keyword
https://read.qxmd.com/read/38465864/the-prevalence-and-disease-course-of-autoimmune-liver-diseases-in-greenland
#1
JOURNAL ARTICLE
Rasmus Hvidbjerg Gantzel, Carina Nørskov Bagge, Gerda Elisabeth Villadsen, Karsten Fleischer Rex, Henning Grønbæk, Michael Lynge Pedersen
Autoimmune liver diseases are rare serious diseases causing chronic inflammation and fibrosis in the liver parenchyma and bile ducts. Yet, the prevalence and burden of autoimmune liver diseases are largely unexplored in Arctic native populations. We investigated the prevalence and management of autoimmune liver diseases in Greenland using nationwide cross-sectional register data and subsequent medical chart reviews validating diagnoses and extracting liver histology examinations and medical treatments. The overall prevalence of autoimmune liver diseases in Greenland was 24...
December 2024: International Journal of Circumpolar Health
https://read.qxmd.com/read/38464929/autoimmune-hepatitis-primary-biliary-cholangitis-overlap-syndrome-complicated-by-various-autoimmune-diseases-a-case-report
#2
Yu-Jie Qin, Ting Gao, Xing-Nian Zhou, Ming-Liang Cheng, Hong Li
BACKGROUND: Autoimmune hepatitis (AIH) and primary biliary cholangitis (PBC) are two common clinical autoimmune liver diseases, and some patients have both diseases; this feature is called AIH-PBC overlap syndrome. Autoimmune thyroid disease (AITD) is the most frequently overlapping extrahepatic autoimmune disease. Immunoglobulin (IgG) 4-related disease is an autoimmune disease recognized in recent years, characterized by elevated serum IgG4 levels and infiltration of IgG4-positive plasma cells in tissues...
February 26, 2024: World Journal of Clinical Cases
https://read.qxmd.com/read/38424613/multi-omics-approaches-for-drug-response-characterization-in-primary-biliary-cholangitis-and-autoimmune-hepatitis-variant-syndrome
#3
JOURNAL ARTICLE
Fan Yang, Leyu Zhou, Yi Shen, Xianglin Wang, Xiaoli Fan, Li Yang
BACKGROUND: Primary biliary cholangitis (PBC) and autoimmune hepatitis (AIH) variant syndrome (VS) exhibit a complex overlap of AIH features with PBC, leading to poorer prognoses than those with PBC or AIH alone. The biomarkers associated with drug response and potential molecular mechanisms in this syndrome have not been fully elucidated. METHODS: Whole-transcriptome sequencing was employed to discern differentially expressed (DE) RNAs within good responders (GR) and poor responders (PR) among patients with PBC/AIH VS...
February 29, 2024: Journal of Translational Medicine
https://read.qxmd.com/read/38390068/a-rare-coexistence-of-sarcoidosis-with-overlap-syndrome-and-sarcoidosis-with-primary-biliary-cholangitis-and-sjogren-s-syndrome-two-distinct-case-reports
#4
Marwa Bougacha, Hana Blibech, Bouchra Bouchabou, Raja Jouini, Imen Helal, Houda Snene, Donia Belkhir, Nadia Mehiri, Rim Ennaifer, Achraf Chedly, Nozha Ben Salah, Bechir Louzir
Sarcoidosis and the overlap syndrome of autoimmune hepatitis and primary biliary cholangitis (PBC) share common clinical, biological, and histological features. The simultaneous occurrence of these diseases have been reported in few cases and suggests that a common pathway which may contribute to granuloma formation in both conditions. We report the cases of two female patients having an association of sarcoidosis and inflammatory liver diseases. The first case is of a 61-year-old woman had been monitored for an overlap syndrome of PBC and autoimmune hepatitis (AIH)...
February 29, 2024: Heliyon
https://read.qxmd.com/read/38358198/prevalence-and-clinicopathological-spectrum-of-auto-immune-liver-diseases-overlap-syndrome
#5
JOURNAL ARTICLE
Annapoorani Varadarajan, Archana Rastogi, Rakhi Maiwall, Chhagan Bihari, Sherin Thomas, Vikrant Sood, Saggere Muralikrishna Shasthry
AIMS: Autoimmune liver diseases (AILD) represent a spectrum of related yet distinct immune-mediated disorders. The literature on the prevalence of these AILDs in Indian population is scarce. This study aims to assess the prevalence and clinicopathological spectrum of various AILDs especially the overlap syndrome. MATERIALS AND METHODS: A 10-year (2011-2020) cross-sectional, retrospective observational study of histological proven cases of AILD was conducted. Clinical, demographic, and laboratory parameters were retrieved...
2024: Indian Journal of Pathology & Microbiology
https://read.qxmd.com/read/38342195/immunopathogenesis-of-primary-biliary-cholangitis-primary-sclerosing-cholangitis-and-autoimmune-hepatitis-themes-and-concepts
#6
REVIEW
Palak J Trivedi, Gideon M Hirschfield, David H Adams, John M Vierling
Autoimmune liver diseases (AILDs) include primary biliary cholangitis (PBC), primary sclerosing cholangitis (PSC) and autoimmune hepatitis (AIH), a family of chronic immune-mediated disorders that target hepatocytes and cholangiocytes. Treatments remain non-specific, variably effective and non-curative, and the need for liver transplantation is disproportionate to their rarity. Development of effective therapies requires better knowledge of pathogenic mechanisms, including the roles of genetic risk, and how the environment and gut dysbiosis cause immune cell dysfunction and aberrant bile acid signalling...
February 9, 2024: Gastroenterology
https://read.qxmd.com/read/38337781/non-invasive-serum-biomarkers-for-the-diagnosis-of-cirrhosis-in-patients-with-autoimmune-hepatitis-aih-and-aih-primary-biliary-cholangitis-overlap-syndrome-aih-pbc-red-cell-distribution-width-to-platelet-ratio-rpr-yielded-the-most-promising-result
#7
JOURNAL ARTICLE
Siwanon Nawalerspanya, Jarukit Tantipisit, Suraphon Assawasuwannakit, Apichat Kaewdech, Naichaya Chamroonkul, Pimsiri Sripongpun
Several serum biomarkers for fibrosis assessment have been proposed in various liver diseases, but in autoimmune hepatitis (AIH) or overlap with primary biliary cholangitis (PBC; AIH-PBC) patients, the data are scarce. This retrospective cross-sectional study was conducted to validate six non-invasive biomarkers in the diagnosis of cirrhosis (F4 fibrosis) in such patients. We included adult patients diagnosed with AIH or AIH-PBC overlap syndrome who underwent a liver biopsy between 2011 and 2021. Laboratory data were collected to calculate the following scores: red cell distribution width to platelet ratio (RPR), aspartate aminotransferase/platelet ratio index (APRI), Fibrosis-4 index (FIB-4), aspartate aminotransferase (AST) to alanine aminotransferase (ALT) ratio (AAR), neutrophil-to-lymphocyte ratio (NLR), and lymphocyte-to-platelet ratio (LPR)...
January 25, 2024: Diagnostics
https://read.qxmd.com/read/38327519/corrigendum-pd-1-pd-l1-immune-checkpoint-therapy-demonstrates-favorable-safety-profile-in-patients-with-autoimmune-and-cholestatic-liver-disease
#8
Lorenz Kocheise, Ignazio Piseddu, Joscha Vonderlin, Eric T Tjwa, Gustav Buescher, Lucy Meunier, Pia Goeggelmann, Francesca Fianchi, Jérôme Dumortier, Mar Riveiro Barciela, Tom J G Gevers, Benedetta Terziroli Beretta-Piccoli, Maria-Carlota Londoño, Sona Frankova, Thomas Roesner, Vincent Joerg, Constantin Schmidt, Fabian Glaser, Jan P Sutter, Thorben W Fründt, Ansgar W Lohse, Samuel Huber, Johann von Felden, Marcial Sebode, Kornelius Schulze
[This corrects the article DOI: 10.3389/fimmu.2023.1326078.].
2024: Frontiers in Immunology
https://read.qxmd.com/read/38313999/chronic-liver-disease-not-everything-is-what-it-seems-autoimmune-hepatitis-primary-biliary-cholangitis-overlap-syndrome
#9
Bruna Rodrigues Barbosa, Laurinda Pereira, Fátima Campante, Ana Paula Pona
Overlap syndrome (OS) is a rare condition that shares characteristics of at least two other recognized diseases, whose early diagnosis impacts treatment decisions and prognosis since the unfavorable course of the OS seems to be worse than that of the diseases alone. OS in autoimmune liver diseases combines characteristic features of autoimmune hepatitis (AIH), primary biliary cholangitis (PBC), and primary sclerosing cholangitis. AIH is a chronic, inflammatory disease of the liver that occurs predominantly in females...
January 2024: Curēus
https://read.qxmd.com/read/38305739/nmr-based-metabolomic-signature-an-important-tool-for-the-diagnosis-and-to-study-pathogenesis-of-autoimmune-hepatitis
#10
JOURNAL ARTICLE
Aikaterini Dimou, Kalliopi Zachou, Christina Kostara, Kalliopi Azariadis, George Giannoulis, Aggeliki Lyberopoulou, Eleni Bairaktari, George N Dalekos
BACKGROUND AIMS: Metabolomics are used to predict, diagnose, and monitor metabolic disorders but altered metabolomic signature has also been reported in diverse diseases, including autoimmune disorders. However, the metabolomic profile in autoimmune hepatitis (AIH), has not been investigated in depth. Therefore, we investigated the metabolomic signature of AIH and its significance as a diagnostic and pathogenetic tool. APPROACH RESULTS: Metabolites in plasma samples from 50 AIH patients at diagnosis, 43 healthy controls (HC), 72 patients with primary biliary cholangitis (PBC), 26 patients with metabolic dysfunction-associated liver disease (MASLD) and 101 patients with chronic viral hepatitis (CVH) were determined by 1H-NMR spectroscopy...
February 5, 2024: Hepatology: Official Journal of the American Association for the Study of Liver Diseases
https://read.qxmd.com/read/38270327/clinical-performance-of-ama-m2-anti-gp210-and-anti-sp100-antibody-levels-in-primary-biliary-cholangitis-when-detected-by-multiplex-bead-based-flow-fluorescent-immunoassay
#11
JOURNAL ARTICLE
Zhan Wang, Yongxin Li, Lisheng Ren, Yujie Li, Tiantian Xu, Wenshuai Li, Weize Gao, Guirong Sun, Mingjun Liu
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...
January 2024: Immunity, Inflammation and Disease
https://read.qxmd.com/read/38250015/add-on-immunosuppressive-therapy-may-benefit-selected-patients-with-primary-biliary-cholangitis-and-autoimmune-phenomena
#12
JOURNAL ARTICLE
Mengqi Li, Sha Chen, Shuxiang Li, Tingting Lv, Buer Li, Shan Shan, Min Li, Na Zeng, Qianyi Wang, Yuanyuan Kong, Hong Ma, Xinyan Zhao, Xiaojuan Ou, Hong You, Weijia Duan, Jidong Jia
BACKGROUND: Mildly elevated levels of transaminase and/or immunoglobulin G (IgG) are common in patients with primary biliary cholangitis (PBC). It is still unclear whether adding immunosuppressive therapy to ursodeoxycholic acid (UDCA) benefits those patients who are not fulfilling the diagnostic criteria of PBC with autoimmune hepatitis (AIH) features. OBJECTIVES: To assess the efficacy of adding immunosuppressive therapy to UDCA for patients with PBC and autoimmune phenomena but not fulfilling the diagnostic criteria of PBC with AIH features...
2024: Therapeutic Advances in Gastroenterology
https://read.qxmd.com/read/38178070/evaluation-of-autoimmune-liver-disease-natural-history-in-patients-referred-to-middle-east-liver-diseases-meld-center
#13
JOURNAL ARTICLE
Seyed Erfan Mehdi Nejad, Mohammad Heiat, Mohammad Javanbakht, Seyed Moayed Alavian, Mohammad Ali Abyazi Haris
BACKGROUND: Autoimmune liver diseases (AILD) are increasing and common forms of chronic liver disease (CLD) with different clinical responses and characteristics which can result in cirrhosis. This study aimed to investigate the natural history and characteristics of AILD in an Iranian population. METHODS: Patients with AILD [Autoimmune Hepatitis (AIH), Primary Biliary Cholangitis (PBC), Primary Sclerosing Cholangitis (PSC) and Overlap Syndrome (OS)] referred to Middle East Liver Diseases (MELD) center, Tehran, Iran, between January 2002 and December 2022 were included in this retrospective cohort study...
January 4, 2024: BMC Gastroenterology
https://read.qxmd.com/read/38152650/primary-biliary-cholangitis-with-features-of-autoimmune-hepatitis-in-a-19-year-old-adolescent-with-14q24-1q24-2-deletion-a-case-report
#14
Yi-Wei Wang, Hsun-Chin Chao, Huei-Shyong Wang, Ju-Li Lin, Chih-Chen Chang, Shiu-Feng Huang
INTRODUCTION: Primary biliary cholangitis (PBC) is a rare and chronic autoimmune liver disease characterized by the progressive destruction of small intrahepatic bile ducts that may eventually lead to cirrhosis. PBC with features of autoimmune hepatitis (AIH) has rarely been reported in pediatric patients with genetic defects. We present the case of an adolescent with chromosome 14q24.1q24.2 deletion who was given the diagnosis of stage IV PBC with features of AIH. CASE PRESENTATION: A 19-year-old male adolescent with multiple congenital abnormalities and an intellectual disability presented with abnormal liver enzymes levels and pruritus for more than 5 years...
2023: Frontiers in Pediatrics
https://read.qxmd.com/read/38151060/influence-of-socioeconomic-factors-on-liver-transplant-survival-outcomes-in-patients-with-autoimmune-liver-disease-in-the-united-states
#15
JOURNAL ARTICLE
Leandro Sierra, Ana Marenco-Flores, Romelia Barba, Daniela Goyes, Bryan Ferrigno, Wilfor Diaz, Esli Medina-Morales, Behnam Saberi, Vilas R Patwardhan, Alan Bonder
INTRODUCTION AND OBJECTIVES: Autoimmune liver diseases (AILDs): autoimmune hepatitis (AIH), primary biliary cholangitis (PBC), and primary sclerosing cholangitis (PSC) have different survival outcomes after liver transplant (LT). Outcomes are influenced by factors including disease burden, medical comorbidities, and socioeconomic variables. MATERIALS AND METHODS: Using the United Network for Organ Sharing database (UNOS), we identified 13,702 patients with AILDs listed for LT between 2002 and 2021...
December 25, 2023: Annals of Hepatology
https://read.qxmd.com/read/38127091/perceived-stigma-in-patients-with-autoimmune-hepatitis
#16
JOURNAL ARTICLE
Jessica P Naftaly, Estée C H Feldman, Rachel N Greenley
Perceived stigma (PS) adversely impacts psychosocial and disease outcomes in patients with chronic liver diseases (CLD), and those with autoimmune hepatitis (AIH) may be at risk for PS given inaccurate assumptions about the origin of their diagnosis. The aims of the current study are to describe the frequency of PS in patients with AIH, compare rates of PS in AIH to rates of PS in primary biliary cholangitis (PBC) and CLD, and examine demographic correlates of PS. 262 adults with AIH (95% female, Mage  = 51...
December 21, 2023: Journal of Clinical Psychology in Medical Settings
https://read.qxmd.com/read/38103932/prognostic-models-and-autoimmune-liver-diseases
#17
REVIEW
Daphne D'Amato, Marco Carbone
Autoimmune liver diseases (AILDs) are complex diseases with unknown causes and immune-mediated pathophysiology. In primary biliary cholangitis (PBC) and autoimmune hepatitis (AIH) disease modifying drugs are available which improve patient quality and quantity of life. In primary sclerosing cholangitis (PSC) no medical therapy is available and the only accepted treatment is liver transplantation (LT). PBC, PSC and AIH possess features that describe the archetype of patients within each disorder. On the other hand, the classical disorders are not homogeneous, and patients within each diagnosis may present with a range of clinical, biochemical, serological, and histological findings...
December 2023: Best Practice & Research. Clinical Gastroenterology
https://read.qxmd.com/read/38025334/the-contribution-of-antinuclear-antibodies-in-primary-biliary-cholangitis-pbc-an-experience-from-the-immunology-laboratory-at-university-hospital-center-hassan-ii-fes-morocco
#18
JOURNAL ARTICLE
I El Mitri, H Kaaouch, M Ouboks, O Ballil
UNLABELLED: Primary biliary cholangitis (PBC) is a chronic autoimmune cholestatic liver disease. If this disease is not treated, it might lead to fibrosis, cirrhosis, and its associated complications.1 Early recognition of this entity and immediate institution of therapy are the primary prognostic modifiers.2 Anti-nuclear autoantibodies can be used for diagnosis, particularly the anti-gp 210 and anti-Sp100 antibodies. Our aim in this study is to describe the epidemiology, clinical, biochemical, and immunological aspects of patients with PBC, thus, emphasizing the role of those antibodies...
2023: Qatar Medical Journal
https://read.qxmd.com/read/38022336/detection-of-lymphadenopathy-as-a-precursor-to-autoimmune-liver-diseases-before-clinical-hepatitis-became-apparent-a-report-of-two-cases
#19
Hiroshi Okano, Hiroki Tanaka, Shimpei Matsusaki, Katsumi Mukai, Akira Nishimura, Kana Asakawa, Youichirou Baba, Tetsuya Murata
Two patients were incidentally diagnosed with intra-abdominal lymphadenopathy on imaging examinations. Although endoscopic ultrasound-guided fine needle aspiration of these areas of lymphadenopathy was performed, their causes remained undetermined. Neither patients had abnormal hepatic enzyme levels at the time lymphadenopathy was detected, but they developed hepatitis 20 months and five months later, respectively. The laboratory data and/or histopathological findings suggested primary biliary cholangitis/cirrhosis (PBC) and autoimmune hepatitis (AIH), respectively...
October 2023: Curēus
https://read.qxmd.com/read/38020818/autoimmune-liver-disease-in-human-immunodeficiency-virus-infected-patients-3-case-series
#20
Maria Ana Rafael, Filipa Bordalo Ferreira, Rita Theias Manso, Francesca Peruzzu, Mariana Cardoso
We present 3 cases of autoimmune liver disease in human immunodeficiency virus (HIV)-infected patients and describe the different diagnostic and therapeutic approaches used in each case. The first patient was diagnosed with primary biliary cholangitis (PBC) with features of autoimmune hepatitis (AIH), requiring second-line therapy due to incomplete response to ursodeoxycholic acid. The second patient was diagnosed with AIH with features of PBC and had the particular challenges of presenting with advanced liver fibrosis and having a past history of disseminated cytomegalovirus infection...
November 2023: GE Portuguese Journal of Gastroenterology
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