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Advances in Cholestatic Liver Diseases, An issue of Clinics in Liver Disease, E-Book

Advances in Cholestatic Liver Diseases, An issue of Clinics in Liver Disease, E-Book

Cynthia Levy

(2016)

Additional Information

Book Details

Abstract

Dr. Levy has secured the field’s top experts to offer the latest clinical reviews on cholestatic liver disease. The issue will include articles devoted to The Gut-Liver Axis; Update on Genetics in Primary Biliary Cirrhosis and Primary Sclerosing Cholangitis; New thoughts on IgG4 Associated Cholangitis; Cancer Risk and Surveillance in PSC; Novel Therapies for PBC; and Liver transplantation for Cholestatic Liver Diseases, to name a few. Dr. Levy’s issue will be the most current authority on cholestatic liver disease.

Table of Contents

Section Title Page Action Price
Front Cover Cover
Advances in Cholestatic Liver \rDiseases i
Copyright\r ii
Contributors iii
CONSULTING EDITOR iii
EDITOR iii
AUTHORS iii
Contents vii
Preface: Advances in Cholestatic Liver Diseases\r vii
The Gut-Liver Axis in Primary Sclerosing Cholangitis\r vii
The Immunogenetics of Autoimmune Cholestasis\r vii
Making Sense of Autoantibodies in Cholestatic Liver Diseases\r vii
New Thoughts on Immunoglobulin G4–Related Sclerosing Cholangitis\r viii
Primary Sclerosing Cholangitis: Multiple Phenotypes, Multiple Approaches\r viii
Cancer Risk and Surveillance in Primary Sclerosing Cholangitis\r viii
Sclerosing Cholangitis in Children and Adolescents\r viii
Novel Therapies on Primary Biliary Cirrhosis\r ix
Understanding and Treating Fatigue in Primary Biliary Cirrhosis and Primary Sclerosing Cholangitis\r ix
Utility of Noninvasive Markers of Fibrosis in Cholestatic Liver Diseases\r ix
Total Parenteral Nutrition–Induced Cholestasis: Prevention and Management\r ix
New Insights on Intrahepatic Cholestasis of Pregnancy\r x
Liver Transplantation for Cholestatic Liver Diseases in Adults\r x
CLINICS IN LIVER DISEASE\r xi
FORTHCOMING ISSUES xi
RECENT ISSUES xi
Preface: Advances in Cholestatic Liver Diseases \r xiii
The Gut-Liver Axis in Primary Sclerosing Cholangitis 1
Key points 1
INTRODUCTION 1
EPIDEMIOLOGY OF PRIMARY SCLEROSING CHOLANGITIS AND INFLAMMATORY BOWEL DISEASE 2
CLINICAL FEATURES OF PRIMARY SCLEROSING CHOLANGITIS AND INFLAMMATORY BOWEL DISEASE 3
PRIMARY SCLEROSING CHOLANGITIS AND INFLAMMATORY BOWEL DISEASE AFTER LIVER TRANSPLANTATION 5
PATHOGENESIS 6
Shared Primary Sclerosing Cholangitis and Inflammatory Bowel Disease Genetic Susceptibility 6
Interleukin-2/Interleukin-2 Receptor α Pathway 6
Macrophage-Stimulating–1 Pathway 7
Fucosyltransferase 2 Pathway 7
Salt-Inducible Kinase-2 Pathway 7
Entero-Hepatic Immune Dysregulation in Primary Sclerosing Cholangitis and Inflammatory Bowel Disease 8
THERAPEUTIC TARGETING OF THE GUT-LIVER AXIS IN PRIMARY SCLEROSING CHOLANGITIS AND INFLAMMATORY BOWEL DISEASE 9
Altering the Microbiome 9
Immunosuppression and Anti-Tumor Necrosis Factor α Therapy 10
Targeted Entero-Hepatic Therapies for Primary Sclerosing Cholangitis and Inflammatory Bowel Disease 10
SUMMARY 11
REFERENCES 11
The Immunogenetics of Autoimmune Cholestasis 15
Key points 15
INTRODUCTION 15
EPIDEMIOLOGIC CONSIDERATIONS: HERITABILITY AND FAMILIAL CLUSTERING 16
HUMAN LEUKOCYTE ANTIGEN ASSOCIATIONS 17
T-CELL SIGNALING 18
TUMOR NECROSIS FACTOR α SIGNALLING 19
MUCOSAL IMMUNE ACTIVATION IN LIVER AUTOIMMUNITY 20
IMMUNO-EPIGENETIC INFLUENCES 22
THERAPEUTIC CONSIDERATIONS AND FUTURE OUTLOOK 25
REFERENCES 25
Making Sense of Autoantibodies in Cholestatic Liver Diseases 33
Key points 33
INTRODUCTION 33
AUTOANTIBODIES IN PRIMARY BILIARY CIRRHOSIS 34
Antimitochondrial Antibodies 34
Anti-nuclear Antibodies 35
AUTOANTIBODIES IN PRIMARY SCLEROSING CHOLANGITIS 36
OTHER AUTOANTIBODIES 37
AUTOANTIBODY DETECTION 37
Indirect Immunofluorescence 38
Solid Phase Immunoassay 39
Other Immunoassay 39
Novel Combo Assay 40
DISCUSSION 40
REFERENCES 40
New Thoughts on Immunoglobulin G4–Related Sclerosing Cholangitis 47
Key points 47
SYNOPSIS 47
Concept of Immunoglobulin G4–Related Disease 48
Brief History 48
Terminology 48
Epidemiology and Demographics 49
CLINICAL FEATURES OF IMMUNOGLOBULIN G4–RELATED SCLEROSING CHOLANGITIS 49
Clinical Manifestations 49
Laboratory Features 49
Radiographic Features 50
Histopathology Features 52
DIAGNOSIS 53
TREATMENT 53
PROGNOSIS AND NATURAL HISTORY 54
PATHOGENESIS OF DISEASE 55
Role of Genetic Factors 56
Role of Autoantibodies and Autoantigens 57
Role of Microbial Antigens 57
Role of the Immunoglobulin G4 Molecule 57
Role of Immune Cells 59
T helper 2 (Th2) and T regulatory cells 59
Chemokine signaling 59
Innate immune cells 59
SUMMARY 60
REFERENCES 60
Primary Sclerosing Cholangitis 67
Key points 67
INTRODUCTION 67
DIAGNOSTIC CRITERIA 68
IDENTIFICATION OF PRIMARY SCLEROSING CHOLANGITIS PHENOTYPES 69
LARGE DUCT PRIMARY SCLEROSING CHOLANGITIS VARIANTS 70
Stricture Type 70
Non–Inflammatory Bowel Disease Large Duct Primary Sclerosing Cholangitis 71
High–Immunoglobulin G4 Primary Sclerosing Cholangitis 71
Primary Sclerosing Cholangitis–Autoimmune Hepatitis Overlap 71
Pediatric Primary Sclerosing Cholangitis 71
Nonwhite Population Large Duct Primary Sclerosing Cholangitis 71
SMALL DUCT PRIMARY SCLEROSING CHOLANGITIS 72
IMMUNOGLOBULIN G4–RELATED SCLEROSING CHOLANGITIS 72
MULTIPLE APPROACHES 72
Diagnosis 72
Management 73
SUMMARY AND DISCUSSION 73
REFERENCES 74
Cancer Risk and Surveillance in Primary Sclerosing Cholangitis 79
Key points 79
A BRIEF INTRODUCTION TO PRIMARY SCLEROSING CHOLANGITIS AND PRIMARY SCLEROSING CHOLANGITIS–ASSOCIATED CANCER RISK 79
PRINCIPLES OF CANCER SURVEILLANCE 80
CHOLANGIOCARCINOMA IN PATIENTS WITH PRIMARY SCLEROSING CHOLANGITIS 82
Epidemiology of Cholangiocarcinoma in Primary Sclerosing Cholangitis 82
Surveillance Strategies in Primary Sclerosing Cholangitis–Associated Cholangiocarcinoma 83
Diagnostic Modalities Relevant for Surveillance of Primary Sclerosing Cholangitis–Cholangiocarcinoma 84
Noninvasive imaging 84
Serum carbohydrate antigen 19-9 84
Endoscopic retrograde cholangiopancreatography 85
Biliary brush cytology 85
Fluorescence in situ hybridization 85
Treatment Options Relevant for Primary Sclerosing Cholangitis–Cholangiocarcinoma Surveillance 86
Suggested Surveillance Strategy for Primary Sclerosing Cholangitis–Cholangiocarcinoma 87
GALLBLADDER CARCINOMA IN PATIENTS WITH PRIMARY SCLEROSING CHOLANGITIS 87
Epidemiology of Gallbladder Carcinoma in Primary Sclerosing Cholangitis 87
SURVEILLANCE STRATEGIES IN PRIMARY SCLEROSING CHOLANGITIS–ASSOCIATED GALLBLADDER CARCINOMA 89
Diagnostic Modalities Relevant for Surveillance of Primary Sclerosing Cholangitis–Gallbladder Carcinoma 89
Treatment Options Relevant for Primary Sclerosing Cholangitis–Gallbladder Carcinoma Surveillance 89
Recommended Surveillance Strategy for Primary Sclerosing Cholangitis–Gallbladder Carcinoma 90
HEPATOCELLULAR CARCINOMA IN PATIENTS WITH PRIMARY SCLEROSING CHOLANGITIS 90
COLORECTAL CARCINOMA IN PATIENTS WITH PRIMARY SCLEROSING CHOLANGITIS 90
Epidemiology of Colorectal Carcinoma in Primary Sclerosing Cholangitis 90
SURVEILLANCE STRATEGIES IN PRIMARY SCLEROSING CHOLANGITIS–ASSOCIATED COLORECTAL CARCINOMA 91
Diagnostic Modalities Relevant to Surveillance of Primary Sclerosing Cholangitis–Colorectal Carcinoma 91
Treatment Options Relevant to Surveillance of Primary Sclerosing Cholangitis–Colorectal Carcinoma 91
Recommended surveillance strategy for primary sclerosing cholangitis–colorectal carcinoma 92
CANCER SURVEILLANCE IN SPECIFIC PRIMARY SCLEROSING CHOLANGITIS SUBPOPULATIONS 92
Cancer Surveillance in Pediatric Primary Sclerosing Cholangitis 92
Cancer Surveillance in Primary Sclerosing Cholangitis Patients Posttransplant 92
SUMMARY 93
REFERENCES 93
Sclerosing Cholangitis in Children and Adolescents 99
Key points 99
DEFINITION 99
HISTORIC BACKGROUND 99
SCLEROSING CHOLANGITIS IN CHILDREN AND ADOLESCENTS 100
DIAGNOSIS 100
EPIDEMIOLOGY 101
GENETIC FEATURES 101
PEDIATRIC SERIES 102
Retrospective Studies 102
Prospective Study 104
TREATMENT AND PROGNOSIS 106
CHOLANGIOCARCINOMA 107
LIVER TRANSPLANTATION 108
SUMMARY 108
REFERENCES 108
Novel Therapies on Primary Biliary Cirrhosis 113
Key points 113
BACKGROUND 113
URSODEOXYCHOLIC ACID 114
FUTURE DIRECTIONS AND EMERGING TREATMENTS 115
Farnesoid X Receptor Agonists/Obeticholic Acid 115
Fibrates 117
Glucocorticoids/Budesonide 122
Immunologic/Molecular Therapies 124
Anti-CD20/rituximab 124
Anti–interleukin-12/ustekinumab 124
Cytotoxic T-lymphocyte antigen 4/abatacept 125
Anti-CXCL10/MDX-1100, NI-08091 125
Mesenchymal stem cells 125
Antiretroviral therapy 126
SUMMARY 126
REFERENCES 126
Understanding and Treating Fatigue in Primary Biliary Cirrhosis and Primary Sclerosing Cholangitis 131
Key points 131
INTRODUCTION 131
THE CLINICAL SCENARIO 132
THE SCALE OF THE PROBLEM 132
ASSESSMENT OF FATIGUE 133
UNDERSTANDING FATIGUE IN PRIMARY BILIARY CIRRHOSIS AND PRIMARY SCLEROSING CHOLANGITIS 133
Central Fatigue 134
Peripheral Fatigue 135
A Model for Pathogenesis 136
TREATMENT OF FATIGUE IN PRIMARY BILIARY CIRRHOSIS AND PRIMARY SCLEROSING CHOLANGITIS 137
Novel Therapies 139
Future Perspectives 140
REFERENCES 140
Utility of Noninvasive Markers of Fibrosis in Cholestatic Liver Diseases 143
Key points 143
WHY EVALUATE FIBROSIS IN CHRONIC CHOLESTATIC LIVER DISEASES? 144
HISTOLOGY AS A GOLD STANDARD FOR ASSESSING FIBROSIS: RELEVANCE AND LIMITATIONS 145
THE (LONG) STORY OF FIBROSIS MARKERS IN CHRONIC CHOLESTATIC LIVER DISEASES 146
NONINVASIVE EVALUATION OF FIBROSIS IN PRIMARY BILIARY CIRRHOSIS: TOWARD A RATIONAL CHOICE 147
NONINVASIVE EVALUATION OF FIBROSIS IN PRIMARY SCLEROSING CHOLANGITIS: NOTHING BUT LIVER STIFFNESS MEASUREMENT? 150
PROGNOSTIC SIGNIFICANCE OF FIBROSIS MARKERS IN CHOLESTATIC LIVER DISEASES 152
SUMMARY 154
REFERENCES 154
Total Parenteral Nutrition–Induced Cholestasis 159
Key points 159
INTRODUCTION 159
Terminology 159
Definition 160
Frequency and Spectrum of Disease 160
Differences Between Children and Adults in Pathophysiology and Risk Factors for Intestinal Failure–Associated Liver Disease 161
Biological Mechanisms for Intestinal Failure–Associated Liver Disease 163
PREVENTION 165
Monitoring 165
Dedicated Expert Teams 167
MANAGEMENT 168
Sepsis 168
Components of Parenteral Nutrition 168
Calories and amino acids 168
Lipids and fish oil 168
Multiprofessional Working 170
Reducing Intestinal Failure–Associated Liver Disease Through Improvements in Central Venous Catheter Care 170
Intestinal Transplantation 170
Isolated Liver Transplantation in Intestinal Failure–Associated Liver Disease 171
SUMMARY 171
REFERENCES 172
New Insights on Intrahepatic Cholestasis of Pregnancy 177
Key points 177
INTRODUCTION 177
EPIDEMIOLOGY 177
RISK FACTORS 178
GENETICS 178
DIAGNOSIS 181
SERUM BIOCHEMISTRY 181
FETAL OUTCOME 182
MATERNAL OUTCOME 183
TREATMENT 183
SUMMARY 184
REFERENCES 185
Liver Transplantation for Cholestatic Liver Diseases in Adults 191
Key points 191
INTRODUCTION 191
PRIMARY BILIARY CIRRHOSIS 192
Identifying Patients Most Likely to Need a Liver Transplant 192
Indications for Transplantation 192
Waiting List Mortality for Patients with Primary Biliary Cirrhosis 193
Model for End-stage Liver Disease Score Exceptions in Primary Biliary Cirrhosis 193
Living Donor Liver Transplantation for Primary Biliary Cirrhosis 193
Posttransplant Outcomes in Primary Biliary Cirrhosis 194
Secondary Cholestatic Disease Posttransplantation 194
PRIMARY SCLEROSING CHOLANGITIS 196
Indications for Transplantation 196
Waiting List Mortality for Patients with Primary Sclerosing Cholangitis 197
Model for End-stage Liver Disease Exception Points for Patients with Primary Sclerosing Cholangitis 197
Posttransplant Outcomes 198
REFERENCES 200