Zhang, J., & Wang, C. (2024) · Naunyn-Schmiedeberg s Archives of Pharmacology · doi:10.1007/s00210-024-03135-2 · Cited by 29
“UCDA has cytoprotective, antiapoptotic and immunomodulatory effects, and can reduce bile toxicity by removing hydrophobic biliary acids and stimulate biliary secretion of bile acids and other organic anions (Terziroli Beretta-Piccoli et al. 2019; Roma et al. 2011).”
Luo, X., & You, X. (2023) · Frontiers in Endocrinology · doi:10.3389/fendo.2023.1225742 · Cited by 26
“In addition, due to the bidirectional relationship of the gut-liver axis, it is unclear whether an altered GM triggers PBC or is a re fl ection of disease status (17).”
BackgroundFloreani, A., Gabbia, D., & Martin, S. D. (2024) · International Journal of Molecular Sciences · doi:10.3390/ijms25042194 · Cited by 27
“Proteins of this molecular pathway are overexpressed in PBC [26].”
BackgroundYang, C., Guo, G., Li, B., Zheng, L., Sun, R., Wang, X., Deng, J., Jia, G., Zhou, X., Cui, L., et al. (2022) · Hepatology International · doi:10.1007/s12072-022-10431-7 · Cited by 27
Self-citationBelimumab treatment in autoimmune hepatitis and primary biliary cholangitis – a case series
Kolev, M., Sarbu, A.-C., Möller, B., Maurer, B., Kollert, F., & Semmo, N. (2023) · Journal of Translational Autoimmunity · doi:10.1016/j.jtauto.2023.100189 · Cited by 27
Changes of Serum IgG Glycosylation Patterns in Primary Biliary Cholangitis Patients
Zeng, X., Li, S., Tang, S., Li, X., Zhang, G., Li, M., Zeng, X., & Hu, C. (2021) · Frontiers in Immunology · doi:10.3389/fimmu.2021.669137 · Cited by 24
“Therefore, new approaches to identifying more specific biomarkers in PBC are needed (20).”
BackgroundImmunologic Responses and the Pathophysiology of Primary Biliary Cholangitis
Chen, R., Tang, R., Ma, X., & Gershwin, M. E. (2022) · Clinics in Liver Disease · doi:10.1016/j.cld.2022.06.003 · Cited by 26
Self-citationJia, H., Chen, J., Zhang, X., Bi, K., Zhou, H., Liu, T., Xu, J. M., & Diao, H. (2022) · Journal of Leukocyte Biology · doi:10.1002/jlb.2a0622-586rrrr · Cited by 23
Alvaro, D., Carpino, G., Craxì, A., Floreani, A., Moschetta, A., & Invernizzi, P. (2020) · Liver International · doi:10.1111/liv.14627 · Cited by 23
Self-citationCai, J., Wu, J., Fang, S., Liu, S., Wang, T., Li, Y., Zou, J., Shi, R., Wang, Z., Yang, L., et al. (2021) · Journal of Ethnopharmacology · doi:10.1016/j.jep.2021.114829 · Cited by 22
Concentration of Serum Matrix Metalloproteinase-3 in Patients With Primary Biliary Cholangitis
Bauer, A., & Habior, A. (2022) · Frontiers in Immunology · doi:10.3389/fimmu.2022.885229 · Cited by 22
Lachiondo‐Ortega, S., Mercado-Gómez, M., Serrano‐Maciá, M., Lopitz‐Otsoa, F., SALAS-VILLALOBOS, T. B., Varela‐Rey, M., Delgado, T. C., & Martínez‐Chantar, M. L. (2019) · Cells · doi:10.3390/cells8121575 · Cited by 23
Xu, Q., Zhu, W., & Yin, Y. (2023) · Medicine · doi:10.1097/md.0000000000036039 · Cited by 20
Hepatobiliary acid-base homeostasis: Insights from analogous secretory epithelia
Trampert, D. C., van de Graaf, S. F. J., Jongejan, A., Elferink, R. P. J. O., & Beuers, U. H. (2020) · Journal of Hepatology · doi:10.1016/j.jhep.2020.10.010 · Cited by 21
Self-citationCadamuro, M., Girardi, N., Gores, G. J., Strazzabosco, M., & Fabris, L. (2020) · Frontiers in Medicine · doi:10.3389/fmed.2020.00115 · Cited by 19
Self-citationLi, Y., Li, Z., Chen, R., Lian, M., Wang, H., Wei, Y., You, Z., Zhang, J., Li, B., Li, Y., et al. (2023) · Nature Communications · doi:10.1038/s41467-023-37213-5 · Cited by 18
Self-citationUmemura, M., Honda, A., Yamashita, M., Chida, T., Noritake, H., Yamamoto, K., Honda, T., Ichimura‐Shimizu, M., Tsuneyama, K., Miyazaki, T., et al. (2024) · Journal of Autoimmunity · doi:10.1016/j.jaut.2024.103287 · Cited by 17
Self-citationYu, K., Li, P., Xu, T., Xu, J., Wang, K., Chai, J., Zhao, D., Liu, Y., Wang, Y., Ma, J., et al. (2020) · Pathology - Research and Practice · doi:10.1016/j.prp.2020.153291 · Cited by 17
Dual B-cell targeting therapy ameliorates autoimmune cholangitis
Zhang, W., Shao, T., Leung, P. S. C., Tsuneyama, K., Heuer, L. S., Young, H. A., Ridgway, W. M., & Gershwin, M. E. (2022) · Journal of Autoimmunity · doi:10.1016/j.jaut.2022.102897 · Cited by 17
Self-citationBauer, A., Habior, A., Wieszczy, P., & Gaweł, D. (2021) · Diagnostics · doi:10.3390/diagnostics11040587 · Cited by 15
“We agree with Terziroli Beretta-Piccoli and coworkers [1] that it is necessary to study the mechanisms causing the production of anti-nuclear antibodies and identify the role of T cell, which distinguish the antigenic target of PBC specific antinuclear antibodies.”
ResultBauer, A., Habior, A., & Gaweł, D. (2022) · Biomedicines · doi:10.3390/biomedicines10040801 · Cited by 15
Animal models of primary biliary cholangitis: status and challenges
Wang, X., Wei, Y., Yang, Y., Yang, Y., Li, H., Li, Y., Zhang, F., & Wang, L. (2023) · Cell & Bioscience · doi:10.1186/s13578-023-01170-9 · Cited by 15
“However, whether BEC damage is the cause or result of autoimmune dysfunction remains unclear [14].”
BackgroundWang, C., Wang, Y., Yao, Y., Wang, J., Tsuneyama, K., Yang, Q., Liu, B., Selmi, C. F., Gershwin, M. E., Yang, S., et al. (2022) · Annals of Translational Medicine · doi:10.21037/atm-21-5448 · Cited by 15
Self-citationWang, R., Lin, Q., Lu, Z., Wen, H., Hu, F., You, J., He, Y., Fang, Y., Bian, Z., Hou, Q., et al. (2024) · Journal of Autoimmunity · doi:10.1016/j.jaut.2023.103163 · Cited by 14
Self-citationReasons why women are more likely to develop primary biliary cholangitis
Ma, D., Ma, J., Zhao, C., & Tai, W. (2024) · Heliyon · doi:10.1016/j.heliyon.2024.e25634 · Cited by 18
Xiao, Q., Zhang, S., Ren, H., Du, R., Li, J., Zhao, J., Gao, Y., Zhu, Y., & Huang, W. (2020) · Chemico-Biological Interactions · doi:10.1016/j.cbi.2020.109062 · Cited by 14
Xu, J., Fu, H., Yang, Y., Yu, H., Ai, X., Lei, Y.-J., Bao, W., & Tang, Y. (2021) · Molecular Immunology · doi:10.1016/j.molimm.2021.04.014 · Cited by 14
Cheng, C., Wang, Z., Wang, L., Zhao, J., Wang, Q., Tian, X., Li, M., & Zeng, X. (2020) · Clinical Rheumatology · doi:10.1007/s10067-020-05457-x · Cited by 14
Xiang, X., Xiao-li, Y., Shen, M., Huang, C., Liu, Y., Fan, X., & Yang, L. (2021) · Canadian Journal of Gastroenterology and Hepatology · doi:10.1155/2021/6691425 · Cited by 12
“*e proportion of UDCA in the bile composition of PBC patients was substantially increased, replacing the toxic BAs and acting as a cytoprotective agent [8, 27].”
van Beers, J. J. B. C., Koek, G. H., & Damoiseaux, J. G. M. C. (2021) · The Journal of Applied Laboratory Medicine · doi:10.1093/jalm/jfab099 · Cited by 12
Bin, L., Liu, Z., & Xu, L. (2020) · Revista Española de Enfermedades Digestivas · doi:10.17235/reed.2020.7483/2020 · Cited by 12
Unmet needs in autoimmune liver diseases
Kolev, M., Kremer, A. E., Sokollik, C., Gershwin, M. E., Beretta‐Piccoli, B. T., Andrade, R. J., Bobzin, C., de Boer, Y. S., de Martin, E., Deganello, A., et al. (2025) · Current Opinion in Immunology · doi:10.1016/j.coi.2025.102565 · Cited by 11
Self-citationLiu, H., Yang, A.-Y., Xiong, Q., Zhong, Y.-D., Liu, D.-X., Huang, P. C., Feng, X., Zhang, Y., & Yang, Y. (2022) · BMC Gastroenterology · doi:10.1186/s12876-022-02538-w · Cited by 10
Kolev, M., Horn, M. P., Semmo, N., & Nagler, M. (2022) · Journal of Translational Autoimmunity · doi:10.1016/j.jtauto.2022.100151 · Cited by 9
Beretta‐Piccoli, B. T., Mieli‐Vergani, G., & Vergani, D. (2024) · Elsevier eBooks · doi:10.1016/b978-0-443-23947-2.00074-6 · Cited by 7
Self-citationGui, H., Wang, W., Li, Q., Li, Z., Lu, J. L., & Xie, Q. (2021) · Immunologic Research · doi:10.1007/s12026-021-09178-4 · Cited by 8
“Of note, a high proportion of AMA and/or PBC-specific ANA-positive patients with normal liver enzymes have been shown to have liver pathology consistent with PBC and to ultimately progress over time to develop overt clinical and biochemical features of PBC [3, 29, 30].”
BackgroundMethodPapamichalis, P. A., Zachou, K., Papamichali, R., Ιωάννου, Μ., Gatselis, N., Dalekos, G. N., & Koukoulis, G. Κ. (2021) · Journal of Translational Internal Medicine · doi:10.2478/jtim-2021-0008 · Cited by 8
“Identifying the molecular basis of PML overexpression in PBC, one could suggest the use of ATRA or As2O3 in an attempt to reverse PML overexpression in bile ducts, as a new specific therapeutic target for PBC patients not responding to ursodeoxycholic acid.[1,2]”
BackgroundSelf-citationLudz, C., Stirnimann, G., Semela, D., Mertens, J. C., Kremer, A. E., Sinnreich, M. F., Sokollik, C., Bernsmeier, C., Bresson‐Hadni, S., McLin, V. A., et al. (2023) · Swiss Medical Weekly · doi:10.57187/smw.2023.40102 · Cited by 10
Self-citationAdvances in clinical detection methods and nanomaterials for autoimmune liver diseases
Chen, J., Zhu, Y., Wang, Q., Ding, J., & Chen, X. (2023) · Nano TransMed · doi:10.1016/j.ntm.2023.100003 · Cited by 8
An Integrated Pathogenetic Model of Primary Biliary Cholangitis
Kouroumalis, E., Tsomidis, I., & Voumvouraki, A. (2025) · Livers · doi:10.3390/livers5020015 · Cited by 7
“Intestinal dysbiosis and increased toxic BA levels play a significant role in the pathogenesis of PBC [197,198].”
Himoto, T., Yamamoto, S., Morimoto, K., Tada, S., Mimura, S., Fujita, K., Tani, J., Morishita, A., & Masaki, T. (2021) · Journal of Clinical Laboratory Analysis · doi:10.1002/jcla.24040 · Cited by 7
Ma, D., Liu, X., Li, J., Wu, H., Ma, J., & Tai, W. (2024) · Digestive and Liver Disease · doi:10.1016/j.dld.2024.05.012 · Cited by 7
Zhang, C., Sun, G., Jin, H., Wei, Y., Zheng, S., Wang, X., Zhao, X., Zhang, D., & Jia, J. (2025) · BMC Medicine · doi:10.1186/s12916-025-04043-9 · Cited by 8
CTLA-4 Expression Plays a Role in PSC and PBC Progression
Meister, P., Steinke-Ramming, C., Beste, M., Lenzen, H., Gerken, G., Canbay, A. E., & Jochum, C. (2020) · Diseases · doi:10.3390/diseases8020021 · Cited by 6
“The autoimmune basis of PBC is supported by the presence of AMA and the characteristic progressive T‐cell predominant lymphocytic cholangitis [1] Reinforcing this approach, there are several autoimmune diseases such as myasthenia gravis [2] [3] [4], Primary Sjögren’s Syndrome [5] and coeliac disease [6], which are lin…”
BackgroundLongitudinal Assessment of Bile Duct Loss in Primary Biliary Cholangitis
Hercun, J., Noureddin, M., Noureddin, N., Eccleston, J. L., Woolridge, D., Liang, T. J., Tana, M. M.-S., Kleiner, D. E., Rodriguez, G. V., Koh, C., et al. (2022) · The American Journal of Gastroenterology · doi:10.14309/ajg.0000000000001985 · Cited by 6
“These clinically relevant findings can only be confirmed through liver biopsy, which provides an overall histological assessment of PBC, as highlighted in expert opinion (15).”
BackgroundSerum Biomarkers for Autoimmune Hepatitis Type 1: the Case for CD48 and a Review of the Literature
Hu, M., You, Z., Li, Y., Huang, B., Cui, N., Wang, R., Wei, Y., Li, B., Liang, J., Liu, Q., et al. (2022) · Clinical Reviews in Allergy & Immunology · doi:10.1007/s12016-022-08935-z · Cited by 5
“Otherwise, 50 patients with primary biliary cholangitis (PBC) and 29 patients with non-alcoholic fatty liver disease (NAFLD) were recruited as disease controls, both with standard diagnosis meeting corresponding criteria [21, 22].”
MethodSelf-citationCurrent and Emerging Molecular Markers of Liver Diseases: A Pathogenic Perspective
Liang, Y., Guo, G. L., & Zhang, L. (2022) · Gene Expression · doi:10.14218/gejlr.2022.00010 · Cited by 5
“88 The primary target antigens in RLM-pattern-associated antibodies are nuclear envelope proteins p62 and gp210.”
BackgroundXia, Y., Yan, S., Chang, Y., Yin, J., Li, H., & Yan, S. (2022) · Evidence-based Complementary and Alternative Medicine · doi:10.1155/2022/1589388 · Cited by 5
Rogalska, M., Błachnio-Zabielska, A. U., Zabielski, P., Janica, J. R., Roszczyc-Owsiejczuk, K., Pogodzińska, K., Andrzejuk, A., Dâbrowski, A., Flisiak, R., & Rogalski, P. (2025) · Nutrients · doi:10.3390/nu17071097 · Cited by 5
The evolving potential of precision medicine in the management of autoimmune liver disease
Norman, G. L., Bizzaro, N., Villalta, D., Vergani, D., Mieli‐Vergani, G., Hirschfield, G. M., & Mähler, M. (2021) · Elsevier eBooks · doi:10.1016/b978-0-12-820239-5.00012-7 · Cited by 5
Self-citationBae, U., Park, B., Cho, M. K., & Bae, E. J. (2022) · Journal of Medicinal Food · doi:10.1089/jmf.2022.k.0015 · Cited by 4
RetractedBeretta‐Piccoli, B. T., Mazzucchelli, L., Taiana, C., Mossi, G., Usai, C., Vergani, D., & Mieli‐Vergani, G. (2019) · Journal of Translational Autoimmunity · doi:10.1016/j.jtauto.2019.100033 · Cited by 4
Self-citationSerum Ghrelin Concentration in Patients With Primary Biliary Cirrhosis (PBC)
Naguib, R., Fayed, A. A., Elkemary, E., & Naguib, H. (2021) · Cureus · doi:10.7759/cureus.20288 · Cited by 4
“Primary biliary cholangitis, formerly known as primary biliary cirrhosis, is an autoimmune progressive liver disease that primarily affects women (with an incidence of 20 to 40 cases per 100,000) [7,18-20].”
BackgroundLian, Z., & Li, L. (2020) · Springer eBooks · doi:10.1007/978-3-030-51709-0_2 · Cited by 4
Self-citationChatzipantelis, P., & Giatromanolaki, A. N. (2021) · PubMed · doi:10.1097/meg.0000000000001876 · Cited by 3
Qin, Z., Cheng, F., Zhang, M., Qian, R., Chen, H., Zhao, Y., Zhang, Y., Dai, Y., Tang, C., Jiang, P., et al. (2024) · Journal of Translational Autoimmunity · doi:10.1016/j.jtauto.2024.100262 · Cited by 3
“The detection of antimitochondrial autoantibody (AMA) is the hallmark of primary biliary cholangitis (PBC) [1].”
Self-citationCheng, T., Li, H., Luo, X., Ju, L., Chen, L., Shao, J., She, Y.-J., Li, M., & Bian, Z. (2021) · Current Medical Science · doi:10.1007/s11596-021-2458-3 · Cited by 3
Role of gut microbiota in primary biliary cholangitis
Jin, W., Song, S., Xu, X., Wen, C., & Hu, C. (2022) · Hepatobiliary & pancreatic diseases international · doi:10.1016/j.hbpd.2022.06.014 · Cited by 3
Wang, Z., Yu, M., & Wang, H. (2025) · Scientific Reports · doi:10.1038/s41598-025-19697-x · Cited by 2
“Furthermore, chronic liver inflammation, as a trademark of PBC, can contribute to progressive muscle atrophy and accelerate the development of sarcopenia 24 .”
Jawed, R., Zhang, M., Wang, C., Yang, S., Jiang, P., Wu, Q., Li, L., Chen, W., Gershwin, M. E., Tian, Y., et al. (2020) · Immunogenetics · doi:10.1007/s00251-020-01192-4 · Cited by 2
Self-citationIncreased sensitivity of gp210 autoantibody detection using a newly designed gp210 antigen
Xu, J., Zhang, H., Wang, C., Jiang, P., Han, C., Dai, Y., Qiu, F., Gong, Y., Jiang, Y., Xu, P., et al. (2021) · Journal of Immunological Methods · doi:10.1016/j.jim.2021.113211 · Cited by 2
Self-citationZarachi, A., Pelechas, E., Tsikou, A., Kalambokis, G. N., Zioga, A., Panteli, K. E., Rapti, I., Filippas-Ntekouan, S., Kastanioudakis, I. G., & Karassa, F. B. (2022) · Mediterranean Journal of Rheumatology · doi:10.31138/mjr.33.3.349 · Cited by 2
Hao, J., Shen, X., Shao, X., Zhu, C., Lu, K., Xu, Y., Chen, Y., Liu, J., Ding, Y., Xie, X., et al. (2023) · Arabian Journal of Chemistry · doi:10.1016/j.arabjc.2023.104968 · Cited by 2
Jiang, P., Wang, C., Zhang, M., Tian, Y., Zhao, W., Xin, J., Huang, Y., Zhao, Z., Sun, W., Long, J., et al. (2023) · Journal of genetics and genomics/Journal of Genetics and Genomics · doi:10.1016/j.jgg.2023.06.004 · Cited by 2
Self-citationLiu, Y., Chen, L., Hao, W., Zhao, K., & Li, C. (2025) · International Journal of Immunopathology and Pharmacology · doi:10.1177/03946320251327621 · Cited by 3
Yang, M., Hu, C., Huang, J., Fu, Y., Zhang, Q., Cheng, Y., Lu, J., Li, G., & Zhang, J. (2024) · Journal of Laboratory Medicine · doi:10.1515/labmed-2023-0127 · Cited by 1
Rogalska, M., Błachnio-Zabielska, A. U., Zabielski, P., Janica, J. R., Roszczyc-Owsiejczuk, K., Pogodzińska, K., Andrzejuk, A., Dâbrowski, A., Flisiak, R., & Rogalski, P. (2025) · Scientific Reports · doi:10.1038/s41598-025-26502-2 · Cited by 1
Yu, Y., Zhang, L., Zhou, J., Wang, J., & Huang, Q. (2025) · Toxicology Research · doi:10.1093/toxres/tfaf151 · Cited by 1
Rigopoulou, E. I., Koutsoumpas, A. L., & Bogdanos, D. P. (2022) · Springer eBooks · doi:10.1007/978-981-19-1946-6_6 · Cited by 1
Zhou, T., Wei, H., & Wang, J. (2023) · PLoS ONE · doi:10.1371/journal.pone.0279431 · Cited by 1
A case report of primary biliary cholangitis combined with ankylosing spondylitis
Hou, C., Ren, C., Luan, L., & Li, S. (2023) · Medicine · doi:10.1097/md.0000000000035655 · Cited by 2
“[7,8] 11] A recent comprehensive investigation found that 28.3% of PBC patients had extrahepatic autoimmune diseases, of which only 0.2% had concurrent ankylosing spondylitis (AS).”
BackgroundInfluentialTrivella, J. P., & Levy, C. (2024) · Elsevier eBooks · doi:10.1016/b978-0-443-26710-9.00018-3 · Cited by 1
Ursodeoxycholic Acid in Liver Cirrhosis: A Chinese Perspective
Gao, W., Li, Z., Chu, H., Yuan, H., Hu, L., Yao, L., Zhang, L. R., Wang, W., Lin, R., & Yang, L. (2022) · Springer eBooks · doi:10.1007/978-981-19-2615-0_6 · Cited by 1
Nutrition in Cholestatic Liver Disease
Goyes, D., Danford, C., Trivedi, H. D., & Bonder, A. (2020) · Current Hepatology Reports · doi:10.1007/s11901-020-00511-3
The Microbiota-Gut-Liver Axis: Implications for the Pathophysiology of Liver Disease
Quigley, E. M. M. (2020) · Springer eBooks · doi:10.1007/978-3-030-51709-0_8 · Cited by 1
Self-citationLe malattie autoimmuni di fegato nell’adulto
Beretta‐Piccoli, B. T., Gottardi, A. D., Vergani, D., & Mieli‐Vergani, G. (2021) · Schweizer Gastroenterologie · doi:10.1007/s43472-021-00040-4
Self-citationDing, D., Liu, Y., Zheng, L., Jia, G., Deng, J., Sun, R., Wang, X., Zhou, X., Zhang, M., Ren, P., et al. (2022) · Research Square · doi:10.21203/rs.3.rs-1521417/v1
Relationship between Gut Microbiota and Development and Prognosis of Autoimmune Liver Disease
李, 玉. (2022) · Advances in Clinical Medicine · doi:10.12677/acm.2022.12111557
Metadata
- Authors
- Terziroli Beretta-Piccoli, B., Mieli-Vergani, G., Vergani, D., Vierling, J. M., Adams, D., Alpini, G., Banales, J. M., Beuers, U., Björnsson, E., Bowlus, C., Carbone, M., Chazouillères, O., Dalekos, G., De Gottardi, A., Harada, K., Hirschfield, G., Invernizzi, P., Jones, D., Krawitt, E., Lanzavecchia, A., Lian, Z.-X., Ma, X., Manns, M., Mavilio, D., Quigley, E. M., Sallusto, F., Shimoda, S., Strazzabosco, M., Swain, M., Tanaka, A., Trauner, M., Tsuneyama, K., Zigmond, E., & Gershwin, M. E.
- Affiliations
- King's College Hospital (GB)
- Baylor College of Medicine (US)
- NIHR Birmingham Biomedical Research Centre (GB)
- Richard L. Roudebush VA Medical Center (US)
- Amsterdam UMC Location University of Amsterdam (NL)
- University of California, Davis (US)
- University of Milan (IT)
- Hôpital Saint-Antoine (FR)
- University of Thessaly (GR)
- Kanazawa University (JP)
- University Health Network (CA)
- NIHR Newcastle Biomedical Research Centre (GB)
- University of Vermont (US)
- Institute for Research in Biomedicine (CH)
- South China University of Technology (CN)
- Shanghai Jiao Tong University (CN)
- Medizinische Hochschule Hannover (DE)
- Houston Methodist (US)
- Kyushu University (JP)
- Yale University (US)
- University of Calgary (CA)
- Teikyo University (JP)
- Medical University of Vienna (AT)
- Tokushima University (JP)
- Tel Aviv University (IL)
- ORCID
- Vergani, Diego
- Vierling, John M.
- Adams, David
- Alpini, Gianfranco
- Beuers, Ulrich
- Bowlus, Christopher
- Carbone, Marco
- Chazouillères, Olivier
- Dalekos, George
- Harada, Kenichi
- Hirschfield, Gideon
- Invernizzi, Pietro
- Jones, David
- Lanzavecchia, Antonio
- Lian, Zhe-Xiong
- Ma, Xiong
- Manns, Michael
- Mavilio, Domenico
- Sallusto, Federica
- Shimoda, Shinji
- Strazzabosco, Mario
- Swain, Mark
- Tanaka, Atsushi
- Trauner, Michael
- Tsuneyama, Koichi
- Zigmond, Ehud
- Gershwin, M. Eric
- Venue
- Journal of Autoimmunity
- Publisher
- Elsevier BV
- Published
- 2019-12
- Volume
- 105
- Pages
- 102328
- ISSN
- 0896-8411 (ISSN-L)
- OpenAlex
- W2974241762
- Cited by
- 114
- References
- 318
- Citations by year
- 2026: 2 · 2025: 13 · 2024: 13 · 2023: 18 · 2022: 26 · 2021: 21 · 2020: 18 · 2019: 3
- FWCI
- 2.23
- Type
- journal article
- Language
- en
- Open access
- Licence
- Locations
- Journal of Autoimmunity (publishedVersion)
- PubMed (publishedVersion)
- Data Archiving and Networked Services (DANS) (submittedVersion)
- BOA (University of Milano-Bicocca) (submittedVersion)
- University of Thessaly Institutional Repository (University of Thessaly) (submittedVersion)
- Institutional Repositories DataBase (IRDB) (submittedVersion)
- Pure Amsterdam UMC (submittedVersion)
- University of Birmingham Research Portal (University of Birmingham) (submittedVersion, cc-by-nc-nd)
- Hirsla (Landspítali University Hospital) (submittedVersion)
- Funders
- National Institute for Health and Care Research (NIHR200243, NF-SI-0513-10091)
- Medical Research Council (MR/L001489/1)
- Japan Society for the Promotion of Science (18K07069, 17H04058)
- Topics
- Health Sciences › Medicine › Hepatology › Liver Diseases and Immunity
- Health Sciences › Medicine › Surgery › Pediatric Hepatobiliary Diseases and Treatments
- Health Sciences › Medicine › Oncology › Drug Transport and Resistance Mechanisms
- Keywords
- primary biliary cirrhosis, antimitochondrial antibodies, antinuclear antibodies, ursodeoxycholic acid, obeticholic acid, liver histopathology, bile acids, biliary epithelium, autoimmune liver disease, disease progression, difficult-to-treat patients, etiopathogenesis
- MeSH terms
- Antibodies, Antinuclear, Autoimmune Diseases, Chenodeoxycholic Acid, Cholagogues and Choleretics, Congresses as Topic, Female, Humans, Liver, Liver Cirrhosis, Biliary, Ursodeoxycholic Acid
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