High levels of soluble CTLA-4 are present in anti-mitochondrial antibody positive, but not in antibody negative patients with primary biliary cirrhosis. by Daniele Saverino, Giampaola Pesce, Princey Antola, Brunetta Porcelli, Ignazio Brusca, Danilo Villalta, Marilina Tampoia, Renato Tozzoli, Elio Tonutti, Maria Grazia Alessio, Marcello Bagnasco, Nicola Bizzaro

“…Primary biliary cirrhosis (PBC) is a chronic autoimmune cholestatic liver disease frequently characterized by anti-mitochondrial autoantibodies (AMA). …”
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Noninvasive diagnostic methods of liver fibrosis in patients with primary biliary cirrhosis and primary sclerosing cholangitis: role of indirect serological markers by A. F. Sheptulina, Ye. N. Shirokova, V. T. Ivashkin

“…Considering prognostic value and dynamic nature of liver fibrosis in chronic cholestatic diseases: primary biliary cirrhosis (PBC) and primary sclerosing cholangitis (PSC), severity assessment by liver biopsy and/or noninvasive methods should be obligatory component of the investigation plan during patient follow-up to estimate treatment response. …”
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Noninvasive diagnosis of AIH/PBC overlap syndrome based on prediction models by Wang Kailing, Li Yong, Pan Jianfeng, He Huifang, Zhao Ziyi, Guo Yiming, Zhang Xiaomei

Subjects: “…primary biliary cirrhosis…”
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CLINICAL DIAGNOSTIC VALUE OF AUTOANTIBODIES IN THE DIAGNOSIS OF AUTOIMMUNE LIVER DISEASES by V. V. Bazarnyi, E. Yu. Mikhailova, E. A. Partylova

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Ursodeoxycholic Acid Influences the Expression of p27kip1 but Not FoxO1 in Patients with Non-Cirrhotic Primary Biliary Cirrhosis by Malgorzata Milkiewicz, Justyna Kopycińska, Agnieszka Kempińska-Podhorodecka, Tara Haas, Dimitrios P. Bogdanos, Elwyn Elias, Piotr Milkiewicz

“…Ursodeoxycholic acid (UDCA) delays progression of primary biliary cirrhosis (PBC) but its effect on p27kip1 expression is uncertain. …”
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Corrigendum: Genome-wide analysis of DNA methylation, copy number variation, and gene expression in monozygotic twins discordant for primary biliary cirrhosis by Carlo eSelmi, Carlo eSelmi

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Decreased interleukin-10 mRNA expression in primary sclerosing cholangitis and primary biliary cirrhosis: A role for IL10 in the pathogenesis? by Mitchell, SA, Fleming, K, Chapman, R

A Review of the Physiological and Immunological Functions of Biliary Epithelial Cells: Targets for Primary Biliary Cirrhosis, Primary Sclerosing Cholangitis and Drug-induced Ductopenias by Chih-Te Wu, Paul A. Davis, VelImir A. Luketic, M. Eric Gershwin

“…These then suggest a myriad of potential roles for BEC in defense from invading microorganisms as well as the pathogenesis of diverse immunologically driven diseases such as primary biliary cirrhosis (PBC), graft-versus-host disease, and primary sclerosing cholangitis (PSC). …”
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Characterization of serum and urinary bile acids in patients with primary biliary cirrhosis by gas-liquid chromatography-mass spectrometry: effect of ursodeoxycholic acid treatment. by A K Batta, R Arora, G Salen, G S Tint, D Eskreis, S Katz

“…These results demonstrate significant changes in the serum and urinary bile acid pattern in primary biliary cirrhosis during ursodeoxycholic acid treatment. …”
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A Case of Rheumatoid Arthritis and Limited Systemic Sclerosis Overlap Successfully Treated with Tocilizumab for Arthritis and Concomitant Generalized Lymphadenopathy and Primary Biliary Cirrhosis by Eiko Saito, Shinji Sato, Shinichi Nogi, Noriko Sasaki, Naofumi Chinen, Kiri Honda, Takayuki Wakabayashi, Chiho Yamada, Naoya Nakamura, Yasuo Suzuki

“…A 57-year-old woman with rheumatoid arthritis (RA) and limited systemic sclerosis (lSSc) was suspected to have lymphadenopathy and primary biliary cirrhosis (PBC). Lymph node biopsy showed reactive follicular lymphadenopathy with intrafollicular plasmacyte infiltration that was interleukin-6 positive by immunohistostaining. …”
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Frequent infiltration of S-100 protein+ CCR5+ immature dendritic cells in damaged bile ducts of primary biliary cirrhosis compared to cholangiocellular carcinoma by Mitsui H, Ohtake H, Ohe R, Yamakawa M

“…Keywords: chemokines, cholangiocellular carcinoma, chronic nonsuppurative destructive cholangitis, dendritic cell, primary biliary cirrhosis…”
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Antibodies to SS-A/Ro-52kD and centromere in autoimmune liver disease: a clue to diagnosis and prognosis of primary biliary cirrhosis. by Granito, A, Muratori, P, Muratori, L, Pappas, G, Cassani, F, Worthington, J, Ferri, S, Quarneti, C, Cipriano, V, de Molo, C, Lenzi, M, Chapman, R, Bianchi, F

“…BACKGROUND: Primary biliary cirrhosis (PBC) may be associated with various rheumatological disorders. …”

Characterization of a Lipoyl Domain-Independent B-Cell Autoepitope on the Human Branched-Chain Acyltransferase in Primary Biliary Cirrhosis and Overlap Syndrome with Autoimmune Hepatitis by Antal Csepregi, Petra Obermayer-Straub, Susanne Kneip, Anne Kayser, Stephanie Loges, Eleonore Schmidt, Elemér Nemesánszky, Ferenc Szalay, Michael P. Manns, Christian P. Strassburg

“…Background and aims: Antimitochondrial antibodies (AMA) which recognize pyruvate acetyltransferase (PDC-E2) represent a highly diagnostic feature of primary biliary cirrhosis (PBC). The analysis of immunofluorescence (IF)-AMA-positive sera in PBC patients indicates a conformational epitope located within the lipoyl binding domain of bovine branched-chain acyltransferase (BCKADC-E2) alone or in combination with AMA directed against PDC-E2 the significance of which is presently unclear. …”
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Autoimmune hepatitis type 2 associated with an unexpected and transient presence of primary biliary cirrhosis-specific antimitochondrial antibodies: a case study and review of the literature by Invernizzi Pietro, Alessio Maria, Smyk Daniel S, Lleo Ana, Sonzogni Aurelio, Fabris Luca, Candusso Manila, Bogdanos Dimitrios P, Iorio Raffaele, Torre Giuliano

“…<p>Abstract</p> <p>Background</p> <p>Unlike other autoimmune liver diseases, primary biliary cirrhosis (PBC) has never been reported in early childhood, while type 2 autoimmune hepatitis (AIH) is eminently a paediatric disease.…”
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Detection of MND and RL/M anti-nuclear antibodies patterns: A reliable diagnostic tool in anti-mitochondrial antibodies negative primary biliary cirrhosis by Granito, A, Pappas, G, Muratori, P, Muratori, L, Ferri, S, Quarneti, C, De Molo, C, Cipriano, V, Cassani, F, Worthington, J, Chapman, R, Bianchi, F

Diagnostic accuracy of anti-nuclear antibodies giving a multiple nuclear dots (MND) or a membranous (RL/M) pattern in primary biliary cirrhosis by Granito, A, Guidi, M, Muratori, P, Worthington, J, Pappas, G, Muratori, L, De Molo, C, Cassani, F, Ferri, S, Lenzi, M, Chapman, R, Bianchi, F

BAT117213: Ileal bile acid transporter (IBAT) inhibition as a treatment for pruritus in primary biliary cirrhosis: study protocol for a randomised controlled trial by Hegade, VS, Kendrick, SFW, Dobbins, RL, Miller, SR, Richards, D, Storey, J, Dukes, G, Gilchrist, K, Vallow, S, Alexander, GJ, Corrigan, M, Hirschfield, GM, Jones, DEJ

“…<p><strong>Background</strong> Pruritus (itch) is a symptom commonly experienced by patients with cholestatic liver diseases such as primary biliary cholangitis (PBC, previously referred to as primary biliary cirrhosis). Bile acids (BAs) have been proposed as potential pruritogens in PBC. …”

Microbial Transglutaminase—The Food Additive, a Potential Inducing Factor in Primary Biliary Cholangitis by Alicja Bauer, Paulina Rosiek, Tomasz Bauer

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Low incidence of positive smooth muscle antibody and high incidence of isolated IgM elevation in Chinese patients with autoimmune hepatitis and primary biliary cirrhosis overlap syndrome: a retrospective study by Zhao Pan, Han Yukun

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Nuclear receptors in regulation of bile acids transport and metabolism by A. F. Sheptulina, Ye. N. Shirokova, V. T. Ivashkin

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