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AIH and PBC

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https://www.readbyqxmd.com/read/30120896/neuropsychiatric-symptoms-in-hepatitis-c-patients-resemble-those-of-patients-with-autoimmune-liver-disease-but-are-different-from-those-in-hepatitis-b-patients
#1
Meike Dirks, Kim Haag, Henning Pflugrad, Anita Blanka Tryc, Ramona Schuppner, Heiner Wedemeyer, Andrej Potthoff, Hans Ludger Tillmann, Kajetan Sandorski, Hans Worthmann, Xiaoqi Ding, Karin Weissenborn
Chronic fatigue, mood alterations and cognitive impairment are frequent accessory symptoms of HCV-infection. Fatigue and mood alterations have also been observed in autoimmune hepatitis (AIH) and primary biliary cholangitis (PBC), but not in hepatitis B virus (HBV)-infection, thus indicating an autoimmune response as possible cause of HCV-infection associated encephalopathy. Data, however, are sparse. This study aims to prove that HCV patients feature similar to those with autoimmune liver disease but contrary to HBV patients regarding neuropsychiatric symptoms...
August 18, 2018: Journal of Viral Hepatitis
https://www.readbyqxmd.com/read/30038926/overlap-syndrome-between-primary-biliary-cholangitis-and-primary-sclerosing-cholangitis
#2
Sridhar Sundaram, Kiran S, Srijan Mazumdar, Akash Shukla
Overlap syndrome indicates the coexistence of 2 or more autoimmune liver diseases in the same individual, occurring simultaneously or sequentially. Cases of overlap of autoimmune hepatitis (AIH) with primary biliary cholangitis (PBC) and of AIH with primary sclerosing cholangitis (PSC) are known and have defined criteria for diagnosis. Overlap between PBC and PSC has been reported in only a few case reports. The cause for the rarity of this entity compared to other overlap syndromes is unclear. We present a case of an overlap syndrome of PBC with PSC in a 35-year-old woman...
2018: ACG Case Reports Journal
https://www.readbyqxmd.com/read/30017212/autoimmune-hepatitis-primary-biliary-cholangitis-overlap-syndrome-long-term-outcomes-of-a-retrospective-cohort-in-a-university-hospital
#3
Omar Yesid Martínez Casas, Gabriel Sebastián Díaz Ramírez, Juan Ignacio Marín Zuluaga, Óscar Santos, Octavio Muñoz Maya, Jorge Hernando Donado Gómez, Juan Carlos Restrepo Gutiérrez
BACKGROUND: Autoimmune hepatitis (AIH) with characteristics of primary biliary cholangitis (PBC) is known as overlap syndrome. Its prevalence and prognosis have not yet been determined comparatively with AIH. METHODS: A retrospective cohort study was conducted comparing patients diagnosed with AIH and AIH-PBC overlap syndrome, followed-up for seven years in a university hospital in Colombia, until 31 December 2016. RESULTS: A total of 210 patients were included (195 women, mean age 48...
July 12, 2018: Gastroenterología y Hepatología
https://www.readbyqxmd.com/read/30005861/support-of-precision-medicine-through-risk-stratification-in-autoimmune-liver-diseases-histology-scoring-systems-and-non-invasive-markers
#4
REVIEW
Lars Bossen, Alessio Gerussi, Vasiliki Lygoura, George F Mells, Marco Carbone, Pietro Invernizzi
Autoimmune liver diseases (AILDs) are complex conditions, which arise from the interaction between a genetic susceptibility and unknown environmental triggers. They represent a relevant cause of liver failure and liver transplantation worldwide. As a testimony of our progress in understanding the biology of AILDs and the disease progression is the overall median survival which has increased over the last decade. However, there are still major challenges such as the lack of therapies and surveillance strategies in primary sclerosing cholangitis (PSC), the management and treatment of non-responders to first-line therapies in primary biliary cholangitis (PBC) and the need for tailoring immunosuppressive drugs in autoimmune hepatitis (AIH)...
July 11, 2018: Autoimmunity Reviews
https://www.readbyqxmd.com/read/29976101/the-role-of-the-chronic-care-model-in-promoting-the-management-of-the-patient-with-rare-liver-disease
#5
Marinos Pericleous, Claire Kelly, Aftab Ala, Simon De Lusignan
INTRODUCTION: The chronic care model (CCM) provides a holistic approach for managing chronic illnesses. Patients with rare liver diseases (RLD) have complex needs, impaired quality of life and often life-threatening complications. Most RLD meet the criteria for a long-term chronic condition and should be viewed through the prism of CCM. We aimed to ascertain whether the CCM has been considered for the frequently-encountered RLD. METHODS: MEDLINE®/PubMed®/Cochrane/EMBASE were searched to identify publications relating to the use of the CCM for the management of six RLD...
August 2018: Expert Review of Gastroenterology & Hepatology
https://www.readbyqxmd.com/read/29969462/dysbiosis-of-oral-microbiota-and-its-association-with-salivary-immunological-biomarkers-in-autoimmune-liver-disease
#6
Kazumichi Abe, Atsushi Takahashi, Masashi Fujita, Hiromichi Imaizumi, Manabu Hayashi, Ken Okai, Hiromasa Ohira
The gut microbiota has recently been recognized to play a role in the pathogenesis of autoimmune liver disease (AILD), mainly primary biliary cholangitis (PBC) and autoimmune hepatitis (AIH). This study aimed to analyze and compare the composition of the oral microbiota of 56 patients with AILD and 15 healthy controls (HCs) and to evaluate its association with salivary immunological biomarkers and gut microbiota. The subjects included 39 patients with PBC and 17 patients with AIH diagnosed at our hospital. The control population comprised 15 matched HCs...
2018: PloS One
https://www.readbyqxmd.com/read/29753567/junctional-adhesion-molecules-jam-b-and-jam-c-promote-autoimmune-mediated-liver-fibrosis-in-mice
#7
Edith Hintermann, Monika Bayer, Clara Benedetta Conti, Sina Fuchs, Michel Fausther, Patrick S Leung, Michel Aurrand-Lions, Richard Taubert, Josef M Pfeilschifter, Mireen Friedrich-Rust, Detlef Schuppan, Jonathan A Dranoff, M Eric Gershwin, Michael P Manns, Beat A Imhof, Urs Christen
Fibrosis remains a serious health concern in patients with chronic liver disease. We recently reported that chemically induced chronic murine liver injury triggers increased expression of junctional adhesion molecules (JAMs) JAM-B and JAM-C by endothelial cells and de novo synthesis of JAM-C by hepatic stellate cells (HSCs). Here, we demonstrate that biopsies of patients suffering from primary biliary cholangitis (PBC), primary sclerosing cholangitis (PSC) or autoimmune hepatitis (AIH) display elevated levels of JAM-C on portal fibroblasts (PFs), HSCs, endothelial cells and cholangiocytes, whereas smooth muscle cells expressed JAM-C constitutively...
July 2018: Journal of Autoimmunity
https://www.readbyqxmd.com/read/29663477/the-impact-of-autoimmune-hepatitis-and-its-treatment-on-health-utility
#8
Lin Lee Wong, Holly F Fisher, Deborah D Stocken, Stephen Rice, Amardeep Khanna, Michael A Heneghan, Ye Htun Oo, George Mells, Stuart Kendrick, Jessica Katharine Dyson, David E J Jones
Patient reporting suggests that the physical and psychological effects of autoimmune hepatitis (AIH) can be substantial. However, health-related quality of life (HRQOL) in patients with AIH remains incompletely characterized and health utility remains to be explored. Treatment for AIH often includes the use of corticosteroids, agents that can be associated with significant side effects. Here we explore the impact of AIH and its treatments on patient-reported HRQOL and health utility in a large cohort of prevalent cases from the United Kingdom Autoimmune Hepatitis (UK-AIH) national study...
April 17, 2018: Hepatology: Official Journal of the American Association for the Study of Liver Diseases
https://www.readbyqxmd.com/read/29554146/evaluation-of-classical-and-novel-autoantibodies-for-the-diagnosis-of-primary-biliary-cholangitis-autoimmune-hepatitis-overlap-syndrome-pbc-aih-os
#9
Henry H Nguyen, Abdel Aziz Shaheen, Natalia Baeza, Ellina Lytvyak, Stefan J Urbanski, Andrew L Mason, Gary L Norman, Marvin J Fritzler, Mark G Swain
BACKGROUND AND AIMS: Up to 20% of Primary Biliary Cholangitis (PBC) patients are estimated to have features that overlap with Autoimmune Hepatitis (AIH). Patients with PBC-AIH overlap syndrome (PBC-AIH OS) have been reported to exhibit suboptimal responses to ursodeoxycholic acid therapy, and are more likely to progress to cirrhosis. Anti-double stranded DNA (anti-dsDNA) and anti-p53 have been previously suggested to be potential autoantibodies for identifying patients with PBC-AIH OS...
2018: PloS One
https://www.readbyqxmd.com/read/29545925/interferon-free-treatment-for-patients-with-chronic-hepatitis-c-and-autoimmune-liver-disease-higher-svr-rates-with-special-precautions-for-deterioration-of-autoimmune-hepatitis
#10
Tatsuo Kanda, Shin Yasui, Masato Nakamura, Shingo Nakamoto, Koji Takahashi, Shuang Wu, Reina Sasaki, Yuki Haga, Sadahisa Ogasawara, Tomoko Saito, Kazufumi Kobayashi, Soichiro Kiyono, Yoshihiko Ooka, Eiichiro Suzuki, Tetsuhiro Chiba, Hitoshi Maruyama, Fumio Imazeki, Mitsuhiko Moriyama, Naoya Kato
Background: Interferon-free treatment can achieve higher sustained virological response (SVR) rates, even in patients in whom hepatitis C virus (HCV) could not be eradicated in the interferon treatment era. Immune restoration in the liver is occasionally associated with HCV infection. We examined the safety and effects of interferon-free regimens on HCV patients with autoimmune liver diseases. Results: All 7 HCV patients with autoimmune hepatitis (AIH) completed treatment and achieved SVR...
February 20, 2018: Oncotarget
https://www.readbyqxmd.com/read/29511554/suitability-of-the-simplified-autoimmune-hepatitis-score-for-the-diagnosis-of-autoimmune-hepatitis-in-a-german-cohort
#11
Hella Wobser, Tanja Paur, Elisabeth Schnoy, Janine Hartl, Gabriele I Kirchner
Background: Several years ago the International Autoimmune Hepatitis Group simplified the previous revised original scoring system for diagnosis of autoimmune hepatitis (AIH) into a scoring system based on only four instead of 13 parameters. Objective: We aimed to evaluate the suitability of the simplified AIH score for diagnosis of AIH in a German cohort with chronic liver diseases. Methods: In this retrospective single-center study, we compared the accuracy of both AIH scores in 70 patients with AIH and 211 patients with chronic liver diseases (PBC ( n  = 52), PSC ( n  = 27), NASH ( n  = 67), DILI ( n  = 15), CHB/C ( n  = 50))...
March 2018: United European Gastroenterology Journal
https://www.readbyqxmd.com/read/29507900/new-scoring-classification-for-primary-biliary-cholangitis-autoimmune-hepatitis-overlap-syndrome
#12
Wei Zhang, Debapriya De, Kahee A Mohammed, Satish Munigala, Guilan Chen, Jin-Ping Lai, Bruce R Bacon
Autoimmune hepatitis (AIH) and primary biliary cholangitis (PBC) are two major immune-mediated chronic liver diseases. Overlap syndrome (OS) is diagnosed if patients have features of both AIH and PBC; however, there is no consensus on the definition or diagnostic criteria for OS. Here, we report a new scoring classification for OS and evaluate its usefulness. This new scoring classification was developed by modifying the International Autoimmune Hepatitis Group classification by selecting histologic features of AIH and PBC along with modifications of biochemical and immunologic characteristics...
March 2018: Hepatology communications
https://www.readbyqxmd.com/read/29503645/autoantibodies-in-autoimmune-hepatitis-can-epitopes-tell-us-about-the-etiology-of-the-disease
#13
REVIEW
Urs Christen, Edith Hintermann
Autoimmune hepatitis (AIH), primary biliary cholangitis (PBC), and primary sclerosing cholangitis (PSC) are serious autoimmune liver diseases that are characterized by a progressive destruction of the liver parenchyma and/or the hepatic bile ducts and the development of chronic fibrosis. Left untreated autoimmune liver diseases are often life-threatening, and patients require a liver transplantation to survive. Thus, an early and reliable diagnosis is paramount for the initiation of a proper therapy with immunosuppressive and/or anticholelithic drugs...
2018: Frontiers in Immunology
https://www.readbyqxmd.com/read/29485711/trends-in-liver-transplantation-for-hepatitis-c-in-a-country-with-reduced-access-to-direct-acting-antiviral-agents
#14
Melisa Dirchwolf, Sebastián Marciano, Diego H Giunta, Maria L Posadas-Martínez, Scott W Biggins, Andrés E Ruf
BACKGROUND: Hepatitis C virus (HCV)-related cirrhosis is a leading indication for liver transplantation (LT) worldwide. Access to effective HCV treatment is inequitable globally. We aimed to analyze whether the introduction of effective HCV treatment caused an impact in LT trends in a middle-income country. METHODS: Cross-sectional analysis of all adult patients who were listed/received a LT in Argentina for HCV, alcohol-related liver disease (ALD), or autoimmune hepatitis/primary biliary cirrhosis (AIH/PBC) from 2007 to 2017...
April 2018: Clinical Transplantation
https://www.readbyqxmd.com/read/29470841/disease-duration-and-stage-influence-bone-microstructure-in-patients-with-primary-biliary-cholangitis
#15
Tobias Schmidt, Constantin Schmidt, Felix N Schmidt, Sebastian Butscheidt, Haider Mussawy, Jan Hubert, Thelonius Hawellek, Nicola Oehler, Florian Barvencik, Ansgar W Lohse, Thorsten Schinke, Christoph Schramm, Michael Amling, Tim Rolvien
Primary biliary cholangitis (PBC) is known to be a major risk factor for osteoporosis reflected by a reduction of bone mineral density (BMD). However, both the extent of the macro- and microstructural alterations of bone as well as the causative factors are unknown. We have retrospectively analyzed a total of 96 patients with PBC and 53 healthy controls matched for age, sex, and body mass index. In addition to dual-energy X-ray absorptiometry (DXA) measurements at the lumbar spine and hip, high-resolution peripheral quantitative computed tomography (HR-pQCT) was used to assess the geometric, volumetric, and microstructural changes of bone at the distal radius and tibia...
June 2018: Journal of Bone and Mineral Research: the Official Journal of the American Society for Bone and Mineral Research
https://www.readbyqxmd.com/read/29465536/primary-cutaneous-amyloidosis-associated-with-autoimmune-hepatitis-primary-biliary-cirrhosis-overlap-syndrome-and-sj%C3%A3-gren-syndrome-a-case-report
#16
Xin Yan, Jinglan Jin
RATIONALE: Primary cutaneous amyloidosis (PCA) is a localized skin disorder characterized by the abnormal deposition of amyloid in the extracellular matrix of the dermis. The association between PCA and other diseases, although rare, has been documented for various autoimmune diseases. PCA associated with autoimmune hepatitis-primary biliary cirrhosis (AIH-PBC) overlap syndrome and Sjögren syndrome (SS) has not been previously reported in the literature. PATIENT CONCERNS: A 50-year-old woman presented with progressive abnormal liver enzyme levels and was referred to our department...
February 2018: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29456412/performance-of-transient-elastography-in-assessing-liver-fibrosis-in-patients-with-autoimmune-hepatitis-primary-biliary-cholangitis-overlap-syndrome
#17
Hui-Min Wu, Li Sheng, Qi Wang, Han Bao, Qi Miao, Xiao Xiao, Can-Jie Guo, Hai Li, Xiong Ma, De-Kai Qiu, Jing Hua
AIM: To investigate the performance of transient elastography (TE) for diagnosis of fibrosis in patients with autoimmune hepatitis-primary biliary cholangitis (AIH-PBC) overlap syndrome. METHODS: A total of 70 patients with biopsy-proven AIH-PBC overlap syndrome were included. Spearman correlation test was used to analyze the correlation of liver stiffness measurement (LSM) and fibrosis stage. Independent samples Student's t -test or one-way analysis of variance was used to compare quantitative variables...
February 14, 2018: World Journal of Gastroenterology: WJG
https://www.readbyqxmd.com/read/29428100/clinical-analysis-of-liver-transplantation-in-autoimmune-liver-diseases
#18
Cheng-Peng Zhong, Zhi-Feng Xi, Qiang Xia
BACKGROUND: Autoimmune liver diseases (ALDs) consist of autoimmune hepatitis (AIH), primary biliary cirrhosis (PBC), primary sclerosing cholangitis (PSC), IgG4-associated cholangitis and overlap syndromes. Patients with these diseases may gradually progress to end-stage liver diseases and need liver transplantation. The present study aimed to explore the prognosis of patients with ALDs after liver transplantation. METHODS: The clinical data of 80 patients with ALD (24 cases of AIH, 35 of PBC, 15 of PSC and 6 of AIH-PBC overlap syndromes) who underwent liver transplantation in Renji Hospital, Shanghai Jiao Tong University School of Medicine from June 2004 to September 2016 were collected retrospectively...
February 2018: Hepatobiliary & Pancreatic Diseases International: HBPD INT
https://www.readbyqxmd.com/read/29380062/the-profiling-of-plasma-free-amino-acids-and-the-relationship-between-serum-albumin-and-plasma-branched-chain-amino-acids-in-chronic-liver-disease-a-single-center-retrospective-study
#19
Akitoshi Sano, Eiji Kakazu, Tatsuki Morosawa, Jun Inoue, Takayuki Kogure, Masashi Ninomiya, Tomoaki Iwata, Teruyuki Umetsu, Takuya Nakamura, Satoshi Takai, Tooru Shimosegawa
BACKGROUND: It is poorly understood how an imbalance of plasma-free amino acids (PFAAs) occurs and how the imbalance shows an association with the serum albumin (sAlb) level during the progression of chronic liver disease (CLDs). The aim of this study is to elucidate the profiles of PFAAs and the relationship between sAlb and PFAAs in recent patients with CLDs during the progression. METHODS: We retrospectively evaluated the 1569 data of PFAAs data obtained from 908 patients with various CLDs (CHC, CHB...
August 2018: Journal of Gastroenterology
https://www.readbyqxmd.com/read/29348111/leveraging-social-networking-sites-for-an-autoimmune-hepatitis-genetic-repository-pilot-study-to-evaluate-feasibility
#20
Megan Comerford, Rachel Fogel, James Robert Bailey, Prianka Chilukuri, Naga Chalasani, Craig Steven Lammert
BACKGROUND: Conventional approaches to participant recruitment are often inadequate in rare disease investigation. Social networking sites such as Facebook may provide a vehicle to circumvent common research limitations and pitfalls. We report our preliminary experience with Facebook-based methodology for participant recruitment and participation into an ongoing study of autoimmune hepatitis (AIH). OBJECTIVE: The goal of our research was to conduct a pilot study to assess whether a Facebook-based methodology is capable of recruiting geographically widespread participants into AIH patient-oriented research and obtaining quality phenotypic data...
January 18, 2018: Journal of Medical Internet Research
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