Autoimmune Liver Disease

Name: Autoimmune Liver Disease | Management and Clinical Practice
Brand: Jossey-Bass
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Management and Clinical Practice

James Neuberger Gideon M. Hirschfield(Herausgeber*in)

Jossey-Bass (Verlag)

1. Auflage

Erschienen am 28. Januar 2020

336 Seiten

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Inhalt

Cover
Title Page
Copyright Page
Contents
Preface
Contributors
Abbreviations
Introduction: The Paradigm and Paradox of Liver Autoimmunity
Section I Scientific Basis of Clinical Autoimmune Liver Diseases
Chapter 1 Introduction to the Physiology, Immunology and Pathology of the Liver and Biliary Tree
Key Points
Liver Cell Types and Organization
Hepatic Metabolism
Bilirubin Metabolism and Transport
Carbohydrate Metabolism
Lipid Metabolism
Protein Metabolism
Metabolic Zonation
Hepatic Transport Systems
Basolateral (Sinusoidal) Transporters
Apical (Canalicular) Transporters
Drug Metabolism
Bile Formation, Secretion and the Enterohepatic Circulation
Bile Acid Synthesis and Metabolism
Enterohepatic Bile Acid Circulation
Death and Regeneration of Hepatocytes
Cell Death
Liver Regeneration
Cholangiocyte Reaction to Biliary Damage
Protective Role of Biliary HCO3 Secretion
Cholangiocytes and Immunity
Biochemical Markers and Patterns of Hepatic Injury
Hepatocellular Necrosis
Cholestasis
References
Chapter 2 Concepts of Autoimmunity Relevant to Autoimmune Liver Diseases
Key Points
Introduction
Role of Innate and Adaptive Immunity in Autoimmunity in the Context of the Liver as an Immune Organ
Overview
Innate Immunity
Liver as an Innate Immune Organ
Adaptive Immunity and Adaptive Immune Functions of the Liver
Role of the Liver as an Adaptive Immune Organ
Generation and Maintenance of Tolerance to Self-antigens
Overview
Central Tolerance
Central T-cell Tolerance
Central B-cell Tolerance
Peripheral Tolerance
T- and B-cell Clonal Anergy
T-cell Mediated Immune Regulation
Natural and Inducible T Regulatory Cells
Peripheral B-cell Regulatory Mechanisms
Regulatory Dendritic Cells
Immunoregulatory Interplay Between Treg and Th17 Cells
Risk Factors for Autoimmune Diseases
Genetics
Complex Genetic and Monogenic Diseases
HLA Risk Alleles
Non-HLA Gene Associations
Critical Role of Epigenetics
Transcription Factor Enhancers and Super Enhancers
MicroRNAs
Sex and Sex Hormones
Vitamin D and Sunlight Exposure
Loss of Immune Tolerance to Autoantigens and Perpetuation of Autoimmune Diseases
Overview
Role of the Microbiome
Mechanisms of Loss of Tolerance to Autoantigens
Bacterial and Viral Infections
Molecular Mimicry of Autoantigens
Neoantigens
Failure of Apoptosis to Conceal Autoantigens and Eliminate Autoreactive Cells
Immune Deviation of Activated T Cells
T-cell Receptor Revision in the Periphery
Perpetuation of Autoimmune Diseases
Epigenetics
Epitope Spreading
Tissue Memory T Cells
Cytokines Promoting Chronic Inflammation and Autoimmunity
Tertiary Lymphoid Structures and Germinal Centers
Epithelial Cell-induced Transformation of iTreg to Th17 Cells
Prevention of Autoimmunity and Therapeutic Control of Autoimmune Diseases
Overview
Strategies to Prevent Autoimmunity
Vitamin D3
Gut Microbiota Manipulation in Pregnancy and Infancy
Oral Tolerance
Strategies to Treat Established Autoimmune Diseases
Inducible T Regulatory T Cells
Epigenetic Enhancer Regulation
References
Chapter 3 Genetics and Risk of Autoimmune Liver Diseases
Key Points
Introduction
HLA Associations in Autoimmune Liver Disease
Non-HLA Associations in Autoimmune Liver Disease
Conclusion
References
Chapter 4 Autoantibodies and Understanding of Autoimmune Liver Diseases
Key Points
Introduction
Methods of Detection
Anti-nuclear Antibody
History
Immunofluorescence Reactivities and Antigenic Targets
Clinical Significance in Autoimmune Liver Disease
Anti-smooth Muscle and Anti-actin Antibodies
History
Immunofluorescence Reactivities, Antigenic Targets, and Clinical Significance
Anti-liver-kidney Microsomal Antibody
History
Immunofluorescence Reactivities, Antigenic Targets, and Clinical Significance
Anti-liver Cytosol Type 1 Antibody
History
Immunofluorescence Reactivities, Antigenic Targets, and Clinical Significance
Anti-soluble Liver Antigen Antibody
History
Immunofluorescence Reactivities, Antigenic Targets, and Clinical Significance
Anti-neutrophil Cytoplasmic Antibody
History
Immunofluorescence Reactivities, Antigenic Targets, and their Clinical Significance
Anti-mitochondrial Antibody
History
Immunofluorescence Reactivities, Antigenic Targets, and their Clinical Significance
Anti-asialoglycoprotein Receptor Antibody
History
Immunofluorescence Reactivities, Antigenic Targets, and their Clinical Significance
Indications for Autoimmune Liver Serology Testing
Concluding Remarks
References
Chapter 5 Environmental Exposure and Risk in Autoimmune Liver Diseases
Key Points
Introduction
Autoimmunity
Interaction of Genes and Environmental Triggers in Autoimmunity
Mechanisms for Triggering Autoimmunity
Primary Biliary Cholangitis
Geo-epidemiology, Clusters, and Case-Control Studies of PBC
Bacterial Infection and PBC
Xenobiotics and PBC
Viruses in PBC
Autoimmune Hepatitis
Prospectus
References
Section II Autoimmune Liver Diseases and Their Clinical Correlation
Chapter 6 Autoimmune Hepatitis
Key Points
Introduction
Definition and Pathophysiology
Epidemiology
Presentation
Adult-onset AIH
Pediatric-onset AIH
Diagnosis
Autoimmune Sclerosing Cholangitis
Treatment
Therapeutic Approach in Adults
First-line Therapy
Withdrawal of Therapy
Second-line Options
Therapeutic Approach in Children
First-line Therapy
Withdrawal of Therapy
Second-line Options
Prognosis
Adulthood
Childhood
References
Chapter 7 Primary Biliary Cholangitis
Key Points
Introduction and Definition
Epidemiology
Etiopathogenesis
Clinical Presentation
Asymptomatic Patients
Symptomatic Patients
Diagnosis
Biochemical Tests
Autoantibodies
Liver Biopsy
Imaging
Differential Diagnosis
Natural History
Disease Course in the UDCA Era
Risk Stratification
Treatment
Ursodeoxycholic Acid
Obeticholic Acid
Fibric Acid Derivatives
Budesonide
Liver Transplantation
Symptom Management
Complications of Liver Disease
Osteoporosis
Advanced Liver Disease
Varices
HCC
Useful Websites
References
Chapter 8 Primary Sclerosing Cholangitis
Key Points
Introduction
Definition
Adult PSC
Epidemiology
Diagnosis
Diagnosis of Variant Phenotypes
Presentation
Malignancy
Cholangiocarcinoma
Gallbladder Cancer
Colorectal Cancer
Prognosis
Risk Stratification: Clinical Characteristics
Natural History Models
ALP and Bilirubin
Non-invasive Evaluation of Fibrosis
Treatment
Medical Treatment
Bile Acid Therapy
Microbiota Modulators
Anti-inflammatory Treatment
Antibiotic Therapy
Endoscopic Treatment
Surveillance for Malignancy
Colorectal Cancer: Colonoscopy
Gallbladder Cancer: Ultrasound
Hepatocellular Cancer: Ultrasound
Cholangiocarcinoma
Bone Mineral Density
Juvenile Sclerosing Cholangitis
Epidemiology in Pediatric Disease
Autoimmune Sclerosing Cholangitis
Diagnosis in Children
Clinical Features in Pediatric Disease
Treatment of Pediatric Disease (ASC and Juvenile PSC)
Prognosis in Pediatric Disease
Conclusion
References
Chapter 9 IgG4-Related Liver and Biliary Disease
Key Points
Introduction and Historical Perspective
Etiology and Pathogenesis of IgG4-RD
Development and Characteristics of an IgG4 Antibody Response
Antigens That May Drive an IgG4-RD Response
IgG4 Antibodies and Pathogenesis
Incidence and Prevalence of IgG4-RD
Clinical Characteristics of IgG4-related Hepatobiliary Disease
Hepatobiliary Disease
Disease Outside the Hepatobiliary System
Diagnostic Criteria, Histologic Features, and Approach to Diagnosis of IgG4-related Hepatobiliary Disease
Diagnostic Criteria for AIP and IgG4-RD
Histologic Diagnosis of IgG4-related Hepatobiliary Disease
Utility of Blood Tests, Including Serum IgG4 Levels in IgG4-RD Diagnostics
Common Alternative Diagnoses to Consider
Radiologic Characteristics of Hepatobiliary IgG4-RD
IgG4-RD and Relationship with Malignancy
Management and Treatment of Patients with IgG4-RD
Monitoring and Follow-up
Conclusions and Future Directions
References
Section III Specific Clinical Challenges
Chapter 10 Managing Acute and Chronic Seronegative Liver Disease
Key Points
Introduction
An Approach to Seronegative Acute Liver Failure
Potential Causes of Seronegative ALF and Features that Suggest an Autoimmune Pathogenesis
Clinical Features of Seronegative ALF
Risk Stratification in Seronegative ALF
Management of Seronegative ALF
N-Acetylcysteine
Corticosteroids
Emergency Liver Transplantation
An Approach to Cryptogenic Chronic Liver Disease
Potential Causes of Seronegative Chronic Liver Disease and Features that Suggest an Autoimmune Pathogenesis
Non-alcoholic Fatty Liver Disease
Wilson Disease
Seronegative Autoimmune Hepatitis
Seronegative Primary Biliary Cholangitis
Conclusion
References
Chapter 11 Managing Pregnant Women with Autoimmune Liver Disease
Key Points
Introduction
Fertility in AIH
Pregnancy Outcomes in AIH
Liver-related Outcomes in Pregnancy
Pregnancy in Cirrhosis
Safety of Medication in Pregnancy
Summary
References
Chapter 12 Bone Health in Patients with Autoimmune Liver Diseases
Key Points
Introduction
Prevalence of Osteoporosis and Fractures
Primary Biliary Cholangitis
Primary Sclerosing Cholangitis
Autoimmune Hepatitis
Pathogenesis
Assessment of Bone Disease
Prevention and Treatment of Bone Loss
General Measures
Pharmacologic Agents
Bisphosphonates
Other Agents
Summary
References
Section IV Transplantation and Its Role in Autoimmune Liver Disease
Chapter 13 Recurrent Autoimmune Liver Disease and Its Impact on Clinical Practice
Key Points
Introduction
Primary Biliary Cholangitis
Recurrence of PBC After LT
Diagnosis of PBC After LT
Risk Factors Associated with PBC Recurrence After LT
Treatment of PBC Recurrence After LT
Prognostic Impact of PBC Recurrence After LT
Primary Sclerosing Cholangitis
Recurrence of PSC After LT
Diagnosis of Recurrence of PSC After LT
Risk Factors Associated with PSC Recurrence After LT
Treatment of PSC Recurrence After LT
Prognostic Impact of PSC Recurrence After LT
Autoimmune Hepatitis
Recurrence of AIH After LT
Risk Factors Associated with AIH Recurrence After LT
Treatment of AIH Recurrence After LT
Prognostic Impact of AIH Recurrence After LT
Conclusions
References
Chapter 14 Recurrent Autoimmune Liver Disease and its Scientific Significance
Key Points
Introduction
Recurrence of PBC
Incidence and Diagnosis of Recurrent PBC
Risk Factors of Recurrent PBC
Impact of Recurrent PBC on Long-term Outcomes
Recurrence of PSC
Incidence and Diagnosis of Recurrent PSC
Risk Factors of Recurrent PSC
Impact of Recurrent PSC on Long-term Outcomes
Recurrence of AIH
Incidence and Diagnosis of Recurrent AIH
Risk Factors of Recurrent AIH
Impact of Recurrent AIH on Long-term Outcomes
Concluding Remarks
References
Section V Controversies in Autoimmune Liver Diseases
Chapter 15 Making Sense of Overlap and Crossover Syndromes
Key Points
Introduction
General Considerations
Liver Biopsy
AIH Scores
Genetics
PBC/AIH Overlap Syndrome
PSC/AIH Overlap Syndrome
Liver Transplantation
Conclusions
References
Chapter 16 The Role of Extrahepatic Autoimmunity in Autoimmune Liver Disease
Key Points
Introduction
Epidemiology
Autoimmune Thyroid Disease
Sjögren Syndrome
Systemic Sclerosis
Systemic Lupus Erythematosus
Rheumatoid Arthritis
Celiac Disease
Inflammatory Bowel Disease
Conclusions
Guidelines for Clinicians
References
Chapter 17 Symptoms, Chronic Disease, and Patient Management
Key Points
Background
Goals of Treatment
Symptoms, Quality of Life, and Health Utility: Key Concepts
Symptoms
Health-related Quality of Life
Health Utility
Symptoms and Their Management in AILD
Impact of Disease-modifying Therapy on Symptoms in AILD
Stage-associated Symptoms
Stage-independent Symptoms
Pruritus
Fatigue and Cognitive Symptoms
Social Isolation Symptoms
Effective Care Delivery in AILD
References
Index
EULA

Abbreviations

AASLD: American Association for the Study of Liver Diseases
ABC: ATP-binding cassette
ABD: autoimmune biliary disease
ACA: anti-centromere antibody
ACR: American College of Rheumatology
ADCC: antibody-dependent cell-mediated cytotoxicity
AFLP: acute fatty liver disease of pregnancy
AIH: autoimmune hepatitis
AILD: autoimmune liver disease
AIP: autoimmune pancreatitis
AIRE: autoimmune regulator
AISC: autoimmune sclerosing cholangitis
ALF: acute liver failure
ALP: alkaline phosphatase
ALT: alanine aminotransferase
AMA: anti-mitochondrial antibody
ANA: anti-nuclear antibody
ANCA: anti-neutrophil cytoplasmic antibody
APC: antigen-presenting cell
APECED: autoimmune polyendocrinopathy/candidiasis/ectodermal dystrophy
APRI: AST-to-platelet ratio index
APS-1: autoimmune polyendocrine syndrome type 1
ASC: autoimmune sclerosing cholangitis
ASGPR: asialoglycoprotein receptor
ASMA: anti-smooth muscle antibody
AST: aspartate aminotransferase
ATX: autotaxin
BA: bile acid
BCOADC: branched-chain 2-oxo acid dehydrogenase complex
BCR: B-cell receptor
BEC: biliary epithelial cell
BET: bromodomain and extra-terminal
BLC: B-lymphocyte chemoattractant
BMD: bone mineral density
Breg: B regulatory cell
BSEP: bile salt export pump
BTSC: biliary tree stem/progenitor cell
C´: complement
CA19-9: carbohydrate antigen 19-9
CC: cryptogenic cirrhosis
CCH: cryptogenic chronic hepatitis
CD: celiac disease
CDCA: chenodeoxycholic acid
CI: confidence interval
CNSDC: chronic non-suppurative destructive cholangitis
CREB: cAMP response element binding protein
CS: Cesarean section
CT: computed tomography
CTD: connective tissue disease
CTL: cytotoxic T lymphocyte
CTLA: cytotoxic T-lymphocyte antigen
CYP: cytochrome P
D3: 1,25-dihydroxyvitamin D3
DAG: diacylglycerol
DAMP: damage-associated molecular pattern
DC: dendritic cell
DCD: donation after circulatory death
DCreg: regulatory dendritic cell
DDLT: deceased donor liver transplant
DILI: drug-induced liver injury
DOCK1: dedicator of cytokinesis 1 (protein)
DXA: dual-energy X-ray absorptiometry
EASL: European Association for the Study of the Liver
EGD: esophagogastroduodenoscopy
EHAID: extrahepatic autoimmune disease
ELF: enhanced liver fibrosis (test)
ELISA: enzyme-linked immunosorbent assay
ELTR: European Liver Transplant Registry
ENA: extractable nuclear antigen
eQTL: expression quantitative trait locus
ERCP: endoscopic retrograde cholangiopancreatography
eRNA: enhancer RNA
EULAR: European League Against Rheumatism
EUS: endoscopic ultrasound
EVL: endoscopic variceal ligation
FDA: Food and Drug Administration
FISH: fluorescence in situ hybridization
FNA: fine needle aspiration
FoxP: forkhead box P3
FXR: farnesoid X receptor
GEF: guanine nucleotide exchange factor
GGT: gamma-glutamyltranspeptidase (or gamma-glutamyltransferase)
GLP: glucagon-like peptide
GWAS: genome-wide association studies
HAI: hepatic/hepatitis activity index
HBRV: human betaretrovirus
HBV: hepatitis B virus
HCC: hepatocellular carcinoma
HCV: hepatitis C virus
HDL: high-density lipoprotein
HLA: human leukocyte antigen
HMG-CoA: 3-hydroxy-3-methylglutaryl-coenzyme A
HPF: high-power field
HpSC: hepatic stem/progenitor cell
HR: hazard ratio
HRQoL: health-related quality of life
HSC: hepatic stellate cell
HSEC: hepatic sinusoidal endothelial cell
HSV: herpes simplex virus
IAC: IgG4-associated cholangitis
IAH: International Autoimmune Hepatitis (score)
IAIHG: International IAH Group
IBD: inflammatory bowel disease
ICU: intensive care unit
IgG4-RD: IgG4-related disease
IIF: indirect immunofluorescence
IL: interleukin
INR: international normalized ratio
IPEX: immunodysregulation polyendocrinopathy enteropathy X-linked (syndrome)
IRF: interferon regulatory factor
ISRE: interferon-stimulated response element
JAK: Janus kinase
LAK: lymphokine-activated killer (cell)
LC1: liver cytosol type 1 (antibody)
LD: linkage disequilibrium
LDL: low-density lipoprotein
LDLT: living donor liver transplant
LE: lupus erythematosus
LFT: liver function test
LKM1: liver kidney microsomal type 1 (antibody)
lncRNA: long non-coding RNA
LPA: lysophosphatidic acid
LPS: lipopolysaccharide
LSEC: liver sinusoidal endothelial cell
LT: liver transplantation
MAGUK: membrane-associated guanylate kinase
MAIT: mucosal invariant T (cell)
MAPK: mitogen-activated protein kinase
MDR: multidrug resistance
MELD: Model for End-stage Liver Disease (score)
MHC: major histocompatibility complex
MIF: macrophage migration inhibitory factor
miRNA: microRNA
MMF: mycophenolate mofetil
6-MMP: 6-methylmercaptopurine
MMTV: mouse mammary tumor virus
6-MP: 6-mercaptopurine
mQTL: methylation quantitative trait locus
MRC: magnetic resonance cholangiography
MRCP: magnetic resonance cholangiopancreatography
MRP: multidrug resistance protein
MS: multiple sclerosis
mTEC: medullary thymic epithelial cell
NAC: N-acetylcysteine
NAFLD: non-alcoholic fatty liver disease
NANA: nuclear anti-neutrophil antibody
NASH: non-alcoholic steatohepatitis
NET: neutrophil extracellular trap
NF?B: nuclear factor kappa B
NICE: National Institute for Health and Care Excellence (UK)
NICU: neonatal intensive care unit
NK: natural killer (cell)
NKT: natural killer T (cell)
NLRP3: nucleotide-binding oligomerization domain (NOD)-like receptor P3
NOD: non-obese diabetic
NSAID: non-steroidal anti-inflammatory drug
NTCP: sodium taurocholate cotransporting polypeptide
OATP: organic anion-transporting polypeptide
OCA: obeticholic acid
OGDC: 2-oxoglutarate dehydrogenase complex
OPG: osteoprotegerin
OR: odds ratio
OS: overlap syndrome
PAMP: pathogen-associated molecular pattern
pANCA: perinuclear anti-neutrophil cytoplasmic antibody
pANNA: perinuclear anti-neutrophil nuclear antibody
PBC: primary biliary cholangitis
PBG: peribiliary gland
PD-1: programmed cell death 1
PDC: pyruvate dehydrogenase complex
PET: positron emission tomography
PKC: protein kinase C
P1NP: procollagen type 1 N-terminal propeptide
PPAR: peroxisome proliferator-activated receptor
PRR: pattern recognition receptor
PSC: primary sclerosing cholangitis
PXR: pregnane X receptor
QALY: quality-adjusted life-year
RA: rheumatoid arthritis
RAG: recombination activating gene
rAIH: recurrent autoimmune hepatitis
RANKL: receptor activator of nuclear factor kappa-B ligand
RDC: reactive ductular cell
RF: rheumatoid...

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