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Lupus, cirrhosis

Yasuhiro Maeda, Shunya Nakane, Osamu Higuchi, Hideki Nakamura, Atsumasa Komori, Kiyoshi Migita, Akihiro Mukaino, Masataka Umeda, Kunihiro Ichinose, Mami Tamai, Shin-Ya Kawashiri, Waka Sakai, Hiroshi Yatsuhashi, Atsushi Kawakami, Hidenori Matsuo
OBJECTIVES: Autonomic dysfunction is closely associated with autoimmune diseases (AID) including primary biliary cirrhosis (PBC). The objective of this study was to determine the prevalence of anti-ganglionic (nicotinic) acetylcholine receptor (gAChR) antibodies in patients with AID. METHODS: We determined the presence of gAChR antibodies in serum samples from 146 patients (systemic lupus erythematosus [SLE] = 32; rheumatoid arthritis [RA] = 43; systemic sclerosis [SSc] = 38; PBC= 33) without information regarding autonomic symptoms, as well as 34 patients with other neurological diseases [OND] and 73 healthy controls [HC]...
August 19, 2016: Modern Rheumatology
Shirley R R Utiyama, Katiane B Zenatti, Heloisa A J Nóbrega, Juliana Z C Soares, Thelma L Skare, Caroline Matsubara, Dominique A Muzzilo, Renato M Nisihara
BACKGROUND: Autoimmune liver diseases (ALDs) are known to be associated with systemic autoimmune rheumatic diseases (SARDs) and their autoantibodies. We aimed to study the prevalence of SARDs and related autoantibodies, as well as their prognostic implications in a group of patients with ALDs. METHODS: This was a cross-sectional study. Sixty patients with ALDs (38.3% with autoimmune hepatitis; 11.7% with primary biliary cirrhosis; 25% with primary sclerosing cholangitis and 25% with overlap syndrome) were studied for the presence of SARDs and their autoantibodies...
August 2016: Immunological Investigations
Hai Long, Heng Yin, Ling Wang, M Eric Gershwin, Qianjin Lu
One of the major disappointments in human autoimmunity has been the relative failure on genome-wide association studies to provide "smoking genetic guns" that would explain the critical role of genetic susceptibility to loss of tolerance. It is well known that autoimmunity refers to the abnormal state that the dysregulated immune system attacks the healthy cells and tissues due to the loss of immunological tolerance to self-antigens. Its clinical outcomes are generally characterized by the presence of autoreactive immune cells and (or) the development of autoantibodies, leading to various types of autoimmune disorders...
July 5, 2016: Journal of Autoimmunity
Doo-Ho Lim, Yong-Gil Kim, Danbi Lee, Soo Min Ahn, Seokchan Hong, Chang-Keun Lee, Bin Yoo
OBJECTIVE: Autoimmune hepatitis (AIH) is a chronic progressive liver disease characterized by circulating autoantibodies and hyperglobulinemia. This study was conducted to identify the features of AIH accompanied by systemic lupus erythematosus (SLE-AIH) that differ from those of primary AIH (P-AIH), and to evaluate factors that affect the outcome for SLE-AIH patients. METHODS: From May 1995 to April 2014, clinical data (including liver pathology) from 164 patients with P-AIH and 23 patients with SLE-AIH were collected from an electronic database at a tertiary referral center...
July 2016: Arthritis Care & Research
Chun-Ting Chen, Yu-Chen Tseng, Chih-Wei Yang, Hsuan-Hwai Lin, Peng-Jen Chen, Tien-Yu Huang, Yu-Lueng Shih, Wei-Kuo Chang, Tsai-Yuan Hsieh, Heng-Cheng Chu
The incidence of Sjögren syndrome (SS) in primary biliary cirrhosis (PBC) patients is high. The influence of SS on the clinical outcomes of PBC patients, however, remains unclear. Our study retrospectively collected data on PBC-only patients and PBC patients with concomitant SS (PBC-SS) to compare the clinical differences of long-term outcomes between them.A total of 183 patients were diagnosed with PBC from January 1999 to December 2014 at our hospital. Of these, the authors excluded patients with diabetes, hypertension, advanced liver cirrhosis at initial diagnosis of PBC (Child-Turcotte-Pugh classification score of ≥7) and other liver diseases (ie, alcoholic liver disease, alpha-antitrypsin deficiency, viral hepatitis, and primary sclerosing cholangitis), and autoimmune diseases such as systemic lupus erythematosus and rheumatoid arthritis...
January 2016: Medicine (Baltimore)
Ke Liu, Biji T Kurien, Sarah L Zimmerman, Kenneth M Kaufman, Diana H Taft, Leah C Kottyan, Sara Lazaro, Carrie A Weaver, John A Ice, Adam J Adler, James Chodosh, Lida Radfar, Astrid Rasmussen, Donald U Stone, David M Lewis, Shibo Li, Kristi A Koelsch, Ann Igoe, Mitali Talsania, Jay Kumar, Jacen S Maier-Moore, Valerie M Harris, Rajaram Gopalakrishnan, Roland Jonsson, James A Lessard, Xianglan Lu, Jacques-Eric Gottenberg, Juan-Manuel Anaya, Deborah S Cunninghame-Graham, Andrew J W Huang, Michael T Brennan, Pamela Hughes, Gabor G Illei, Corinne Miceli-Richard, Edward C Keystone, Vivian P Bykerk, Gideon Hirschfield, Gang Xie, Wan-Fai Ng, Gunnel Nordmark, Per Eriksson, Roald Omdal, Nelson L Rhodus, Maureen Rischmueller, Michael Rohrer, Barbara M Segal, Timothy J Vyse, Marie Wahren-Herlenius, Torsten Witte, Bernardo Pons-Estel, Marta E Alarcón-Riquelme, Joel M Guthridge, Judith A James, Christopher J Lessard, Jennifer A Kelly, Susan D Thompson, Patrick M Gaffney, Courtney G Montgomery, Jeffrey C Edberg, Robert P Kimberly, Graciela S Alarcón, Carl L Langefeld, Gary S Gilkeson, Diane L Kamen, Betty P Tsao, W Joseph McCune, Jane E Salmon, Joan T Merrill, Michael H Weisman, Daniel J Wallace, Tammy O Utset, Erwin P Bottinger, Christopher I Amos, Katherine A Siminovitch, Xavier Mariette, Kathy L Sivils, John B Harley, R Hal Scofield
OBJECTIVE: More than 80% of autoimmune disease predominantly affects females, but the mechanism for this female bias is poorly understood. We suspected that an X chromosome dose effect accounts for this, and we undertook this study to test our hypothesis that trisomy X (47,XXX; occurring in ∼1 in 1,000 live female births) would be increased in patients with female-predominant diseases (systemic lupus erythematosus [SLE], primary Sjögren's syndrome [SS], primary biliary cirrhosis, and rheumatoid arthritis [RA]) compared to patients with diseases without female predominance (sarcoidosis) and compared to controls...
May 2016: Arthritis & Rheumatology
H J Reich, M Awad, A Ruzza, M A De Robertis, D Ramzy, N Nissen, S Colquhoun, F Esmailian, A Trento, J Kobashigawa, L S Czer
PURPOSE: Combined heart-liver transplantation is an increasingly accepted treatment for select patients with heart and liver disease. Despite growing optimism, heart-liver transplantation remains an infrequent operation. We report our institutional experience with heart-liver transplantation. METHODS: All combined heart-liver transplantations at Cedars-Sinai Medical Center from 1998-2014 were analyzed. Primary outcomes were patient and graft survival and secondary outcomes included rejection, infection, reoperation, length of stay, and readmission...
November 2015: Transplantation Proceedings
Dongdong Li, Siyuan Zhu, Tingting Wang, Jingna An, Lanlan Wang, Chuanmin Tao
OBJECTIVE: Early detection of hepatitis C virus (HCV) is an important step in preventing progression to cirrhosis and hepatocellular carcinoma. Serologic assays for anti-HCV antibody are valuable first-line tests in the screening and diagnosis of HCV infection. This study's aim was to evaluate the sensitivity and specificity of Elecsys Anti-HCV II assay for HCV screening. DESIGN AND METHODS: A total of 1,044 routine sera, 20 known HCV-positive samples, plus 54 preselected weakly positive samples were tested for anti-HCV with Elecsys Anti-HCV II assay, Elecsys Anti-HCV assays, InTec HCV enzymoimmunoassay (EIA), and Livzon Anti-HCV EIA...
September 2016: Journal of Clinical Laboratory Analysis
Chinmay Bera, Joy Sarojini Michael, Deepak Burad, Suzana B Shirly, Sridhar Gibikote, Banumathi Ramakrishna, Ashish Goel, C E Eapen
BACKGROUND: Xpert™ MTB/Rif is a multiplex hemi-nested real-time PCR-based assay to detect presence of M. tuberculosis within 2 hours of sample collection. The present study aimed at assessing efficacy of Xpert™ MTB/Rif assay for diagnosing peritoneal tuberculosis. METHODS: Patients with exudative ascites, fluid negative for acid-fast bacilli on auramine O fluorescence staining and unyielding fluid cytology for malignant cells, were included. Ultrasound-guided omental biopsy samples were obtained in all...
September 2015: Indian Journal of Gastroenterology: Official Journal of the Indian Society of Gastroenterology
Antony Payton, Piers Dawes, Hazel Platt, Cynthia C Morton, David R Moore, Jonathan Massey, Michael Horan, William Ollier, Kevin J Munro, Neil Pendleton
Cognitive abilities (memory, processing speed, vocabulary, and fluid intelligence) are correlated with educational attainment and occupational status, as well as physical and mental health. The variation in cognitive abilities observed within a population has a substantial genetic contribution (heritability ∼50%) and yet the identification of genetic polymorphisms from both genome-wide association and candidate studies have to date only uncovered a limited number of genetic variants that exert small genetic effects...
March 2016: American Journal of Medical Genetics. Part B, Neuropsychiatric Genetics
Sachin Devidas Dhole, Archana S Kher, Radha G Ghildiyal, Manjusha P Tambse
AIM: The main aim of the study is to study the clinical profile of disorders of the liver and hepatobiliary system in paediatric patients and to correlate the histopathology findings of liver biopsy in chronic liver disease. Another aim being to assess the prognosis and to know the outcome and the effects of treatment in chronic liver diseases in paediatric age group. MATERIALS AND METHODS: It was a prospective study, included the clinical profile of Chronic Liver Diseases (CLD) in children and the histopathological correlation...
July 2015: Journal of Clinical and Diagnostic Research: JCDR
Sigrid M C Broekaert, Almut Böer-Auer, Katrin Kerl, Ilka Herrgott, Xenia Schulz, Gisela Bonsmann, Randolf Brehler, Dieter Metze
BACKGROUND: Neutrophilic urticarial dermatosis (NUD) comprises a particular autoinflammatory condition within the spectrum of aseptic neutrophilic dermatoses characterized by a distinct urticarial eruption clinically and a neutrophilic dermatosis histopathologically. OBJECTIVE: In this study, we reviewed skin biopsies of lesional skin of patients seen in our outpatient clinic for autoimmune dermatoses and in allergy department from 1982 to 2014 that fulfilled these criteria...
January 2016: American Journal of Dermatopathology
Lara Bossini-Castillo, Elena López-Isac, Javier Martín
Systemic sclerosis (SSc) is a clinically heterogeneous connective tissue disorder of complex etiology. The development of large-scale genetic studies, such as genome-wide association studies (GWASs) or the Immunochip platform, has achieved remarkable progress in the knowledge of the genetic background of SSc. Herein, we provide an updated picture SSc genetic factors, offering an insight into their role in pathogenic mechanisms that characterize the disease. We review the most recent findings in the HLA region and the well-established non-HLA loci...
November 2015: Journal of Autoimmunity
Robert J Basseri, Michael T Schmidt, Benjamin Basseri
The hepatitis C virus (HCV) is the most common blood-borne pathogen and currently infects over two hundred and fifty million individuals worldwide. Chronic HCV infection may result in cirrhosis, hepatocellular carcinoma, and liver failure. An exceedingly rare extrahepatic manifestation of HCV is autoimmune hemolytic anemia (AIHA). We discuss an interesting case of direct Coombs'-positive AIHA in a treatment-naive 53-year-old male with a past medical history of HCV cirrhosis, genotype 3a, who presented with fatigue, abdominal pain, and jaundice...
October 2010: Clinical Journal of Gastroenterology
Hiromasa Ohira, Kazumichi Abe, Atsushi Takahashi
Liver dysfunction is often observed in patients with rheumatic diseases. Underlying disease is the major cause of liver dysfunction associated with adult-onset Still's disease, polymyositis/dermatomyositis, and vasculitis syndrome, whereas drug-induced liver injury is common in rheumatoid arthritis and systemic lupus erythematosus. These diseases may be accompanied by autoimmune hepatitis and primary biliary cirrhosis (PBC). PBC is particularly common in CREST (calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactylia, and telangiectasia) syndrome and Sjögren's syndrome...
February 2012: Clinical Journal of Gastroenterology
Toru Shizuma
Although autoimmune diseases often coexist, concomitant cases of systemic lupus erythematosus (SLE) and primary biliary cirrhosis (PBC) are uncommon. In this review paper, 34 cases of SLE with concomitant PBC found in English and Japanese scientific literature and Japanese proceedings were reviewed and summarized, including cases with liver dysfunction complicated by SLE. Of the 34 reported concomitant cases of SLE and PBC, 97.1% (33/34) were females, and PBC was diagnosed initially in 69.0% (20/29), except for five cases in which both SLE and PBC were simultaneously diagnosed...
2015: Journal of Immunology Research
Kishore Malyavantham, Bianca Weinstock-Guttman, Lakshmanan Suresh, Robert Zivadinov, Thomas Shanahan, Darlene Badgett, Murali Ramanathan
UNLABELLED: To compare frequencies of autoreactive antibody responses to endogenous disease-associated antigens in healthy controls (HC), relapsing and progressive MS and to assess their associations with clinical and MRI measures of MS disease progression. METHODS: The study analyzed 969 serum samples from 315 HC, 411 relapsing remitting MS (RR-MS), 128 secondary progressive MS (SP-MS), 33 primary progressive MS (PP-MS) and 82 patients with other neurological diseases for autoantibodies against two putative MS antigens CSF114(Glc) and KIR4...
2015: PloS One
Daniel Smyk, Eirini I Rigopoulou, Nicola Bizzaro, Dimitrios P Bogdanos
Environmental and genetic factors appear to be involved in the pathogenesis of primary biliary cirrhosis (PBC), a chronic cholestatic liver disease characterized by immune-mediated destruction of the small and medium sized intrahepatic bile ducts. Environmental factors include exposure to various infectious, xenobiotic and chemical compounds. These exposures may occur occupationally, through water or air contamination, pharmacological administration or by elective exposure, to name a few. Hair dyes are compounds that have been implicated in the development of several autoimmune diseases, including systemic lupus erythematosus (SLE) and PBC...
April 2013: Auto- Immunity Highlights
Daniele Saverino, Rita Simone, Marcello Bagnasco, Giampaola Pesce
CTLA-4, initially described as a membranebound molecule, is a costimulatory receptor transducing a potent inhibitory signal. Increasing evidence shows the CTLA-4 gene to be an important susceptibility locus for autoimmune endocrinopathies and other autoimmune disorders. A soluble form of cytotoxic T-lymphocyte-associated antigen-4 (sCTLA-4) has been established and shown to possess CD80/CD86 binding activity and in vitro immunoregulatory functions. sCTLA-4 is generated by alternatively spliced mRNA. Whereas low levels of sCTLA-4 are detected in normal human serum, increased serum levels are observed in several autoimmune diseases (e...
November 2010: Auto- Immunity Highlights
Cornelia Schuster, Kay D Gerold, Kilian Schober, Lilli Probst, Kevin Boerner, Mi-Jeong Kim, Anna Ruckdeschel, Thomas Serwold, Stephan Kissler
CLEC16A variation has been associated with multiple immune-mediated diseases, including type 1 diabetes, multiple sclerosis, systemic lupus erythematosus, celiac disease, Crohn's disease, Addison's disease, primary biliary cirrhosis, rheumatoid arthritis, juvenile idiopathic arthritis, and alopecia areata. Despite strong genetic evidence implicating CLEC16A in autoimmunity, this gene's broad association with disease remains unexplained. We generated Clec16a knock-down (KD) mice in the nonobese diabetic (NOD) model for type 1 diabetes and found that Clec16a silencing protected against autoimmunity...
May 19, 2015: Immunity
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