Autoimmune Liver Disease : Management and Clinical Practice (Hardcover)

· 제목 : Autoimmune Liver Disease : Management and Clinical Practice (Hardcover) 
· 분류 : 외국도서 > 의학 > 위장병학
· ISBN : 9781119532606
· 쪽수 : 336쪽
· 출판일 : 2020-04-02

Introduction: The Paradigm and Paradox of Liver Autoimmunity, 00

Section I Scientific Basis of Clinical Autoimmune Liver Diseases

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

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

Peripheral Tolerance

Risk Factors for Autoimmune Diseases

Genetics

Critical Role of Epigenetics

Sex and Sex Hormones

Vitamin D and Sunlight Exposure

Loss of Immune Tolerance to Autoantigens and Perpetuation of Autoimmune Diseases

Overview

Mechanisms of Loss of Tolerance to Autoantigens

Perpetuation of Autoimmune Diseases

Prevention of Autoimmunity and Therapeutic Control of Autoimmune Diseases

Overview

Strategies to Prevent Autoimmunity

Strategies to Treat Established Autoimmune Diseases

References

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

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

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

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

Therapeutic Approach in Children

Prognosis

Adulthood

Childhood

References

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

References

Useful Websites

8 Primary Sclerosing Cholangitis

Key Points

Introduction

Definition

Adult PSC

Epidemiology

Diagnosis

Presentation

Malignancy

Prognosis

Treatment

Surveillance for Malignancy

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

9 IgG4-associated 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

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

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

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

Summary

References

Section IV Transplantation and Its Role in Autoimmune Liver Disease

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

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

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

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

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

Effective Care Delivery in AILD

References