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Primary biliary cirrhosis

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
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
Chen-Yi Liao, Chi-Hsiang Chung, Pauling Chu, Kuang-Yu Wei, Tseng-Min Feng, Fu-Huang Lin, Chang-Huei Tsao, Chia-Chao Wu, Wu-Chien Chien
BACKGROUND: We evaluated the risk of osteoporosis in patients with primary biliary cirrhosis (PBC) using a nationwide population-based dataset. METHODS: In a cohort study of 986,713 individuals, we selected 2,493 PBC patients who were aged 18 years or older and had been diagnosed with PBC, based on the International Classification of Disease (ICD-9-CM) codes 571.6, during 20002010. The control cohort comprised 9,972 randomly selected, propensity matched patients (by age, gender, and index date), without PBC...
2018: PloS One
Lars Bossen, Henriette Ytting, Peter Jepsen, Ole Hamberg, Peter Ott, Henning Grønbæk
The name of chronic liver disease: primary biliary cirrhosis, has been changed to: primary biliary cholangitis, primarily because of the stigma associated with the word "cirrhosis", as only a minority of the patients develop cirrhosis. In this review we present data on epidemiology and discuss the current treatments with focus on ursodeoxycholic acid and the newly described effects of the farnesoid receptor agonist obeticholic acid.
March 5, 2018: Ugeskrift for Laeger
Yue Yang, Long Chen, Yuan Jia, Yang Liu, Lei Wen, Yaoxian Liang, Yuan An, Shi Chen, Yin Su, Zhanguo Li
To analyze the clinical spectrum, laboratory characteristics, and outcomes of monoclonal gammopathy (MG) in patients with rheumatic diseases. Screening for the presence of MG was performed in 872 inpatients with rheumatic diseases from January 2010 to July 2017. A total of 41 patients were enrolled. Their clinical and biological features in addition to outcomes were described. For each patient with primary Sjögren syndrome (pSS), 2 age- and sex-matched pSS patients without MG were selected as controls. Risk factors for the presence of MG and malignant hematological neoplasias were assessed...
March 13, 2018: Clinical Rheumatology
Albert Parés
Primary cholangitis (cirrhosis) is a chronic cholestatic disease with an unquestionable female predominance. It is characterised by inflammation of the small and medium size bile ducts, and can eventually progress to cirrhosis. Most patients remain asymptomatic and are diagnosed by the casual finding of an anicteric biochemical cholestasis with increased alkaline phosphatase. The pathogenesis is unknown and of presumed autoimmune origin in genetic susceptible subjects. M2-type antimitochondrial antibodies, and specific antinuclear antibodies (gp210 and Sp100) are typical and specific of the disease...
March 8, 2018: Medicina Clínica
Peng Zhang, Qianjin Lu
Immunological tolerance loss is fundamental to the development of autoimmunity; however, the underlying mechanisms remain elusive. Immune tolerance consists of central and peripheral tolerance. Central tolerance, which occurs in the thymus for T cells and bone marrow for B cells, is the primary way that the immune system discriminates self from non-self. Peripheral tolerance, which occurs in tissues and lymph nodes after lymphocyte maturation, controls self-reactive immune cells and prevents over-reactive immune responses to various environment factors...
March 5, 2018: Cellular & Molecular Immunology
Kyohei Abe, Hiroaki Shiba, Kenei Furukawa, Taro Sakamoto, Yuichi Ishida, Katsuhiko Yanaga
Liver transplant recipients are considered to be at high risk for Clostridium difficile infection, with an incidence of 2.7-8.0%, which is three times higher than that among other patients. A case of a patient who suffered from pseudomembranous colitis five times after living donor liver transplantation is reported. A 60-year-old woman underwent splenectomy and living donor liver transplantation using the left lobe of her spouse for primary biliary cirrhosis. The patient made a satisfactory recovery, except for splenic vein thrombosis...
March 1, 2018: Clinical Journal of Gastroenterology
Jennifer Sammon, Sandra Fischer, Ravi Menezes, Hooman Hosseini-Nik, Sara Lewis, Bachir Taouli, Kartik Jhaveri
BACKGROUND: Combined hepatocellular-cholangiocarcinoma (cHCC-CC) is a rare primary liver tumor, which has overlapping imaging features with mass forming intra-hepatic cholangiocarcinoma (ICC) and hepatocellular carcinoma (HCC). Previous studies reported imaging features more closely resemble ICC and the aim of our study was to examine the differential MRI features of cHCC-CC and ICC with emphasis on enhancement pattern observations of gadolinium enhanced MRI. METHODS: Institutional review board approval with consent waiver was obtained for this retrospective bi-centric study...
February 27, 2018: Cancer Imaging: the Official Publication of the International Cancer Imaging Society
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...
February 27, 2018: Clinical Transplantation
Tapas K Tejura, Alejandro Pita, Christian Romero, Yuri S Genyk, Linda Sher, Suzanne L Palmer
PURPOSE: To identify long-term post-operative imaging findings resulting from right lobe liver donation. METHODS: This retrospective imaging review consisted of 42 adults (20 males, 22 females, mean age 36.6 years, range 18.7-55.9 years) who underwent right lobe liver donation with pre- and post-operative imaging between 1999 and 2006. The mean follow-up period was 30.2 months (range 12.3-69.6 months). Follow-up imaging evaluation included assessment of the biliary tree, particularly isolated bile ducts draining to and terminating at the cut surface (orphan ducts)...
February 22, 2018: Abdominal Radiology
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)
Diego Andrés Rodríguez Lugo, Jorge Julián Coronado Tovar, Giovana Alejandra Solano Villamarin, Wiliam Otero Regino
Primary biliary cholangitis (PBC) is a chronic autoimmune cholangiopathy characterized by a selective destruction of biliary epithelial cells of small and medium caliber hepatic ducts, which mainly affects women. The main symptoms are fatigue and pruritus, however, a large proportion of patients may be asymptomatic. The diagnosis is based on AMA titers >1:40, alkaline phosphatase >1.5 times the upper limit for more than 24 weeks and compatible liver histology. It is associated with multiple autoimmune diseases mainly extrahepatic, thyroid diseases, bone diseases, among others...
October 2017: Revista de Gastroenterología del Perú: órgano Oficial de la Sociedad de Gastroenterología del Perú
Palittiya Sintusek, Nawarat Posuwan, Piyaporn Wanawongsawad, Suttiruk Jitraruch, Yong Poovorawan, Voranush Chongsrisawat
AIM: To assess the seroprevalence of hepatitis B virus (HBV) immunity among previously vaccinated pediatric liver transplant recipients and present a case report of de novo hepatitis B infection after liver transplantation. METHODS: This study focused on children with chronic liver diseases who received primary hepatitis B immunization and had a complete dataset of anti-HBs before and after liver transplantation between May 2001 and June 2017. Medical records were retrospectively reviewed for potential factors relating to HBV immunity loss...
February 14, 2018: World Journal of Gastroenterology: WJG
Tengda Li, Yuanlan Huang, Peng Liu, Yun Liu, Jie Guo, Weiwei Zhang, Mingli Gu, Cheng Qian, Anmei Deng
Primary biliary cirrhosis (PBC) is an autoimmune liver disease. Its histological characteristics, such as progressive intrahepatic bile duct destruction, cholestasis, and liver cirrhosis, are caused by the body's autoimmune disorders. Interleukin (IL)-35 has two subunits (p35 and Ebi3) and is a member of the IL-12 family of heterodimeric cytokines. IL-35 has immunosuppressive functions and plays an important role in many autoimmune diseases. In this study, we compared plasma levels of IL-35 and relative mRNA expression levels of p35 and Ebi3 in peripheral blood mononuclear cells (PBMCs) from 70 PBC patients and 70 healthy individuals...
2018: Tohoku Journal of Experimental Medicine
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
Manabu Hayashi, Kazumichi Abe, Masashi Fujita, Ken Okai, Atsushi Takahashi, Yoshihiro Nozawa, Hiromasa Ohira
Non-invasive predictors for the development of cirrhosis-related conditions are needed for patients with primary biliary cholangitis (PBC). We investigated the association between cytokeratin-18 fragments (M30 and M65) and liver histology, treatment response and the development of cirrhosis-related conditions in patients with PBC. We retrospectively reviewed the clinical data of 111 individuals with biopsy-proven PBC. Serum M30 and M65 levels were measured using stored sera. M30 were significantly decreased after treatment, but there was no significant change in the M65 levels...
January 29, 2018: Medical Molecular Morphology
Paola Quarello, Francesco Tandoi, Francesca Carraro, Elena Vassallo, Michele Pinon, Renato Romagnoli, Ezio David, Dominic Dell Olio, Mauro Salizzoni, Franca Fagioli, Pier Luigi Calvo
BACKGROUND: Hematopoietic stem cell transplantation (HSCT) is curative in patients with primary immunodeficiencies. However, pre-HSCT conditioning entails unacceptably high risks if the liver is compromised. The presence of a recurrent opportunistic infection affecting the biliary tree and determining liver cirrhosis with portal hypertension posed particular decisional difficulties in a seven-year-old child with X-linked CD40-ligand deficiency. We aim at adding to the scanty experience available on such rare cases, as successful management with sequential liver transplantation (LT) and HSCT has been reported in detail only in one young adult to date...
January 26, 2018: Transplantation
Ryuta Ide, Akihiko Oshita, Takashi Nishisaka, Hideki Nakahara, Shiomi Aimitsu, Toshiyuki Itamoto
Primary biliary cholangitis (PBC) is a progressive cholestatic liver disease characterized by the presence of highly specific antimitochondrial antibodies, portal inflammation and lymphocyte-dominated destruction of the intrahepatic bile ducts, which leads to cirrhosis. While its pathogenesis remains unclear, PBC that shows histological progression to fibrosis carries a high risk of carcinogenesis; the same is true of viral liver diseases. In patients with PBC, the development of hepatocellular carcinoma (HCC) is rare; the development of combined hepatocellular carcinoma and cholangiocellular carcinoma (cHCC-CCC) is extraordinary...
December 28, 2017: World Journal of Hepatology
Samuel Torres-Landa, Armando Salim Muñoz-Abraham, Brett E Fortune, Ananta Gurung, Jeffrey Pollak, Sukru H Emre, Manuel I Rodriguez-Davalos, Michael L Schilsky
De-novo malignancies carry an incidence ranging between 3%-26% after transplant and account for the second highest cause of post-transplant mortality behind cardiovascular disease. While the majority of de-novo malignancies after transplant usually consist of skin cancers, there has been an increasing rate of solid tumor cancers over the last 15 years. Although, recurrence of hepatocellular carcinoma (HCC) is well understood among patients transplanted for HCC, there are increasing reports of de-novo HCC in those transplanted for a non-HCC indication...
December 28, 2017: World Journal of Hepatology
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