Autoimmune liver disease : management and clinical practice /: management and clinical practice. (2020)

• Record Type: Book
• Further Information: Note: Edited by James Neuberger, Gideon M. Hirschfield.
• Editors: Neuberger, James; Hirschfield, Gideon M
• Contents: Preface xv Contributors xvii Abbreviations xxi Introduction: The Paradigm and Paradox of Liver Autoimmunity 1; M. Eric Gershwin Section I Scientific Basis of Clinical Autoimmune Liver Diseases 3 1 Introduction to the Physiology, Immunology and Pathology of the Liver and Biliary Tree 5; Marco Carbone and Mario Strazzabosco Key Points 5 Liver Cell Types and Organization 6 Hepatic Metabolism 7 Bilirubin Metabolism and Transport 7 Carbohydrate Metabolism 7 Lipid Metabolism 8 Protein Metabolism 8 Metabolic Zonation 8 Hepatic Transport Systems 9 Basolateral (Sinusoidal) Transporters 9 Apical (Canalicular) Transporters 11 Drug Metabolism 11 Bile Formation, Secretion and the Enterohepatic Circulation 12 Bile Acid Synthesis and Metabolism 13 Enterohepatic Bile Acid Circulation 13 Death and Regeneration of Hepatocytes 14 Cell Death 14 Liver Regeneration 15 Cholangiocyte Reaction to Biliary Damage 16 Protective Role of Biliary HCO3 − Secretion 16 Cholangiocytes and Immunity 16 Biochemical Markers and Patterns of Hepatic Injury 17 Hepatocellular Necrosis 18 Cholestasis 18 References 20 2 Concepts of Autoimmunity Relevant to Autoimmune Liver Diseases 21; Isaiah G. Roepe and John M. Vierling Key Points 21 Introduction 22 Role of Innate and Adaptive Immunity in Autoimmunity in the Context of the Liver as an Immune Organ 22 Overview 22 Innate Immunity 23 Liver as an Innate Immune Organ 25 Adaptive Immunity and Adaptive Immune Functions of the Liver 25 Role of the Liver as an Adaptive Immune Organ 28 Generation and Maintenance of Tolerance to Self‐antigens 28 Overview 28 Central Tolerance 29 Central T‐cell Tolerance 29 Central B‐cell Tolerance 29 Peripheral Tolerance 30 T‐ and B‐cell Clonal Anergy 30 T‐cell Mediated Immune Regulation 30 Natural and Inducible T Regulatory Cells 30 Peripheral B‐cell Regulatory Mechanisms 31 Regulatory Dendritic Cells 31 Immunoregulatory Interplay Between Treg and Th17 Cells 31 Risk Factors for Autoimmune Diseases 31 Genetics 31 Complex Genetic and Monogenic Diseases 31 HLA Risk Alleles 32 Non‐HLA Gene Associations 32 Critical Role of Epigenetics 33 Transcription Factor Enhancers and Super Enhancers 33 MicroRNAs 33 Sex and Sex Hormones 33 Vitamin D and Sunlight Exposure 34 Loss of Immune Tolerance to Autoantigens and Perpetuation of Autoimmune Diseases 34 Overview 34 Role of the Microbiome 35 Mechanisms of Loss of Tolerance to Autoantigens 35 Bacterial and Viral Infections 35 Molecular Mimicry of Autoantigens 36 Neoantigens 36 Failure of Apoptosis to Conceal Autoantigens and Eliminate Autoreactive Cells 36 Immune Deviation of Activated T Cells 37 T‐cell Receptor Revision in the Periphery 37 Perpetuation of Autoimmune Diseases 37 Epigenetics 37 Epitope Spreading 38 Tissue Memory T Cells 38 Cytokines Promoting Chronic Inflammation and Autoimmunity 38 Tertiary Lymphoid Structures and Germinal Centers 39 Epithelial Cell‐induced Transformation of iTreg to Th17 Cells 39 Prevention of Autoimmunity and Therapeutic Control of Autoimmune Diseases 39 Overview 39 Strategies to Prevent Autoimmunity 39 Vitamin D3 39 Gut Microbiota Manipulation in Pregnancy and Infancy 39 Oral Tolerance 39 Strategies to Treat Established Autoimmune Diseases 40 Inducible T Regulatory T Cells 40 Epigenetic Enhancer Regulation 40 References 44 3 Genetics and Risk of Autoimmune Liver Diseases 47; George F. Mells Key Points 47 Introduction 48 HLA Associations in Autoimmune Liver Disease 50 Non‐HLA Associations in Autoimmune Liver Disease 53 Conclusion 61 References 62 4 Autoantibodies and Understanding of Autoimmune Liver Diseases 65; Benedetta Terziroli Beretta‐Piccoli, Giorgina Mieli‐Vergani, and Diego Vergani Key Points 65 Introduction 66 Methods of Detection 66 Anti‐nuclear Antibody 68 History 68 Immunofluorescence Reactivities and Antigenic Targets 68 Clinical Significance in Autoimmune Liver Disease 70 Anti‐smooth Muscle and Anti‐actin Antibodies 73 History 73 Immunofluorescence Reactivities, Antigenic Targets, and Clinical Significance 73 Anti‐liver‐kidney Microsomal Antibody 74 History 74 Immunofluorescence Reactivities, Antigenic Targets, and Clinical Significance 75 Anti‐liver Cytosol Type 1 Antibody 76 History 76 Immunofluorescence Reactivities, Antigenic Targets, and Clinical Significance 76 Anti‐soluble Liver Antigen Antibody 76 History 76 Immunofluorescence Reactivities, Antigenic Targets, and Clinical Significance 77 Anti‐neutrophil Cytoplasmic Antibody 77 History 77 Immunofluorescence Reactivities, Antigenic Targets, and their Clinical Significance 77 Anti‐mitochondrial Antibody 78 History 78 Immunofluorescence Reactivities, Antigenic Targets, and their Clinical Significance 78 Anti‐asialoglycoprotein Receptor Antibody 79 History 79 Immunofluorescence Reactivities, Antigenic Targets, and their Clinical Significance 79 Indications for Autoimmune Liver Serology Testing 79 Concluding Remarks 81 References 82 5 Environmental Exposure and Risk in Autoimmune Liver Diseases 83; Ying Qi Li and Andrew L. Mason Key Points 83 Introduction 84 Autoimmunity 84 Interaction of Genes and Environmental Triggers in Autoimmunity 85 Mechanisms for Triggering Autoimmunity 86 Primary Biliary Cholangitis 89 Geo‐epidemiology, Clusters, and Case–Control Studies of PBC 92 Bacterial Infection and PBC 93 Xenobiotics and PBC 96 Viruses in PBC 97 Autoimmune Hepatitis 99 Prospectus 101 References 102 Section II Autoimmune Liver Diseases and Their Clinical Correlation 103 6 Autoimmune Hepatitis 105; Aliya Gulamhusein and Patrick McKiernan Key Points 105 Introduction 106 Definition and Pathophysiology 106 Epidemiology 107 Presentation 108 Adult‐onset AIH 108 Pediatric‐onset AIH 109 Diagnosis 109 Autoimmune Sclerosing Cholangitis 112 Treatment 112 Therapeutic Approach in Adults 113 First-line Therapy 113 Withdrawal of Therapy 114 Second‐line Options 114 Therapeutic Approach in Children 115 First-line Therapy 115 Withdrawal of Therapy 115 Second‐line Options 115 Prognosis 116 Adulthood 116 Childhood 116 References 118 7 Primary Biliary Cholangitis 123; Alessio Gerussi and Marco Carbone Key Points 123 Introduction and Definition 124 Epidemiology 124 Etiopathogenesis 125 Clinical Presentation 126 Asymptomatic Patients
• Edition: 1st
• Publisher Details: Chichester : Wiley Blackwell
• Publication Date: 2020
• Extent: 1 online resource
• Subjects: 616.362079; Liver -- Diseases -- Immunological aspects; Autoimmune diseases
• Languages: English
• ISBNs: 9781119532620; 9781119532613
• Related ISBNs: 9781119532606
• Notes: Note: Includes bibliographical references and index.; Note: Description based on CIP data; resource not viewed.
• Access Rights: Legal Deposit; Only available on premises controlled by the deposit library and to one user at any one time; The Legal Deposit Libraries (Non-Print Works) Regulations (UK).
• Access Usage: Restricted: Printing from this resource is governed by The Legal Deposit Libraries (Non-Print Works) Regulations (UK) and UK copyright law currently in force.