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Background. Primary biliary cholangitis (PBC) is a chronic autoimmune liver disease. As an infrequent disease, a Facebook group was created for patients to share experiences and problems. In fact, during the COVID-19 pandemic, patient analysis could only be done through remote connection systems. Therefore, to analyze patients' quality of life (QoL), we exploited social networks and online data collection platforms. Objectives. A survey was carried out to evaluate the QoL of patients with PBC during the COVID-19 pandemic. Materials and Methods. A Facebook group was used for patient enrolment. Age, sex, diagnosis, years since diagnosis, associated diseases, histological stage of the disease, value of elastography, and current therapy were collected. PBC 40 online questionnaire was submitted to patients to assess their QoL. Results. 78 patients participated in the study: 75 females, and 3 males, the mean (±SD) age was 46.4±11.5. The main diagnoses were PBC in 66 patients and overlapping syndrome PBC + autoimmune hepatitis in 10. Histology was available in 45 patients, of whom 34 were stages 1-2 and 11 stages 3-4. The main therapy was ursodeoxycholic acid in 56 pts. The questionnaire is divided into 6 domains, covering fatigue, emotional, social, and cognitive functions, general symptoms, and itching. The mean and standard deviation of the scores were computed. Interpretation of the results obtained by applying a quantitative scale showed no impairment for social, mild impairment for general symptoms, itching, cognitive and emotional function, and moderate impairment for fatigue. No correlation was found between scores and disease duration. Conclusions. This study demonstrates that online questionnaires are a viable substitute for paper questionnaires and that data collected from online surveys on Facebook can have scientific relevance; PBC had the greatest impact on QoL on fatigue and the least on social aspects. [ABSTRACT FROM AUTHOR]

The bibliometric analysis uses the citation count of an article to measure its impact in the scientific community, but no study has been undertaken to determine the most influential papers in the field of primary biliary cholangitis (PBC). This study aimed to investigate the global research interest regarding PBC in dentistry using a bibliometric approach. We searched the Web of Science Core Collection database to find the top 100 most cited (T100) articles focusing on PBC. The information about each article including citations, authors, journals, countries, institutions, and keywords was recorded for bibliometric analysis. The T100 articles related to PBC were published from 1983 to 2019 and were originated from 26 countries. A total of 805 different authors were from 342 different institutions, and articles written by them were published in 35 journals. The five most frequently occurring keywords were "biochemical response," "ursodeoxycholic acid," "primary biliary cirrhosis," "antimitochondrial antibody," and "autoimmunity." The T100 articles were classified into different research focuses: pathogenesis (41%), treatment (20%), prognosis (12%), epidemiology (9%), diagnosis (8%), and others (10%). These 100 articles included 32 observational studies, 29 basic research articles, 15 reviews, eight meta-analyses, 12 clinical trials, and four clinical guidelines. The 100 top-cited articles are marked with the leading countries, institutions, journals, hotspots, and development trends in the PBC field that could provide the foundation for further investigations. [ABSTRACT FROM AUTHOR]

Copyright of Journal of Clinical Hepatology / Linchuang Gandanbing Zazhi is the property of Journal of Clinical Hepatology and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Copyright of Turkiye Klinikleri Journal of Internal Medicine / Türkiye Klinikleri İç Hastalıkları Dergisi is the property of Turkiye Klinikleri and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Background: The Albumin-Bilirubin (ALBI) score and grade are widely used to stratify patients with primary biliary cholangitis (PBC) into different disease statuses and risk levels. Recent studies have increasingly highlighted the role of gut microbiota in autoimmune liver diseases. This study aimed to investigate the differences in gut microbiota among PBC patients with varying ALBI grades. Methods: Clinical data and stool samples were collected from outpatient and inpatient PBC patients between 2019 and 2022. Gut microbiota profiles were obtained using 16S rDNA sequencing of stool samples. We analyzed alpha diversity, beta diversity, LEfSe analysis and pathway function prediction. Additionally, various machine learning methods—including random forest (RF), lasso, gradient boosting machine (GBM) and support vector machine (SVM)—were employed to identify key features and to build and validate predictive models using bootstrap techniques. Results: Clinical characteristics of ALBI grade 1 patients were comparatively better than those of ALBI grade 2 and 3 patients, including multiple laboratory indices. Gut microbiota analysis revealed that species richness and balance were higher in ALBI grade 1 patients. Both the comparison of the most abundant genera and the linear discriminant analysis (LDA) in LEfSe demonstrated that Lachnospira had a higher abundance and better discriminative ability in ALBI grade 1. Pathway function prediction indicated that sulfur metabolism was upregulated in higher ALBI grades. Furthermore, RF identified 10 specific genera, which were then used to build and validate models for discriminating PBC patients according to their ALBI grades. All three models, developed using different machine learning methods, demonstrated good discrimination ability (mean AUC 0.75–0.80). Conclusion: This study highlights significant differences in gut microbiota profiles among PBC patients with different ALBI grades. The increased abundance of Lachnospira and upregulation of sulfur metabolism pathways are notable in patients with lower ALBI grades. The machine learning models developed based on gut microbiota features offer promising tools for discriminating between PBC patients with varying disease severities, which could enhance the precision of treatment strategies. [ABSTRACT FROM AUTHOR]

Copyright of Practical Pharmacy & Clinical Remedies is the property of Editorial Department of Practical Pharmacy & Clinical Remedies and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Corticosteroids and immunosuppressive drugs can alleviate the symptoms of most autoimmune diseases and induce remission by restraining the autoimmune attack and limiting the damage to the target tissues. However, four autoimmune nondegenerative diseases--adult advanced type 1 diabetes mellitus, Hashimoto's thyroiditis, Graves' disease, and advanced primary biliary cholangitis--are refractory to these drugs. This article suggests that the refractoriness of certain autoimmune diseases is due to near-total loss of secreting cells coupled with the extremely low regenerative capacity of the affected tissues. The near-complete destruction of cells responsible for secreting insulin, thyroid hormones, or biliary HCO3 -- diminishes the protective effects of immunosuppressants against further damage. The slow regeneration rate of these cells hinders tissue recovery, even after drug-induced immune suppression, thus preventing remission. Although the liver can fully regenerate after injury, severe primary biliary cholangitis may impair this ability, preventing liver recovery. Consequently, these four autoimmune diseases are resistant to immunosuppressive drugs and corticosteroids. In contrast, early stages of type 1 diabetes and early primary biliary cholangitis, where damage to secreting cells is partial, may benefit from immunosuppressant treatment. In contrast to these four diseases, chronic degenerative autoimmune conditions like multiple sclerosis may respond positively to corticosteroid use despite the limited regenerative potential of the affected tissue (the central nervous system). The opposite is true for acute autoimmune conditions like Guillain--Barré syndrome. [ABSTRACT FROM AUTHOR]

Background: Primary biliary cholangitis (PBC) can impact both the quality of life and the survival of patients. The study aimed to determine the survival rate and associated variables in patients with PBC. Materials and Methods: This cohort research comprised 65 patients diagnosed with PBC who were admitted to the pathology section between January 2010 and December 2019. Survival was determined by reviewing hospital medical data and following up with the patients. The impact of demographic factors, clinical, laboratory, and histopathological aspects on patient survival time was investigated using Kaplan-Meier survival analysis and Cox regression. Results: The average period of follow-up was 6.25 years with a standard deviation of 3.2 years. In surviving patients, the baseline bilirubin level was 2.83, but in deceased or transplanted patients, it was 8.95 (P = 0.002). The baseline albumin level was 3.99 in surviving patients and 3.66 in deceased or transplanted patients (P = 0.024). The incidence of cirrhosis in those who survived was 1.8%, but in patients who died or underwent a transplant, it was 40%. Out of 65 cases, 3 patients (4.7%) died and 7 (10%) had liver transplants. Survival rates of patients vary based on factors such as jaundice (P = 0.002), weariness (P = 0.03), cirrhosis (P < 0.001), and vitiligo (P = 0.033). There were notable variations in the average Mayo score between the two groups of patients who had liver transplantation and survived, with scores of 7.21 and 5.61, respectively. Conclusion: The study found that aspartate aminotransferase and alanine aminotransferase levels, baseline and final bilirubin, albumin, antinuclear antibody, the presence of cirrhosis, and jaundice significantly influenced patient survival with PBC. [ABSTRACT FROM AUTHOR]

Autoimmune diseases of the liver and biliary tract require timely and accurate diagnosis. This study evaluates the D-tek panel (D-Tek, Mons, Belgium) of 10 immunodot antigens for its effectiveness in diagnosing autoimmune hepatitis (AIH) and primary biliary cholangitis (PBC). We retrospectively analysed serum samples from 111 patients who had undergone routine testing, including indirect immunofluorescence (IIF) and enzyme-linked immunosorbent assays (ELISA), to confirm or exclude autoimmune liver or biliary tract disease. The panel tested for M2/nPDC, M2/OGDC-E2, M2/BCOADC-E2, M2/PDC-E2, gp210, sp100, LKM1, LC1, SLA, and F-actin antigens. Results showed that all positive IIF+ELISA results were confirmed by the immunodot panel, except for two samples from patients who had never been diagnosed with AIH. The immunodot test identified over 20 additional autoantibodies in samples initially negative by IIF, corroborated by laboratory imaging and medical history. The immunodot technique proved to be a quick, sensitive, and specific method with high overall accuracy. This study suggests that the immunodot technique may be an effective screening and confirmatory method for autoimmune liver diseases, potentially improving diagnostic efficiency and accuracy in clinical practice. [ABSTRACT FROM AUTHOR]

Primary biliary cholangitis (PBC) is a chronic autoimmune cholestatic disease characterized by the destruction of the small intrahepatic bile ducts, which can progress to liver cirrhosis. The gold standard in the treatment of PBC is ursodeoxycholic acid (UDCA), which is indicated in all patients with PBC because it improves not only biochemical parameters but also patients' survival. An important milestone in the identification of patients at risk is the assessment of biochemical response to UDCA. Patients who respond to treatment have a lower incidence of hepatic events and better prognosis than patients who do not. Several scoring systems can be used to assess the response and identify non-responders who will benefit from second-line treatment. Obeticholic acid (OCA) is currently the only approved second-line treatment for PBC, which is effective for non-responders to UDCA therapy or patients, who have not tolerated UDCA therapy. However, OCA is contraindicated in advanced liver cirrhosis and portal hypertension. Moreover, pruritus may be a limiting factor for the administration of OCA. Fibrates have shown promising data supporting their use in non-responders to UDCA because they improve the biochemical parameters and elastographic findings and have possible antipruritic effects. Therefore, the idea of a triple treatment seems interesting. Clinical research is focusing on several other groups of drugs: peroxisome proliferator-activated receptor (PPAR) δ- and α/δ agonists, non-steroidal farnesoid X receptor agonists, fibroblast growth factor 19 modulators, and inhibitors of nicotinamide adenine dinucleotide phosphate oxidase 1 and 4. [ABSTRACT FROM AUTHOR]

Primary biliary cholangitis (PBC), formerly known as primary biliary cirrhosis, is a liver disease. This is a chronic autoimmune disease, which can worsen over time and recur periodically. If left untreated, biliary cirrhosis may lead to liver failure. Considering that PBC is a rare disease in all age groups, especially in children, the purpose of this report was to describe a girl with hepatitis A virus (HAV) who was then diagnosed with PBC. This child is the first case reported in Iran. The patient is a 7-year-old girl with a pale complexion and eyes with a yellowish cross who was referred to the doctor. Considering that this patient had hepatitis, a blood test was requested to check the level of cholesterol and liver enzymes. The absence of cholestatic liver enzymes was observed in tests. Then, an anti-mitochondrial (AMA) test was requested for the patient, the result of which was negative. Finally, with imaging and biopsy, the diagnosis of PBC was confirmed for the patient. After the definite diagnosis of the disease, the child was treated with ursodeoxycholic acid (UDCA). The child in question is suffering from two medical and immunological diseases, HAV and PBC. Since this child was first infected with HAV, it is possible that the cause of PBC was HAV. [ABSTRACT FROM AUTHOR]

LINKED CONTENT: This article is linked to Mayo et al papers. To view these articles, visit https://doi.org/10.1111/apt.17755 and https://doi.org/10.1111/apt.17798 [ABSTRACT FROM AUTHOR]

Anti-mitochondrial antibody (AMA) is not always present in patients with primary biliary cholangitis (PBC). We aimed to determine the additional value of anti-hexokinase 1 (anti-HK1) and anti-kelch-like 12 (anti-KLHL12) antibody in PBC and analyzed the biochemical and immunological parameters of 212 subjects, including PBC patients and healthy controls. Serum anti-gp210 and sp100 antibodies were determined by an immunoblotting test (IBT). Enzyme-linked immunosorbent assay (ELISA) was employed to evaluate anti-HK1 and anti-KLHL12. The diagnostic value of anti-HK1 and anti-KLHL12 to PBC was analyzed by constructing a receiver operating characteristic (ROC) curve. ROC analyses didn't show a very good performance of serum anti-HK1 for PBC diagnosis; the AUC was 0.664 with a sensitivity of 53.3 % and a specificity of 79.2 %. Regarding anti-KLHL12, ROC analysis yielded an AUC of 0.626, with a sensitivity of 45.7 % and a specificity of 93.8 %. For AMA-negative PBC patients, the AUC increased to 0.790 for KLHL12, and 0.708 for HK1. AMA combined with anti-HK1 or anti-KLHL12 antibody significantly improved the diagnostic sensitivity of PBC from 82 to about 95 %, respectively. In AMA-negative PBC patients, the sensitivities for anti-HK1 (62.50 %) and anti-KLHL12 (75 %) antibodies were higher than for anti-gp210 (37.5 %) and anti-sp100 antibody (43.75 %). When these four antibodies were combined, the overall sensitivity increased to 87.5 %. The determination of anti-HK1 and anti-KLHL12 facilitates the diagnosis of PBC, particularly in AMA-negative patients. Adding anti-HK1 and anti-KLHL12 antibodies to clinical detection enables early diagnosis and timely treatment, potentially improving patient prognosis. [ABSTRACT FROM AUTHOR]

In assessing individual cardiovascular risk, dyslipidemia is known for emerging as a pivotal factor significantly contributing to major cardiovascular events. However, dyslipidemic patients frequently present with concurrent medical conditions, each with varying frequencies of occurrence; cholangitis, whether acute or chronic, and hepatic steatosis, along with associated conditions, are strongly associated with specific forms of dyslipidemia, and these associations are reasonably well elucidated. Conversely, evidence linking biliary disease to hepatic steatosis is comparatively scant. This narrative review aims to bridge this gap in knowledge concerning the interplay between dyslipidemia, cholangitis, and hepatic steatosis. By addressing this gap, clinicians can better identify patients at heightened risk of future major cardiovascular events, facilitating more targeted interventions and management strategies. The review delves into the intricate relationships between dyslipidemia and these hepatic and biliary clinical conditions, shedding light on potential mechanisms underlying their associations. Understanding these complex interactions is crucial for optimizing cardiovascular risk assessment as well and devising tailored treatment approaches for patients with dyslipidemia and associated hepatic disorders. Moreover, elucidating these connections empowers clinicians with the knowledge needed to navigate the multifaceted landscape of cardiovascular risk assessment and management effectively. By exploring the intricate relationships between dyslipidemia, cholangitis, and hepatic steatosis (without forgetting the possible clinical consequences of hepatic steatosis itself), this review not only contributes to the existing body of knowledge but also offers insights into potential avenues for further research and clinical practice. Thus, it serves as a valuable resource for healthcare professionals striving to enhance patient care and outcomes in the context of cardiovascular disease and associated hepatic conditions. [ABSTRACT FROM AUTHOR]

Primary biliary cholangitis (PBC) is a cholestatic liver disease characterized by immune-mediated injury to small bile ducts. Although PBC is an autoimmune disease, the effectiveness of conventional immunosuppressive therapy is disappointing. Nearly 40% of PBC patients do not respond to the first-line drug UDCA. Without appropriate intervention, PBC patients eventually progress to liver cirrhosis and even death. There is an urgent need to develop new therapies. The gut–liver axis emphasizes the interconnection between the gut and the liver, and evidence is increasing that gut microbiota and bile acids play an important role in the pathogenesis of cholestatic diseases. Dysbiosis of gut microbiota, imbalance of bile acids, and immune-mediated bile duct injury constitute the triad of pathophysiology in PBC. Autoimmune cholangitis has the potential to be improved through immune system modulation. Considering the failure of conventional immunotherapies and the involvement of gut microbiota and bile acids in the pathogenesis, targeting immune factors associated with them, such as bile acid receptors, microbial-derived molecules, and related specific immune cells, may offer breakthroughs. Understanding the gut microbiota–bile acid network and related immune dysfunctions in PBC provides a new perspective on therapeutic strategies. Therefore, we summarize the latest advances in research of gut microbiota and bile acids in PBC and, for the first time, explore the possibility of related immune factors as novel immunotherapy targets. This article discusses potential therapeutic approaches focusing on regulating gut microbiota, maintaining bile acid homeostasis, their interactions, and related immune factors. [ABSTRACT FROM AUTHOR]

Background: Previous studies have suggested that the gut microbiota (GM) is closely associated with the development of autoimmune cholestatic liver disease (ACLD), but limitations, such as the presence of confounding factors, have resulted in a causal relationship between the gut microbiota and autoimmune cholestatic liver disease that remains uncertain. Thus, we used two-sample Mendelian randomization as a research method to explore the causal relationship between the two. Methods: Pooled statistics of gut microbiota from a meta-analysis of genomewide association studies conducted by the MiBioGen consortium were used as an instrumental variable for exposure factors. The Pooled statistics for primary biliary cholangitis (PBC) and primary sclerosing cholangitis (PSC) were obtained from the R9 version of the FinnGen database (https://r9.finngen.fi/). Inversevariance Weighted (IVW), cML-MA, MR-Egger regression, Weighted median (WME), Weighted mode (WM), and Simple mode (SM) were used to detect the association between intestinal flora and the causal relationship between intestinal flora and ACLD, in which IVW method was dominant, was assessed based on the effect indicator dominance ratio (odds ratio, OR) and 95% confidence interval (CI). Sensitivity analysis, heterogeneity test, gene pleiotropy test, MR pleiotropy residual sum and outlier test (MR-PRESSO) were combined to verify the stability and reliability of the results. Reverse Mendelian randomization analysis was performed on gut microbiota and found to be causally associated with ACLD. Results: The IVW results showed that the relative abundance of the genus Clostridium innocuum group, genus Butyricicoccus, and genus Erysipelatoclostridium was negatively correlated with the risk of PBC, that is, increased abundance reduced the risk of PBC and was a protective, and the relative abundance of the genus Eubacterium hallii was positively correlated with the risk of PSC, which is a risk factor for PSC. Family Clostridiaceae1 and family Lachnospiraceae were negatively correlated with the risk of PSC, which is a protective factor for PSC. Conclusion: This study found a causal relationship between gut microbiota and ACLD. This may provide valuable insights into gut microbiota-mediated pathogenesis of ACLD. It is necessary to conduct a large-sample randomized controlled trial (RCT) at a later stage to validate the associated role of the relevant gut microbiota in the risk of ACLD development and to explore the associated mechanisms. [ABSTRACT FROM AUTHOR]

Objective: Vitamins and homocysteine (Hcy) are involved in liver metabolism and related to the pathogenesis of autoimmune liver disease (AILD), but consensus is lacking. This study aims to systematically summarize relevant evidence to clarify the association of serum vitamins and Hcy levels with AILD. Methods: The English and Chinese literature was searched until August 29, 2023. Studies were included if they were observational studies of investigating serum vitamins and Hcy levels in patients with AILD and their healthy comparisons. Quality assessment was performed by using the Newcastle–Ottawa Scale, and a meta‐analysis was conducted using ReviewManager 5.3. The protocol was registered in the international prospective register of systematic reviews (PROSPERO), with registration number CRD42023455367. Results: A total of 25 case–control studies comprising 3487 patients (1673 patients and 1814 healthy controls) were included for analysis. There were 548 autoimmune hepatitis (AIH) cases, 1106 primary biliary cholangitis (PBC) cases, and 19 primary sclerosing cholangitis (PSC) cases. We found that serum A and E were decreased in both AIH and PBC/PSC; but vitamin C was reduced only in patients with PBC, not AIH. In addition, decreased content of 25(OH)D3 was found in both AIH and PBC. However, levels of 25(OH)D did not differ between the patients and controls, and were independent of disease types and the country. Only one study that met the inclusion criteria reported vitamin B6, B9, B12, and Hcy changes, and found that vitamin B6 and B9 were significantly decreased in patients with PBC, while serum vitamin B12 and Hcy levels were significantly elevated in them. One eligible study each confirmed a reduction in plasma vitamin K1 and 1,25(OH)2D3 in patients with PBC. Conclusion: Most vitamins are deficient in AILD, so appropriate vitamin supplementation should be necessary. Further studies with larger sample sizes are needed to validate these findings. [ABSTRACT FROM AUTHOR]

Gender Medicine has had an enormous expansion over the last ten years. Autoimmune liver diseases include several conditions, i.e., autoimmune hepatitis (AIH), primary biliary cholangitis (PBC), primary sclerosing cholangitis (PSC), and conditions involving the liver or biliary tree overlapping with AIH, as well as IgG4-related disease. However, little is known about the impact of sex in the pathogenesis and natural history of these conditions. The purpose of this review is to provide an update of the gender disparities among the autoimmune liver diseases by reviewing the data published from 1999 to 2023. The epidemiology of these diseases has been changing over the last years, due to the amelioration of knowledge in their diagnosis, pathogenesis, and treatment. The clinical data collected so far support the existence of sex differences in the natural history of autoimmune liver diseases. Notably, their history could be longer than that which is now known, with problems being initiated even at a pediatric age. Moreover, gender disparity has been observed during the onset of complications related to end-stage liver disease, including cancer incidence. However, there is still an important debate among researchers about the impact of sex and the pathogenesis of these conditions. With this review, we would like to emphasize the urgency of basic science and clinical research to increase our understanding of the sex differences in autoimmune liver diseases. [ABSTRACT FROM AUTHOR]

Background: Primary biliary cholangitis (PBC) is a chronic biliary autoimmune liver disease characterized by intrahepatic cholestasis. Swertia mussotii Franch. (SMF) is a Tibetan medicine with hepatoprotective and anti-inflammatory activities. In this study, the therapeutic effect and potential mechanisms of SMF on PBC were investigated by bioinformatics analysis and in vitro experimental validation, with the aim of promoting the progress of SMF and PBC research. Methods: We first explored the therapeutic effects and key targets of SMF on PBC using a network pharmacology approach, further screened the core targets using the GSE79850 dataset, and finally validated the results using molecular docking techniques and in vitro experiments. Results: By bioinformatics analysis, we identified core targets of SMF for PBC treatment (STAT3, JAK2, TNF-α, and IL-1β) and important signaling pathways: JAK-STAT, TNF, and PI3K-AKT. The molecular docking results showed that the significant components of SMF had good binding properties to the core targets. In vitro experiments showed that SMF extracts improved the extent of epithelial-mesenchymal transition in human intrahepatic biliary epithelial cells and had a significant reversal effect on epithelial-mesenchymal transition process markers and potential targets in PBC. Conclusion: SMF may exert its therapeutic effects on PBC by acting on important targets such as STAT3, JAK2, TNF-α, IL-1β, Vimentin, and E-cadherin and the pathways in which they are involved. [ABSTRACT FROM AUTHOR]

Background and Aim: Primary biliary cholangitis (PBC) is a chronic autoimmune cholangiopathy, characterized by the presence of some autoantibodies in the serum. This study aimed to evaluate the clinical significance of AMA‐M2, anti‐gp210 and anti‐sp100 antibody levels detected by multiplex bead‐based flow fluorescent immunoassay (MBFFI) in PBC. Methods: This study cohort included 238 PBC patients, 81 autoimmune hepatitis (AIH) patients, 62 systemic lupus erythematosus (SLE) patients, and 118 healthy controls. Serum AMA‐M2, anti‐gp210 and anti‐sp100 antibody were detected by MBFFI and immunoblotting assay (IBT). The relationship between three antibody levels and cirrhosis, liver function, cholestasis markers and therapeutic effect to ursodesoxycholic acid (UDCA) was evaluated in PBC. Results: MBFFI were presented good coincidence rate (87.39%–95.38%) with IBT. The level of AMA‐M2, anti‐gp210 and anti‐sp100 antibodies in PBC patients were higher than other disease group and healthy controls (p ＜.01). When compared with the healthy controls group, the AUC of AMA‐M2, anti‐gp210 and anti‐sp100 antibodies were 0.9245, 0.7619, and 0.6789, respectively. In addition, gp210 antibody levels have diagnostic value in patients with liver cirrhosis (AUC: 0.7567). We found that when combine detect these three antibodies, the sensitivity was higher than individually detection. High level of serum anti‐gp210 antibody could be related to worse liver function and more severe cholestasis in PBC patients. Moreover, serum antibody levels may decrease or remained flat in patients who responded well to UDCA. Conclusion: The detection of AMA‐M2, anti‐gp210 and anti‐sp100 antibody levels by MBFFI showed good performance in the diagnosis of PBC. Serum anti‐gp210 antibody level is related to cirrhosis, poor liver function and severe cholestasis in PBC. [ABSTRACT FROM AUTHOR]

Keywords: anti-mitochondrial antibodies; HEp-2 cell lines; IFI; murine triple-tissue; primary biliary cholangitis EN anti-mitochondrial antibodies HEp-2 cell lines IFI murine triple-tissue primary biliary cholangitis e142 e143 2 07/13/20 20200801 NES 200801 To the Editor, We read with interest the paper by Florin et al. [[1]] on the prevalence of primary biliary cholangitis (PBC)-related antibodies in patients with systemic sclerosis. These authors found a discrepancy between the two methods - indirect immunofluorescence (IIF) on murine triple-tissue sections (liver-kidney-stomach - LKS) and lineblot - used in their study to detect anti-mitochondrial antibodies (AMA). Anti-mitochondrial antibodies, HEp-2 cell lines, IFI, murine triple-tissue, primary biliary cholangitis. [Extracted from the article]

Clinical observations suggest that the prevalence of autoimmune diseases is changing over time. Both autoimmune liver diseases and multiple sclerosis have shown a significant increase in the last decades. Although the coexistence of autoimmune diseases within individuals and families is a common phenomenon, the extent to which liver disease and multiple sclerosis co-occur is not clear. Case reports and few studies have reported the possible coexistence of multiple sclerosis with thyroid diseases, inflammatory bowel disease, psoriasis, and rheumatoid arthritis. It is unknown whether there is a definite association between multiple sclerosis and autoimmune liver diseases. We reviewed the literature to summarize the available studies on the association between different autoimmune liver diseases (autoimmune hepatitis, primary biliary cholangitis, and primary sclerosing cholangitis) and treated or untreated multiple sclerosis. [ABSTRACT FROM AUTHOR]

Primary biliary cholangitis (PBC) is a chronic, progressive autoimmune disease whose natural course leads to hepatic cirrhosis and failure, with an eventual need for liver transplantation. Treatment with ursodeoxycholic acid delays disease progression, but unfortunately, many patients do not respond to therapy. Obeticholic acid has shown promise as adjuvant therapy for incomplete responders but may exacerbate symptoms or even allow disease progression. Fibrates have been investigated for PBC and have shown therapeutic promise. However, they have been linked to rare but potentially severe cases of liver injury. In this review, we present an overview of PBC along with current therapies approved by the U.S. Food and Drug Administration (the literature was reviewed on PubMed from August 2022 through November 2023). We will also discuss fibrate efficacy, overall safety, and the rare cases of liver injury. We find that, overall, fibrates are a relatively safe and effective adjuvant treatment for PBC. [ABSTRACT FROM AUTHOR]