keyword
https://read.qxmd.com/read/38002661/primary-biliary-cholangitis-pbc-autoimmune-hepatitis-aih-variant-syndrome-clinical-features-response-to-therapy-and-long-term-outcome
#21
JOURNAL ARTICLE
Markus Graf, Christian M Lange, Mona M Langer, Jörn M Schattenberg, Jessica Seessle, Julia Dietz, Annika Vermehren, Florian A Michael, Antonia Mondorf, Stefan Zeuzem, Anita Pathil, Christiana Graf
INTRODUCTION: Standardization of diagnostic criteria of autoimmune hepatitis (AIH) and primary biliary cholangitis (PBC) variant syndrome (AIH-PBC VS) has not been achieved so far and evidence-based recommendations for monitoring and treatment of the disease are still lacking. Our study aimed to assess the prevalence, biochemical, and serological features, as well as the clinical course, of VS. METHODS: We performed a retrospective study including all patients with VS between 1999 and 2020 in four German centers...
November 11, 2023: Journal of Clinical Medicine
https://read.qxmd.com/read/37954487/accuracy-of-controlled-attenuation-parameter-measurement-for-the-detection-of-steatosis-in-autoimmune-liver-diseases
#22
JOURNAL ARTICLE
Silja Steinmann, Johannes Hartl, Sören Weidemann, Katja Füssel, Claudia Kroll, Marcial Sebode, Ansgar Wilhelm Lohse, Christoph Schramm
BACKGROUND & AIMS: Concurrent fatty liver disease represents an emerging challenge in the care of individuals with autoimmune liver diseases (AILD). Therefore, we aimed to validate the ultrasound-based method of controlled-attenuation parameter (CAP) as a non-invasive tool to detect hepatic steatosis in individuals with AILD. METHODS: The diagnostic performance of CAP to determine biopsy-proven hepatic steatosis (>5%) was assessed in individuals with AILD (autoimmune hepatitis [AIH], primary biliary cholangitis [PBC], primary biliary cholangitis [PSC], or variant syndromes) who underwent liver biopsy at the University Medical Center Hamburg-Eppendorf between 2015-2020 by calculating the area under the receiver operating characteristic (AUROC) curves...
December 2023: JHEP reports: innovation in hepatology
https://read.qxmd.com/read/37900994/diagnostic-role-of-transient-elastography-in-patients-with-autoimmune-liver-diseases-a-systematic-review-and-meta-analysis
#23
JOURNAL ARTICLE
Hong Chen, Yue Shen, Sheng-Di Wu, Qin Zhu, Cheng-Zhao Weng, Jun Zhang, Mei-Xia Wang, Wei Jiang
BACKGROUND: Noninvasive methods have been developed to detect fibrosis in many liver diseases due to the limits of liver biopsy. However, previous studies have focused primarily on chronic viral hepatitis and nonalcoholic fatty liver disease. The diagnostic value of transient elastography for autoimmune liver diseases (AILDs) is worth studying. AIM: To compare the diagnostic accuracy of imaging techniques with serum biomarkers of fibrosis in AILD. METHODS: The PubMed, Cochrane Library and EMBASE databases were searched...
October 21, 2023: World Journal of Gastroenterology: WJG
https://read.qxmd.com/read/37877134/a-novel-web-based-online-nomogram-to-predict-advanced-liver-fibrosis-in-patients-with-autoimmune-hepatitis-primary-biliary-cholangitis-overlap-syndrome
#24
JOURNAL ARTICLE
Zhiyi Zhang, Jian Wang, Yun Chen, Yiguang Li, Li Zhu, Huali Wang, Yilin Liu, Jiacheng Liu, Shengxia Yin, Xin Tong, Xiaomin Yan, Yuxin Chen, Chuanwu Zhu, Jie Li, Yuanwang Qiu, Chao Wu, Rui Huang
BACKGROUND: Patients with autoimmune hepatitis-primary biliary cholangitis (AIH-PBC) overlap syndrome have a worse prognosis compared to AIH or PBC alone and accurately predicting the severity and dynamically monitoring the progression of disease are therefore essential. We aimed to develop a nomogram-based model to predict advanced liver fibrosis in patients with AIH-PBC overlap syndrome. METHODS: A total of 121 patients with AIH-PBC overlap syndrome were retrospectively included and randomly assigned to a development set and a validation set...
December 2023: Journal of translational autoimmunity
https://read.qxmd.com/read/37832129/the-clinical-value-of-the-monocyte-to-high-density-lipoprotein-cholesterol-ratio-and-alkaline-phosphatase-to-platelet-ratio-in-primary-biliary-cholangitis
#25
JOURNAL ARTICLE
Yuehong Lao, Xi Li, Wei Zhou, Haiqing Zhu, Yanting Jiang, Sihui Li, Min Jin, Jian Wang
This study aimed to evaluate the clinical value of the monocyte to high-density lipoprotein cholesterol ratio (MHR) and alkaline phosphatase-to-platelet ratio (APPR) in the diagnosis and prognosis of primary biliary cholangitis (PBC). Clinical and laboratory data were retrospectively collected and analyzed from 92 PBC patients, 92 patients with autoimmune hepatitis (AIH), 120 patients with chronic hepatitis B (CHB) and 124 healthy controls (HCs). We compared the levels of MHR and APPR among the groups with PBC, AIH, CHB and HCs, and analyzed the correlations between MHR and APPR with laboratory indices including aspartate aminotransferase platelet ratio index, fibrosis index based on 4 factors, and Mayo score in PBC...
October 13, 2023: Medicine (Baltimore)
https://read.qxmd.com/read/37823420/autoantibodies-to-beta-tubulin-in-autoimmune-liver-diseases-relation-to-panca-and-clinical-relevance
#26
JOURNAL ARTICLE
Beate Preuß, Amelie Frank, Birgit Terjung, Ulrich Spengler, Christoph Berg, Reinhild Klein
There was evidence that pANCA (perinuclear antineutrophil cytoplasmic antibodies) in autoimmune liver diseases react with human beta-tubulin-5 (TBB5). Here we re-evaluate the specificity and clinical relevance of anti-TBB5 antibodies. Patients with untreated autoimmune hepatitis (AIH; n=53), AIH under immunosuppressive therapy (AIH-IS; n=125), primary sclerosing cholangitis (PSC; n=40), primary biliary cholangitis (PBC; n=250), non-autoimmune liver diseases (n=158), inflammatory bowel diseases (IBD; n=30), and healthy individuals (n=62) were tested by enzyme linked immunosorbent assay (ELISA) for IgG- and IgA-antibodies against recombinant human TBB5...
October 12, 2023: Clinical and Experimental Immunology
https://read.qxmd.com/read/37818371/regulatory-t-cell-deficiency-leads-to-features-of-autoimmune-liver-disease-overlap-syndrome-in-scurfy-mice
#27
JOURNAL ARTICLE
Kaan Yilmaz, Stefanie Haeberle, Yong Ook Kim, Marvin J Fritzler, Shih-Yen Weng, Benjamin Goeppert, Verena K Raker, Kerstin Steinbrink, Detlef Schuppan, Alexander Enk, Eva N Hadaschik
INTRODUCTION: Scurfy mice have a complete deficiency of functional regulatory T cells (Treg) due to a frameshift mutation in the Foxp3 gene. The impaired immune homeostasis results in a lethal lymphoproliferative disorder affecting multiple organs, including the liver. The autoimmune pathology in scurfy mice is in part accompanied by autoantibodies such as antinuclear antibodies (ANA). ANA are serological hallmarks of several autoimmune disorders including autoimmune liver diseases (AILD)...
2023: Frontiers in Immunology
https://read.qxmd.com/read/37789337/causal-effects-of-gut-microbiome-on-autoimmune-liver-disease-a-two-sample-mendelian-randomization-study
#28
JOURNAL ARTICLE
Yugang Fu, Jiacheng Li, Yingying Zhu, Chong Chen, Jing Liu, Simin Gu, Yiyuan Zheng, Yong Li
BACKGROUND: Epidemiological studies have indicated a potential link between the gut microbiome and autoimmune liver disease (AILD) such as autoimmune hepatitis (AIH), primary biliary cholangitis (PBC), and primary sclerosing cholangitis (PSC). The relationship between the gut microbiome and autoimmune liver disease is still uncertain due to confounding variables. In our study, we aim to shed light on this relationship by employing a two-sample Mendelian randomization approach. METHODS: We conducted a two-sample Mendelian randomization (MR) study using the R package "TwoSampleMR"...
October 3, 2023: BMC Medical Genomics
https://read.qxmd.com/read/37752719/autoimmune-liver-diseases-and-diabetes-a-propensity-score-matched-analysis-and-a-proportional-meta-analysis
#29
JOURNAL ARTICLE
Anne-Sofie H Jensen, Marie Winther-Sørensen, Johan Burisch, Annika Bergquist, Henriette Ytting, Lise L Gluud, Nicolai J Wewer Albrechtsen
BACKGROUND AND AIMS: Patients with some chronic liver diseases have increased risk of diabetes. Whether this is also the case for patients with autoimmune liver diseases is unknown. The study aimed to calculate risk and worldwide prevalence of diabetes in patients with autoimmune hepatitis (AIH), primary biliary cholangitis (PBC) and primary sclerosing cholangitis (PSC). METHODS: We performed a case-control study using data from the United Kingdom Biobank (UKB) and compared frequency of type 1 diabetes (T1D) and type 2 diabetes (T2D) in AIH and PBC with age-, sex-, BMI- and ethnicity-matched controls...
September 26, 2023: Liver International: Official Journal of the International Association for the Study of the Liver
https://read.qxmd.com/read/37586960/autoimmune-hepatitis-primary-biliary-cholangitis-overlaps-a-syndrome-both-well-understood-and-poorly-understood
#30
EDITORIAL
Oyedele A Adeyi
No abstract text is available yet for this article.
August 10, 2023: Annals of Diagnostic Pathology
https://read.qxmd.com/read/37569584/combined-salivary-proteome-profiling-and-machine-learning-analysis-provides-insight-into-molecular-signature-for-autoimmune-liver-diseases-classification
#31
JOURNAL ARTICLE
Giulia Guadalupi, Cristina Contini, Federica Iavarone, Massimo Castagnola, Irene Messana, Gavino Faa, Simona Onali, Luchino Chessa, Rui Vitorino, Francisco Amado, Giacomo Diaz, Barbara Manconi, Tiziana Cabras, Alessandra Olianas
Autoimmune hepatitis (AIH) and primary biliary cholangitis (PBC) are autoimmune liver diseases that target the liver and have a wide spectrum of presentation. A global overview of quantitative variations on the salivary proteome in presence of these two pathologies is investigated in this study. The acid-insoluble salivary fraction of AIH and PBC patients, and healthy controls (HCs), was analyzed using a gel-based bottom-up proteomic approach combined with a robust machine learning statistical analysis of the dataset...
July 30, 2023: International Journal of Molecular Sciences
https://read.qxmd.com/read/37564051/antibodies-against-multiple-post-translationally-modified-proteins-aid-in-diagnosis-of-autoimmune-hepatitis-and-associate-with-complete-biochemical-response-to-treatment
#32
JOURNAL ARTICLE
Michelle D van den Beukel, Anna E C Stoelinga, Adriaan J van der Meer, Stef van der Meulen, Lu Zhang, Maarten E Tushuizen, Bart van Hoek, Leendert A Trouw
BACKGROUND: (Auto)immune mediated and cholestatic liver disease (AILD) includes autoimmune hepatitis (AIH), primary biliary cholangitis (PBC) and primary sclerosing cholangitis (PSC). Especially AIH is characterized by the presence of autoantibodies and elevated serum immunoglobulins. In rheumatoid arthritis, autoantibodies against post-translational modifications (PTMs) such as citrullination (Cit) and carbamylation (CarP) are used as diagnostic and prognostic markers, respectively. We studied the presence of six anti-PTM antibodies in patients with the three AILDs and non-AILD...
2023: Frontiers in Medicine
https://read.qxmd.com/read/37505225/itaconate-inhibits-cd103-trm-cells-and-alleviates-hepatobiliary-injury-in-mouse-models-of-primary-sclerosing-cholangitis
#33
JOURNAL ARTICLE
Yikang Li, Bo Li, Xiao Xiao, Qiwei Qian, Rui Wang, Zhuwan Lyu, Ruiling Chen, NaNa Cui, Yiyan Ou, Xiting Pu, Qi Miao, Qixia Wang, Min Lian, M Eric Gershwin, Ruqi Tang, Xiong Ma, Zhengrui You
BACKGROUND AIMS: Primary sclerosing cholangitis (PSC) is a chronic progressive liver disease characterized by infiltration of intrahepatic tissue-resident memory CD8+ T cells (TRM). Itaconate has demonstrated therapeutic potential in modulating inflammation. An unmet need for PSC is the reduction of biliary inflammation, and we hypothesized that itaconate may directly modulate pathogenic TRM. METHODS: The numbers of intrahepatic CD103+ TRM were evaluated by immunofluorescence in PSC (n = 32) and serum levels of itaconate in PSC (n = 64), primary biliary cholangitis (PBC) (n = 60), autoimmune hepatitis (AIH) (n = 49), and healthy controls (n = 109) were determined by LC-MS/MS...
July 26, 2023: Hepatology: Official Journal of the American Association for the Study of Liver Diseases
https://read.qxmd.com/read/37488645/overlap-syndrome-of-autoimmune-hepatitis-and-primary-biliary-cholangitis-complicated-with-atypical-hepatocellular-carcinoma-a-case-report
#34
JOURNAL ARTICLE
Teodora Surdea-Blaga, Roxana L Cărăguț, Cosmin Caraiani, Zeno Spârchez, Nadim Al Hajjar, Dan L Dumitrașcu
BACKGROUND: Hepatocellular carcinoma (HCC) is a primary tumor of the liver. The majority of HCCs are associated most frequently with chronic B or C viral hepatitis, alcohol intake or aflatoxin exposure. Cirrhosis is a strong risk factor associated with HCC. The causes of liver cirrhosis are chronic viral hepatitis, alcohol intake, metabolic diseases (NAFLD), hemocromathosis, alfa 1 antitrypsisn deficiency. All aetiologic forms of cirrhosis are at risk to be complicated by HCC development, but the risk is higher for patients diagnosed with chronic viral hepatitis...
July 25, 2023: Journal of Medical Case Reports
https://read.qxmd.com/read/37468373/histology-and-clinical-correlations-in-autoimmune-hepatitis-primary-biliary-cholangitis-and-autoimmune-hepatitis-primary-biliary-cholangitis-overlap-syndrome
#35
JOURNAL ARTICLE
Yuanxin Liang, Binny Khandakar, Yansheng Hao, Yiqin Xiong, Bella L Liu, Xuchen Zhang
OBJECTIVES: The diagnosis of autoimmune hepatitis (AIH), primary biliary cholangitis (PBC), and AIH-PBC overlap syndrome (OS) relies on their histologic features and clinical findings. In this study, we aimed to identify specific morphologic features of these diseases and evaluate their clinical correlation. METHODS: We included initial biopsies from untreated patients with AIH (n = 14), PBC (n = 10), and OS (n = 7). Histologic features of the portal tract, portal-lobular interface, and hepatic lobule, fibrosis, as well as clinical data including serology, autoantibodies, treatment, and prognosis were reviewed and analyzed...
December 2023: Annals of Diagnostic Pathology
https://read.qxmd.com/read/37398174/novel-autoantibody-targets-identified-in-patients-with-autoimmune-hepatitis-aih-by-phip-seq-reveals-pathogenic-insights
#36
Arielle Klepper, Andrew Kung, Sara E Vazquez, Anthea Mitchell, Sabrina Mann, Kelsey Zorn, Isaac Avila-Vargas, Swathi Kari, Melawit Tekeste, Javier Castro, Briton Lee, Maria Duarte, Mandana Khalili, Monica Yang, Paul Wolters, Jennifer Price, Emily Perito, Sandy Feng, Jacquelyn J Maher, Jennifer Lai, Christina Weiler-Normann, Ansgar W Lohse, Joseph DeRisi, Michele Tana
Autoimmune hepatitis (AIH) is a severe autoimmune disease, characterized by the presence of autoantibodies. However, the role of autoantibodies in the pathophysiology of AIH remains uncertain. Here, we employed Phage Immunoprecipitation-Sequencing (PhIP-Seq) to identify novel autoantibodies in AIH. Using these results, a logistic regression classifier was able to predict which patients had AIH, indicating the presence of a distinct humoral immune signature. To further investigate the autoantibodies most specific to AIH, significant peptides were identified relative to a broad array of controls (298 patients with non-alcoholic fatty liver disease (NAFLD), primary biliary cholangitis (PBC), or healthy controls)...
June 27, 2023: medRxiv
https://read.qxmd.com/read/37395201/prevalence-of-autoimmune-cholestatic-and-nonalcoholic-fatty-liver-disease-in-celiac-disease
#37
JOURNAL ARTICLE
Asif Ali Hitawala, Ashraf Almomani, Somtochukwu Onwuzo, Antoine Boustany, Prabhat Kumar, Imad Asaad
BACKGROUND: While there is higher prevalence of autoimmune, cholestatic and fatty liver disease in celiac disease (CeD), most data is from small-scale studies. We evaluated the prevalence and risk factors of the same using large cohort data. METHODS: A population-based cross-sectional study was conducted using Explorys, a multi-institutional database. Prevalence and risk factors of autoimmune hepatitis (AIH), primary biliary cholangitis (PBC), primary sclerosing cholangitis (PSC) and nonalcoholic fatty liver disease (NAFLD) in CeD were assessed...
June 22, 2023: European Journal of Gastroenterology & Hepatology
https://read.qxmd.com/read/37325616/novel-hla-allele-associations-with-susceptibility-staging-symptomatic-state-autoimmune-hepatitis-and-hepatocellular-carcinoma-events-for-primary-biliary-cholangitis-in-the-japanese-population
#38
JOURNAL ARTICLE
Seik-Soon Khor, Kazuko Ueno, Nao Nishida, Minae Kawashima, Yosuke Kawai, Yoshihiro Aiba, Yuki Hitomi, Masao Nagasaki, Minoru Nakamura, Katsushi Tokunaga
Primary biliary cholangitis (PBC) is a rare autoimmune disease with a clear predisposition for human leukocyte antigen (HLA)-DR/DQ -associated loss of immune tolerance for the E2 component of the pyruvate dehydrogenase complex. Three-field-resolution HLA imputation of 1,670 Japanese PBC patients and 2,328 healthy controls was conducted using Japanese population-specific HLA reference panels. Eighteen previously reported Japanese PBC-associated HLA alleles were confirmed and extended to 3-field-resolution, including HLA-DRB1*08:03 to HLA-DRB1*08:03:02 , HLA-DQB1*03:01 to HLA-DQB1*03:01:01 , HLA-DQB1*04:01 to HLA-DQB1*04:01:01 and HLA-DQB1*06:04 to HLA-DQB1*06:04:01 ...
2023: Frontiers in Immunology
https://read.qxmd.com/read/37302951/prevalence-and-significance-of-antimitochondrial-antibodies-in-autoimmune-hepatitis-aih-results-from-a-large-multicentre-study-of-the-international-aih-group
#39
MULTICENTER STUDY
Nikolaos K Gatselis, Kalliopi Zachou, Aldo J Montano Loza, Eduardo Luiz Rachid Cançado, Teruko Arinaga-Hino, Paolo Muratori, Cumali Efe, Annarosa Floreani, Pietro Invernizzi, Athushi Takahashi, Akinobu Takaki, Benedetta Terziroli Beretta-Piccoli, Bart van Hoek, Ellina Lytvyak, Laura Vilar Guedes, Tugrul Purnak, Nora Cazzagon, Vasiliki Lygoura, Pinelopi Arvaniti, Eirini I Rigopoulou, Luigi Muratori, George N Dalekos
BACKGROUND & AIMS: Antimitochondrial antibodies (AMA) are specific markers for the diagnosis of primary biliary cholangitis (PBC) but can also be found occasionally in patients with autoimmune hepatitis (AIH). The present large multicentre cohort study assessed the prevalence and significance of AMA in AIH-patients. METHODS: 123 AMA-positive AIH-patients were investigated and compared with 711 age-matched AMA-negative AIH-patients and 69 patients with AIH/PBC variant...
October 2023: European Journal of Internal Medicine
https://read.qxmd.com/read/37300838/a-nationwide-study-of-autoimmune-liver-diseases-in-the-faroe-islands-incidence-prevalence-and-causes-of-death-2004-2021
#40
JOURNAL ARTICLE
Kári Rubek Nielsen, Jóngerð Midjord, Herborg Líggjasardóttir Johannesen, Henning Grønbæk
Background and aims: Autoimmune liver diseases are rare diseases, and population-based studies on the epidemiology of autoimmune hepatitis (AIH), primary biliary cholangitis (PBC), and primary sclerosing cholangitis (PSC) are sparse. We aimed to assess the incidence of AIH, PBC, and PSC in the Faroe Islands. Methods: All cases of AIH, PBC, and PSC diagnosed in the Faroe Islands between January 1st , 2004, and December 31st , 2021, were included in this nationwide registry-based cohort study. In addition, we searched all medical records to assess diagnostic criteria and cause of death...
December 2023: International Journal of Circumpolar Health
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