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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
#42
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
#43
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
#44
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
#45
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
#46
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
#47
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
#48
Xinhe Zhang, Xuyong Lin, Xuedan Li, Lin Guan, Yiling Li, Ningning Wang
Primary sclerosing cholangitis (PSC), autoimmune hepatitis (AIH), and ulcerative colitis (UC) are immune diseases of the digestive system. Some patients develop overlap syndrome, the presentation of two or more of the clinical, biochemical, immunological, and histological features of these conditions simultaneously or sequentially. The incidence of UC in PSC-AIH overlap syndrome is as high as 50%. In contrast, PSC-AIH overlap syndrome is rare in UC patients. However, because it has a low prevalence and has been studied in less detail, PSC is often misdiagnosed as primary biliary cholangitis (PBC) in its early stage...
2023: Frontiers in Immunology
#49
JOURNAL ARTICLE
Hui Liu, Yuqi Wang, Peng Wang, Xin Wang, Yunyun Yi, Xin Li
Autoimmune hepatitis (AIH) is often complicated with immune diseases, which greatly affected the course and clinical outcome of AIH. We aimed to systematically assess clinical characteristics, prognosis in autoimmune hepatitis accompanied by immune diseases. Clinical records of 358 patients with AIH from Beijing Ditan Hospital in China were retrospectively reviewed. The clinical features of AIH with immune diseases were compared retrospectively, including clinical characteristics, prognosis and outcome. Prevalence of immune diseases in patients with AIH was 26...
May 23, 2023: Clinical and Experimental Medicine
#50
JOURNAL ARTICLE
Yun Ye, Qian Zhang, Zhong-Hua Lu, You-Wen Tan
BACKGROUND: Only a few cases of chronic hepatitis B (CHB) with primary biliary cholangitis (PBC) have been reported based on histological evidence from liver biopsies. AIM: To observe the clinicopathological features and outcomes of 11 patients with CHB infection complicated by PBC. METHODS: Eleven patients with CHB and PBC who underwent liver biopsy at the Zhenjiang Third Hospital, affiliated with Jiangsu University, and Wuxi Fifth People's Hospital, from January 2005 to September 2020, were selected...
April 27, 2023: World Journal of Hepatology
#51
JOURNAL ARTICLE
E S Sbikina, E V Vinnitskaya, S N Batskikh, Y G Sandler, K G Saliev, T Y Khaimenova, D S Bordin
BACKGROUND: Despite the well-studied pathogenesis, the etiology of autoimmune liver disease (AILD) remains unknown. AIM: To determine the significance of hepatitis A, B, C and E viruses in the development and progression of AILD. MATERIALS AND METHODS: A single-center case-control study included 139 patients with AILD: autoimmune hepatitis - AIH ( n =46), primary biliary cholangitis - PBS ( n =74), primary sclerosing cholangitis - PSC ( n =19)...
March 30, 2023: Terapevticheskiĭ Arkhiv
#52
JOURNAL ARTICLE
Christoph Schultheiß, Silja Steinmann, Edith Willscher, Lisa Paschold, Ansgar W Lohse, Mascha Binder
BACKGROUND: Variant syndromes of autoimmune hepatitis (AIH) and primary biliary cholangitis (PBC) share diagnostic features of both entities, but their immunological underpinnings remain largely unexplored. METHODS: We performed blood profiling of 23 soluble immune markers and immunogenetics in a cohort of 88 patients with autoimmune liver diseases (29 typical AIH, 31 typical PBC and 28 with clinically PBC/AIH variant syndromes). The association with demographical, serological and clinical features was analyzed...
May 1, 2023: Hepatology Communications
#53
JOURNAL ARTICLE
Michelle Harriz Braga, Guilherme Grossi Lopes Cançado, Paulo Lisboa Bittencourt, Cláudia Alves Couto, Laura Vilar Guedes, André Mourão Costa Lima, Maria Lucia Gomes Ferraz, Cristiane Alves Villela-Nogueira, Mateus Jorge Nardelli, Luciana Costa Faria, Nathalia Mota de Faria Gomes, Elze Maria Gomes Oliveira, Vivian Rotman, Maria Beatriz Oliveira, Simone Muniz Carvalho Fernandes da Cunha, Marlone Cunha-Silva, Liliana Sampaio Costa Mendes, Claudia Alexandra Pontes Ivantes, Liana Codes, Valéria Ferreira de Almeida E Borges, Fabio Heleno de Lima Pace, Mario Guimarães Pessoa, Izabelle Venturini Signorelli, Gabriela Perdomo Coral, João Galizzi Filho, Aline Lopes Chagas, Debora Raquel Benedita Terrabuio, Eduardo Luiz Rachid Cançado
INTRODUCTION AND OBJECTIVES: Primary biliary cholangitis (PBC) and autoimmune hepatitis (AIH) and PBC overlap syndrome (AIH/PBC) have been associated with a higher risk of hepatocellular carcinoma (HCC) and extra-hepatic malignancy (EHM). This study aims to assess potential risk factors associated with cancer development in PBC and AIH/PBC. MATERIALS AND METHODS: The Brazilian Cholestasis Study Group database was reviewed to compare clinical and laboratory features of PBC patients with HCC and EHM with those without cancer...
2023: Annals of Hepatology
#54
JOURNAL ARTICLE
Hartl Johannes, Wehmeyer Malte, Alali Shaima, Sebode Marcial, Weidemann Sören, Ptashynska Anastasiya, Weiler-Normann Christina, Schramm Christoph, Lohse Ansgar Willhelm, Krech Till, Schrader Jörg
BACKGROUNDS/AIMS: Liver diseases may affect the liver heterogeneously, especially in autoimmune hepatitis (AIH). Hence, the aim of this study was to assess the added benefit of double biopsy of both liver lobes during minilaparoscopy in guiding treatment decisions in patients with AIH. METHODS: We identified all patients with AIH or variant syndromes (AIH/PBC and AIH/PSC) at our center, who underwent a double biopsy of both liver lobes via minilaparoscopy between 01/2016 and 12/2020...
April 17, 2023: Liver International: Official Journal of the International Association for the Study of the Liver
#55
Daniel A Guifarro, Diana De Oliveira-Gomes, Renato Beas, Marcel J Yibirin-Wakim, Eleazar E Montalvan-Sanchez
Overlap syndrome (OS) is a term that comprises the presentation of multiple hepatic disease characteristics in the same patient, such as the presence of autoimmune hepatitis (AIH) features in addition to primary sclerosing cholangitis (PSC) or primary biliary cholangitis (PBC). Standard therapy for AIH is immunosuppression, while ursodeoxycholic acid is the preferred treatment for PBC. Additionally, liver transplantation (LT) may be considered in severe cases. Hispanics have been found to have a higher prevalence of chronic liver disease and develop more complications associated with portal hypertension at the time of listing for LT...
March 2023: Curēus
#56
Marlone Cunha-Silva, Eloy Vianey Carvalho de França, Raquel Dias Greca, Daniel Ferraz de Campos Mazo, Larissa Bastos Eloy da Costa, Priscilla Brito Sena de Moraes, Clauber Teles Veiga, Guilherme Rossi Assis-Mendonça, Ilka de Fátima Santana Ferreira Boin, Raquel Silveira Bello Stucchi, Tiago Sevá-Pereira
COVID-19 is commonly associated with high serum levels of pro-inflammatory cytokines, and the post-infection status can disturb self-tolerance and trigger autoimmune responses. We are reporting a 45-year-old male who was admitted with fatigue, jaundice, elevated liver enzymes (with cholestatic pattern), and acute kidney injury two weeks after recovering from a mild SARS-CoV-2 infection. Serologies for viral hepatitis and anti-mitochondrial antibody were negative, while anti-nuclear and anti-smooth muscle antibodies were positive...
2023: Autopsy & Case Reports
#57
REVIEW
Costantino Sgamato, Alba Rocco, Debora Compare, Stefano Minieri, Stefano Andrea Marchitto, Simone Maurea, Gerardo Nardone
Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), causing coronavirus disease 2019 (COVID-19), can trigger autoimmunity in genetically predisposed individuals through hyperstimulation of immune response and molecular mimicry. Here we summarise the current knowledge about auto-immune liver diseases (AILDs) and SARS-CoV-2, focusing on: (1) The risk of SARS-CoV-2 infection and the course of COVID-19 in patients affected by AILDs; (2) the role of SARS-CoV-2 in inducing liver damage and triggering AILDs; and (3) the ability of vaccines against SARS-CoV-2 to induce autoimmune responses in the liver...
March 28, 2023: World Journal of Gastroenterology: WJG
#58
REVIEW
Jixuan Wang, Zhiwen Sun, Jingri Xie, Wanli Ji, Yang Cui, Zongxiong Ai, Guoying Liang
Autoimmune hepatitis (AIH), primary biliary cholangitis (PBC), primary sclerosing cholangitis (PSC), and IgG4-related sclerosing cholangitis (IgG4-SC) are the four main forms of autoimmune liver diseases (AILDs), which are all defined by an aberrant immune system attack on the liver. Most previous studies have shown that apoptosis and necrosis are the two major modes of hepatocyte death in AILDs. Recent studies have reported that inflammasome-mediated pyroptosis is critical for the inflammatory response and severity of liver injury in AILDs...
2023: Frontiers in Immunology
#59
REVIEW
Claire Kelly, Yoh Zen, Michael A Heneghan
Autoimmune liver diseases (AILDs) are a group of conditions where immune-mediated liver damage can lead to the need for transplantation. Collectively, they account for almost a quarter of all liver transplants. Outcomes in terms of graft and patient survival for all liver transplants have improved markedly over decades with improvements in patient selection, surgical techniques and longer-term care and this is also seen in patients with AILDs. The current five- and ten-year survival rates post-transplant in autoimmune disease are excellent, at 88% and 78%, respectively...
2023: Journal of Clinical and Experimental Hepatology
#60
JOURNAL ARTICLE
Sunil Taneja, Rohit Mehtani, Arka De, Suvradeep Mitra, Sahaj Rathi, Nipun Verma, Madhumita Premkumar, Ranjana Minz, Ajay Duseja, Ashim Das, Virendra Singh, Radha K Dhiman, Yogesh K Chawla
BACKGROUND AND AIMS: Autoimmune liver disease (AILD) comprises of autoimmune hepatitis (AIH), primary biliary cholangitis (PBC) and primary sclerosing cholangitis (PSC) with a spectrum of overlap amongst the three. We analyzed the spectrum and treatment outcomes of patients with AILD presenting to a tertiary care center in India. METHODS: A retrospective analysis of AILD patients from June 2008 to April 2021 was performed. The diagnosis was based on clinical, biochemical, imaging, serological, and histological characteristics...
2023: Journal of Clinical and Experimental Hepatology
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