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A D Aravinthan, A C Doyle, A Issachar, M Dib, D Peretz, M S Cattral, A Ghanekar, I D McGilvray, M Selzner, P D Greig, D R Grant, N Selzner, L B Lilly, E L Renner
Liver transplantation (LT) is the treatment of choice for end-stage autoimmune liver diseases. However, the underlying disease may recur in the graft in some 20% of cases. The aim of this study is to determine whether LT using living donor grafts from first-degree relatives results in higher rates of recurrence than grafts from more distant/unrelated donors. 263 patients, who underwent a first LT in the Toronto liver transplant program between January 2000 and March 2015 for autoimmune liver diseases, and had at least 6 months of post-LT follow-up, were included in this study...
April 18, 2016: American Journal of Transplantation
Olivier Chazouillères
BACKGROUND: Some patients present with features of both primary biliary cirrhosis (PBC) or primary sclerosing cholangitis (PSC) on the one hand and autoimmune hepatitis (AIH) on the other hand, either simultaneously or consecutively. The term 'overlap syndrome (OS)' is used to describe these settings, but lack of universal agreement on what precisely constitutes an OS has generated considerable confusion. The low prevalence of OS (roughly 10% of PBC or PSC) has made it impracticable to perform randomized controlled trials...
2015: Digestive Diseases
Chalermrat Bunchorntavakul, K Rajender Reddy
Overlapping features between autoimmune hepatitis (AIH) and cholestatic disorders (primary biliary cirrhosis (PBC), primary sclerosing cholangitis (PSC), or indeterminate cholestasis), so-called overlap syndromes, usually have a progressive course toward cirrhosis and liver failure without adequate treatment. The diagnosis of overlap syndrome requires the prominent features of classic AIH and secondary objective findings of PBC or PSC. Empiric treatment for patients with AIH-PBC overlap is immunosuppressive therapy plus ursodeoxycholic acid...
February 2015: Clinics in Liver Disease
S Righi, E Fiorini, C De Molo, V Cipriano, F Cassani, L Muratori, M Lenzi, A M Morselli Labate, C Serra
OBJECTIVE: Acoustic radiation force impulse (ARFI) is a new software-based technique that evaluates liver stiffness during B-mode ultrasonography. The purpose of this study was to evaluate the accuracy of ARFI in distinguishing patients with chronic autoimmune liver disease from healthy subjects. MATERIAL AND METHODS: We enrolled 9 adult patients (8 women, 1 man; age 48.1 ± 12.8 years) with chronic autoimmune disease (primary biliary cirrhosis (PBC, n = 3), autoimmune hepatitis (AIH, n = 2), primary sclerosing cholangitis (PSC, n = 1) and overlap syndromes, (n = 3) who underwent a liver biopsy and 11 healthy volunteers (age 34...
December 2012: Journal of Ultrasound
Rahima A Bhanji, Andrew L Mason, Safwat Girgis, Aldo J Montano-Loza
BACKGROUND/AIMS: The term overlap syndrome describes variant forms of autoimmune hepatitis (AIH) that present in combination with either characteristics of primary biliary cirrhosis (PBC), or primary sclerosing cholangitis (PSC). This study analysed the outcomes and evidence of recurrent liver disease after liver transplantation in patients with overlap syndromes compared with patients transplanted for single autoimmune liver disease. METHODS: We evaluated 231 adult patients who received a liver transplant as a result of autoimmune liver diseases; including 103 with PBC, 84 with PSC, 32 with AIH and 12 with overlap syndrome (7 AIH-PBC and 5 AIH-PSC)...
February 2013: Liver International: Official Journal of the International Association for the Study of the Liver
P J Trivedi, G M Hirschfield
BACKGROUND: Autoimmune hepatitis (AIH), primary biliary cirrhosis (PBC) and primary sclerosing cholangitis (PSC) all nestle within the family of autoimmune liver diseases, whereby the result of immune-mediated liver injury gives rise to varied clinical presentations. Some patients demonstrate a phenotype whereby there is evidence of either PBC or PSC together with overlapping features of AIH. Due to an absence of well-validated diagnostic criteria and a lack of large therapeutic trials, treatment of overlap conditions is empiric and extrapolated from data derived from the primary autoimmune liver diseases...
September 2012: Alimentary Pharmacology & Therapeutics
Takashi Himoto, Hirohito Yoneyama, Kazutaka Kurokohchi, Michio Inukai, Hisashi Masugata, Fuminori Goda, Reiji Haba, Seishiro Watanabe, Shoichi Senda, Tsutomu Masaki
BACKGROUND: Autoantibodies to p53 (anti-p53) are rarely present in the sera of patients with autoimmune diseases or the sera of patients with malignancies. OBJECTIVE: To examine the prevalence of anti-p53 in patients with autoimmune liver disease including autoimmune hepatitis (AIH), primary biliary cirrhosis (PBC), AIH⁄PBC overlap syndrome (AIH⁄PBC OS) and primary sclerosing cholangitis (PSC), and to determine the clinical significance of anti-p53 in autoimmune liver diseases...
March 2012: Canadian Journal of Gastroenterology, Journal Canadien de Gastroenterologie
Oscar Mauricio Santos, Edison Muñoz Ortiz, Camilo Pérez, Juan Carlos Restrepo
Overlap syndromes are cases of liver diseases that share clinical, serological, histological and radiological criteria of autoimmune hepatitis (AIH), primary biliary cirrhosis (PBC) or primary sclerosing cholangitis (PSC). No definitions have been fully established and therefore there is no solid evidence on the diagnosis and treatment. This article presents the cases of three adult patients with overlapping features of AIH and PSC. Orthotopic liver transplantation was considered the best therapeutic alternative due to advanced disease progression in one patient, while medical treatment was provided in the remaining two patients...
April 2012: Gastroenterología y Hepatología
Hind I Fallatah, Hisham O Akbar
Autoimmune liver diseases (AILDs) are common leading causes for liver cirrhosis and terminal stage of liver disease. They have variable prevalence among patients with liver disease and have two major clinical and biochemical presentations. Autoimmune hepatitis (AIH) is the typical example of hepatocellular AILD, but it can also be presented under a cholestatic pattern. AIH has a scoring diagnostic system and respond in most cases to the treatment with prednisolone and azathioprine. Primary biliary cirrhosis (PBC) is the second most common AILD, with a cholestatic presentation and characterized by positive antimitochondrial antibody (AMA)...
2011: Comparative Hepatology
S Arulprakash, Anand D Sasi, Murali R Bala, T Pugazhendhi, S Jeevan Kumar
"Overlap syndrome" is used to describe variant forms of autoimmune hepatitis (AIH) which present with characteristics of AIH and primary biliary cirrhosis (PBC) or primary sclerosing cholangitis (PSC). Patients with overlap syndromes present with both hepatitic and cholestatic serum liver tests and have histological features of AIH and PBC or PSC. AIH-PBC is the most common form of overlap syndrome, affecting almost 10% of adults with AIH or PBC. Transitions from PBC to AIH-PBC overlap syndrome have also been reported...
July 2010: Journal of the Association of Physicians of India
Kirsten Muri Boberg, Roger W Chapman, Gideon M Hirschfield, Ansgar W Lohse, Michael P Manns, Erik Schrumpf
Some patients present with overlapping features between disorders within the spectrum of autoimmune liver diseases (i.e. autoimmune hepatitis (AIH), primary biliary cirrhosis (PBC), and primary sclerosing cholangitis (PSC)) and are commonly classified as having an "overlap syndrome". Standardized definitions of "overlap syndromes" are lacking. The aim of this report by the International Autoimmune Hepatitis Group (IAIHG) is to evaluate if there are important reasons to classify conditions with overlapping features between autoimmune liver diseases as separate diagnostic entities...
February 2011: Journal of Hepatology
Dekai Qiu, Qixia Wang, Hui Wang, Qing Xie, Guoqing Zang, Hong Jiang, Chuantao Tu, Jinsheng Guo, Shuncai Zhang, Jianshe Wang, Yi Lu, Ying Han, Lei Shen, Xiaoyu Chen, Xiqi Hu, Xiaojin Wang, Chengwei Chen, Qingchun Fu, Xiong Ma
BACKGROUND & AIMS: In 1999, the International Autoimmune Hepatitis Group (IAIHG) revised the diagnostic criteria for autoimmune hepatitis (AIH). It subsequently developed the simplified criteria in 2008 to enhance clinical applicability and practicability. In this study, we validated the simplified diagnostic criteria in Chinese patients with AIH or other chronic liver diseases in comparison with the revised original criteria. METHODS: Diagnostic scores were determined using the revised original criteria and the simplified criteria in 405 patients with diverse liver diseases...
February 2011: Journal of Hepatology
W Brian Hyslop, Andrea S Kierans, Polytimi Leonardou, Karen Fritchie, Jama Darling, Mohamed Elazazzi, Richard C Semelka
PURPOSE: To describe the MR findings of overlap syndromes of autoimmune chronic liver diseases. METHODS: Review of clinical and radiological databases between March 2001 and July 2008 for patients with a clinical diagnosis and liver biopsy features compatible with overlap syndrome who had also undergone an abdominal MRI yielded 15 adult patients. MR features of overlap syndrome were reviewed by two radiologists by consensus. Two radiologists independently reviewed the studies in a blinded fashion for primary sclerosing cholangitis (PSC)-type or non-PSC-type features to assess interobserver agreement...
February 2010: Journal of Magnetic Resonance Imaging: JMRI
Andreas Teufel, Arndt Weinmann, George J Kahaly, Catherine Centner, Anja Piendl, Marcus Wörns, Ansgar W Lohse, Peter R Galle, Stephan Kanzler
BACKGROUND: Although the pathomechanisms of autoimmune diseases in various organs remain unresolved, an accumulation of autoimmune diseases in individual patients has been observed. An overlap of autoimmune hepatitis (AIH) and primary biliary cirrhosis (PBC) or primary sclerosing cirrhosis has been well documented. However, the overlap of autoimmune diseases other than PBC or PSC has not yet been investigated in a large cohort. GOAL: The goal of our analysis was to investigate the incidence of concurrent autoimmune diseases in patients with AIH...
March 2010: Journal of Clinical Gastroenterology
Marina G Silveira, Keith D Lindor
Conditions exhibiting features of two different autoimmune liver diseases are commonly designated overlap syndromes, although there is no current agreement on what constitutes an overlap syndrome or specific diagnostic criteria. As in the classic autoimmune liver diseases, such as autoimmune hepatitis (AIH), primary biliary cirrhosis (PBC) and primary sclerosing cholangitis (PSC), the etiology is unknown but presumed to be related to alterations of immune regulation. Distinction of these clinical entities is important for management as outcomes may differ from outcomes of patients with diagnosis of classic autoimmune liver diseases...
December 2007: Expert Review of Gastroenterology & Hepatology
Christian Rust, Ulrich Beuers
The three major immune disorders of the liver are autoimmune hepatitis (AIH), primary biliary cirrhosis (PBC) and primary sclerosing cholangitis (PSC). Variant forms of these diseases are generally called overlap syndromes, although there has been no standardised definition. Patients with overlap syndromes present with both hepatitic and cholestatic serum liver tests and have histological features of AIH and PBC or PSC. The AIH-PBC overlap syndrome is the most common form, affecting almost 10% of adults with AIH or PBC...
June 7, 2008: World Journal of Gastroenterology: WJG
T Al-Chalabi, B C Portmann, W Bernal, I G McFarlane, M A Heneghan
BACKGROUND: Primary sclerosing cholangitis/autoimmune hepatitis (PSC/AIH) and primary biliary cirrhosis/AIH (PBC/AIH) overlap syndromes are poorly defined variants of AIH. Few large patient series exist, and there are little data on long-term outcomes. AIM: To compare presentation, clinical course and outcome of patients with PSC/AIH and PBC/AIH, with patients with definite AIH. Methods Two hundred and thirty-eight AIH patients were compared with 10 PBC/AIH patients and 16 PSC/AIH patients presenting consecutively between 1971 and 2005 at a single centre...
July 2008: Alimentary Pharmacology & Therapeutics
Ignacio Guerrero-Hernández, Aldo Montaño-Loza, Juan Fernando Gallegos-Orozco, Mariana Weimersheimer-Sandoval
BACKGROUND AND OBJECTIVE: The diagnosis of an autoimmune liver disease is based on clinical, biochemical, immunological and histological criteria particular to each disease, such as autoimmune hepatitis (AIH), primary biliary cirrhosis (PBC) or primary sclerosing cholangitis (PSC). Hepatic overlap syndromes (OS) are defined by the presence of a well established autoimmune liver disease, primarily AIH plus two or more characteristics associated with another liver disease (PBC or PSC); however the association of HAI and PSC is considered rare in adult population, and only 43 cases have been reported in the literature...
July 2007: Revista de Gastroenterología de México
Paolo Muratori, Alessandro Granito, Georgios Pappas, Luigi Muratori, Marco Lenzi, Francesco B Bianchi
Autoimmune liver disease (ALD) includes a spectrum of diseases which comprises both cholestatic and hepatitic forms: autoimmune hepatitis (AIH), primary biliary cirrhosis (PBC), primary sclerosing cholangitis (PSC) and the so called "overlap" syndromes where hepatitic and cholestatic damage coexists. All these diseases are characterized by an extremely high heterogeneity of presentation, varying from asymptomatic, acute (as in a subset of AIH) or chronic (with aspecific symptoms such as fatigue and myalgia in AIH or fatigue and pruritus in PBC and PSC)...
February 2008: Molecular Aspects of Medicine
H P Dienes
Autoimmune liver diseases encompass autoimmune hepatitis, primary biliary cirrhosis (PBC) and primary sclerosing cholangitis (PSC) as lesions of the biliary tract. The term autoimmune cholangitis has not been generally accepted, so it remains an entitiy waiting for precise definition. AIH is a chronic progressive necroinflammatory liver disease mostly occuring in female individuals and leading to ultimate autodestruction of the liver if not treated. Histopathology of the liver reflects the gerneral understanding of the underlying immune especially self reactive CD4 + T-helper cells mediated mechanisms in destruction of liver cells displaying a typical but by no means pathognomonic histopathological pattern...
2005: Verhandlungen der Deutschen Gesellschaft Für Pathologie
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