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E-raamat: Autoimmune Liver Disease: Management and Clinical Practice

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  • Keel: eng
  • ISBN-13: 9781119532620
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A practical guide to autoimmune liver diseases through pathogenesis, diagnosis, and management

In Autoimmune Liver Disease Management and Clinical Practice, practitioners will learn about the current state of autoimmune liver disease and how to focus on their diagnosis and treatment. The four-part book begins with a thorough investigation of current immunological thinking as it relates to the autoimmunity of the liver. It also covers the four major hepatic autoimmune liver diseases in both adults and children, their management and the role of liver transplantation, and learned approaches to patient management and empowerment.

Expert authors in the field have come together to provide a thorough examination of autoimmune liver disease to help support clinicians assisting patients. The text provides an in-depth look at topics including:

&;      The four major hepatic autoimmune liver diseases, their diagnosis, and potential disease management

&;      The use (and misuse) of autoantibodies in diagnosis and treatment

&;      The role and timing of liver transplantation and the impact of recurrent autoimmune liver disease as well as de novo autoimmune hepatitis

&;      Optimal approaches to managing patients and keeping care personalised

With breadth, depth and current-day relevance, Autoimmune Liver Disease sheds light on recent developments in management of liver disease for practitioners, nurses, and health care professionals.

Preface xv
Contributors xvii
Abbreviations xxi
Introduction: The Paradigm and Paradox of Liver Autoimmunity 1(2)
M. Eric Gershwin
Section I Scientific Basis of Clinical Autoimmune Liver Diseases 3(100)
1 Introduction to the Physiology, Immunology and Pathology of the Liver and Biliary Tree
5(16)
Marco Carbone
Mario Strazzabosco
Key Points
5(1)
Liver Cell Types and Organization
6(1)
Hepatic Metabolism
7(2)
Bilirubin Metabolism and Transport
7(1)
Carbohydrate Metabolism
7(1)
Lipid Metabolism
8(1)
Protein Metabolism
8(1)
Metabolic Zonation
8(1)
Hepatic Transport Systems
9(2)
Basolateral (Sinusoidal) Transporters
9(2)
Apical (Canalicular) Transporters
11(1)
Drug Metabolism
11(1)
Bile Formation, Secretion and the Enterohepatic Circulation
12(2)
Bile Acid Synthesis and Metabolism
13(1)
Enterohepatic Bile Acid Circulation
13(1)
Death and Regeneration of Hepatocytes
14(2)
Cell Death
14(1)
Liver Regeneration
15(1)
Cholangiocyte Reaction to Biliary Damage
16(1)
Protective Role of Biliary HCO3- Secretion
16(1)
Cholangiocytes and Immunity
16(1)
Biochemical Markers and Patterns of Hepatic Injury
17(3)
Hepatocellular Necrosis
18(1)
Cholestasis
18(2)
References
20(1)
2 Concepts of Autoimmunity Relevant to Autoimmune Liver Diseases
21(26)
Isaiah G. Roepe
John M. Vierling
Key Points
21(1)
Introduction
22(1)
Role of Innate and Adaptive Immunity in Autoimmunity in the Context of the Liver as an Immune Organ
22(6)
Overview
22(1)
Innate Immunity
23(2)
Liver as an Innate Immune Organ
25(1)
Adaptive Immunity and Adaptive Immune Functions of the Liver
25(3)
Role of the Liver as an Adaptive Immune Organ
28(1)
Generation and Maintenance of Tolerance to Self-antigens
28(3)
Overview
28(1)
Central Tolerance
29(1)
Central T-cell Tolerance
29(1)
Central B-cell Tolerance
29(1)
Peripheral Tolerance
30(1)
T- and B-cell-Clonal Anergy
30(1)
T-cell Mediated Immune Regulation
30(1)
Natural and Inducible T Regulatory Cells
30(1)
Peripheral B-cell Regulatory Mechanisms
31(1)
Regulatory Dendritic Cells
31(1)
Immunoregulatory Interplay Between Treg and Th17 Cells
31(1)
Risk Factors for Autoimmune Diseases
31(3)
Genetics
31(1)
Complex Genetic and Monogenic Diseases
31(1)
HLA Risk Alleles
32(1)
Non-HLA Gene Associations
32(1)
Critical Role of Epigenetics
33(1)
Transcription Factor Enhancers and Super Enhancers
33(1)
MicroRNAs
33(1)
Sex and Sex Hormones
33(1)
Vitamin D and Sunlight Exposure
34(1)
Loss of Immune Tolerance to Autoantigens and Perpetuation of Autoimmune Diseases
34(5)
Overview
34(1)
Role of the Microbiome
35(1)
Mechanisms of Loss of Tolerance to Autoantigens
35(1)
Bacterial and Viral Infections
35(1)
Molecular Mimicry of Autoantigens
36(1)
Neoantigens
36(1)
Failure of Apoptosis to Conceal Autoantigens and Eliminate Autoreactive Cells
36(1)
Immune Deviation of Activated T Cells
37(1)
T-cell Receptor Revision in the Periphery
37(1)
Perpetuation of Autoimmune Diseases
37(1)
Epigenetics
37(1)
Epitope Spreading
38(1)
Tissue Memory T Cells
38(1)
Cytokines Promoting Chronic Inflammation and Autoimmunity
38(1)
Tertiary Lymphoid Structures and Germinal Centers
39(1)
Epithelial Cell-induced Transformation of iTreg to Th17 Cells
39(1)
Prevention of Autoimmunity and Therapeutic Control of Autoimmune Diseases
39(5)
Overview
39(1)
Strategies to Prevent Autoimmunity
39(1)
Vitamin D3
39(1)
Gut Microbiota Manipulation in Pregnancy and Infancy
39(1)
Oral Tolerance
39(1)
Strategies to Treat Established Autoimmune Diseases
40(1)
Inducible T Regulatory T Cells
40(1)
Epigenetic Enhancer Regulation
40(4)
References
44(3)
3 Genetics and Risk of Autoimmune Liver Diseases
47(18)
George F. Melts
Key Points
47(1)
Introduction
48(2)
HLA Associations in Autoimmune Liver Disease
50(3)
Non-HLA Associations in Autoimmune Liver Disease
53(8)
Conclusion
61(1)
References
62(3)
4 Autoantibodies and Understanding of Autoimmune Liver Diseases
65(18)
Benedetto Terziroli Beretta-Piccoli
Giorgina Mieli-Vergani
Diego Vergani
Key Points
65(1)
Introduction
66(1)
Methods of Detection
66(2)
Anti-nuclear Antibody
68(5)
History
68(1)
Immunofluorescence Reactivities and Antigenic Targets
68(2)
Clinical Significance in Autoimmune Liver Disease
70(3)
Anti-smooth Muscle and Anti-actin Antibodies
73(1)
History
73(1)
Immunofluorescence Reactivities, Antigenic Targets, and Clinical Significance
73(1)
Anti-liver-kidney Microsomal Antibody
74(2)
History
74(1)
Immunofluorescence Reactivities, Antigenic Targets, and Clinical Significance
75(1)
Anti-liver Cytosol Type 1 Antibody
76(1)
History
76(1)
Immunofluorescence Reactivities, Antigenic Targets, and Clinical Significance
76(1)
Anti-soluble Liver Antigen Antibody
76(1)
History
76(1)
Immunofluorescence Reactivities, Antigenic Targets, and Clinical Significance
77(1)
Anti-neutrophil Cytoplasmic Antibody
77(1)
History
77(1)
Immunofluorescence Reactivities, Antigenic Targets, and their Clinical Significance
77(1)
Anti-mitochondrial Antibody
78(1)
History
78(1)
Immunofluorescence Reactivities, Antigenic Targets, and their Clinical Significance
78(1)
Anti-asialoglycoprotein Receptor Antibody
79(1)
History
79(1)
Immunofluorescence Reactivities, Antigenic Targets, and their Clinical Significance
79(1)
Indications for Autoimmune Liver Serology Testing
79(2)
Concluding Remarks
81(1)
References
82(1)
5 Environmental Exposure and Risk in Autoimmune Liver Diseases
83(20)
Ying Qi Li
Andrew L. Mason
Key Points
83(1)
Introduction
84(1)
Autoimmunity
84(5)
Interaction of Genes and Environmental Triggers in Autoimmunity
85(1)
Mechanisms for Triggering Autoimmunity
86(3)
Primary Biliary Cholangitis
89(10)
Geo-epidemiology, Clusters, and Case-Control Studies of PBC
92(1)
Bacterial Infection and PBC
93(3)
Xenobiotics and PBC
96(1)
Viruses in PBC
97(2)
Autoimmune Hepatitis
99(2)
Prospectus
101(1)
References
102(1)
Section II Autoimmune Liver Diseases and Their Clinical Correlation 103(78)
6 Autoimmune Hepatitis
105(18)
Aliya Gulamhusein
Patrick McKiernan
Key Points
105(1)
Introduction
106(1)
Definition and Pathophysiology
106(1)
Epidemiology
107(1)
Presentation
108(1)
Adult-onset AIH
108(1)
Pediatric-onset AIH
109(1)
Diagnosis
109(3)
Autoimmune Sclerosing Cholangitis
112(1)
Treatment
112(4)
Therapeutic Approach in Adults
113(1)
First-line Therapy
113(1)
Withdrawal of Therapy
114(1)
Second-line Options
114(1)
Therapeutic Approach in Children
115(1)
First-line Therapy
115(1)
Withdrawal of Therapy
115(1)
Second-line Options
115(1)
Prognosis
116(2)
Adulthood
116(1)
Childhood
116(2)
References
118(5)
7 Primary Biliary Cholangitis
123(20)
Alessio Gerussi
Marco Carbone
Key Points
123(1)
Introduction and Definition
124(1)
Epidemiology
124(1)
Etiopathogenesis
125(1)
Clinical Presentation
126(1)
Asymptomatic Patients
126(1)
Symptomatic Patients
126(1)
Diagnosis
127(3)
Biochemical Tests
127(1)
Autoantibodies
128(1)
Liver Biopsy
128(2)
Imaging
130(1)
Differential Diagnosis
130(1)
Natural History
130(1)
Disease Course in the UDCA Era
131(1)
Risk Stratification
132(2)
Treatment
134(5)
Ursodeoxycholic Acid
134(1)
Obeticholic Acid
135(1)
Fibric Acid Derivatives
136(1)
Budesonide
137(1)
Liver Transplantation
137(1)
Symptom Management
137(2)
Complications of Liver Disease
139(2)
Osteoporosis
139(1)
Advanced Liver Disease
140(1)
Varices
140(1)
HCC
140(1)
Useful Websites
141(1)
References
141(2)
8 Primary Sclerosing Cholangitis
143(20)
Mette Vesterhus
Benedetta Terziroli Beretta-Piccoli
Kirsten Muri Boberg
Giorgina Mieli-Vergani
Key Points
143(1)
Introduction
144(1)
Definition
145(1)
Adult PSC
145(10)
Epidemiology
145(1)
Diagnosis
146(1)
Diagnosis of Variant Phenotypes
147(1)
Presentation
147(2)
Malignancy
149(1)
Cholangiocarcinoma
149(1)
Gallbladder Cancer
150(1)
Colorectal Cancer
150(1)
Prognosis
150(1)
Risk Stratification: Clinical Characteristics
150(1)
Natural History Models
151(1)
ALP and Bilirubin
151(1)
Non-invasive Evaluation of Fibrosis
151(1)
Treatment
152(1)
Medical Treatment
152(1)
Bile Acid Therapy
153(1)
Microbiota Modulators
153(1)
Anti-inflammatory Treatment
154(1)
Antibiotic Therapy
154(1)
Endoscopic Treatment
154(1)
Surveillance for Malignancy
154(1)
Colorectal Cancer: Colonoscopy
155(1)
Gallbladder Cancer: Ultrasound
155(1)
Hepatocellular Cancer: Ultrasound
155(1)
Cholangiocarcinoma
155(1)
Bone Mineral Density
155(1)
Juvenile Sclerosing Cholangitis
155(4)
Epidemiology in Pediatric Disease
155(1)
Autoimmune Sclerosing Cholangitis
156(1)
Diagnosis in Children
156(2)
Clinical Features in Pediatric Disease
158(1)
Treatment of Pediatric Disease (ASC and Juvenile PSC)
158(1)
Prognosis in Pediatric Disease
159(1)
Conclusion
159(1)
References
160(3)
9 IgG4-Related Liver and Biliary Disease
163(18)
Eleanor Barnes
Emma L. Culver
Key Points
163(1)
Introduction and Historical Perspective
164(1)
Etiology and Pathogenesis of IgG4-RD
165(2)
Development and Characteristics of an IgG
G4
Antibody Response
165(1)
Antigens That May Drive an IgG4-RD Response
166(1)
IgG4 Antibodies and Pathogenesis
167(1)
Incidence and Prevalence of IgG4-RD
167(1)
Clinical Characteristics of IgG4-related Hepatobiliary Disease
168(1)
Hepatobiliary Disease
168(1)
Disease Outside the Hepatobiliary System
168(1)
Diagnostic Criteria, Histologic Features, and Approach to Diagnosis of IgG4-related Hepatobiliary Disease
168(5)
Diagnostic Criteria for AIP and IgG4-RD
168(2)
Histologic Diagnosis of IgG4-related Hepatobiliary Disease
170(1)
Utility of Blood Tests, Including Serum IgG4 Levels in IgG4-RD Diagnostics
171(1)
Common Alternative Diagnoses to Consider
172(1)
Radiologic Characteristics of Hepatobiliary IgG4-RD
173(1)
IgG4-RD and Relationship with Malignancy
173(1)
Management and Treatment of Patients with IgG4-RD
174(1)
Monitoring and Follow-up
175(1)
Conclusions and Future Directions
175(2)
References
177(4)
Section III Specific Clinical Challenges 181(52)
10 Managing Acute and Chronic Seronegative Liver Disease
183(18)
Marcus C. Robertson
Peter C. Hayes
Key Points
183(1)
Introduction
184(1)
An Approach to Seronegative Acute Liver Failure
184(1)
Potential Causes of Seronegative ALF and Features that Suggest an Autoimmune Pathogenesis
185(3)
Clinical Features of Seronegative ALF
188(1)
Risk Stratification in Seronegative ALF
188(1)
Management of Seronegative ALF
189(3)
N-Acetylcysteine
190(1)
Corticosteroids
190(1)
Emergency Liver Transplantation
191(1)
An Approach to Cryptogenic Chronic Liver Disease
192(5)
Potential Causes of Seronegative Chronic Liver Disease and Features that Suggest an Autoimmune Pathogenesis
192(2)
Non-alcoholic Fatty Liver Disease
194(1)
Wilson Disease
194(1)
Seronegative Autoimmune Hepatitis
195(2)
Seronegative Primary Biliary Cholangitis
197(1)
Conclusion
197(1)
References
198(3)
11 Managing Pregnant Women with Autoimmune Liver Disease
201(18)
Eleni Theocharidou
Michael A. Heneghan
Key Points
201(1)
Introduction
202(1)
Fertility in AIH
202(1)
Pregnancy Outcomes in AIH
202(4)
Liver-related Outcomes in Pregnancy
206(3)
Pregnancy in Cirrhosis
209(1)
Safety of Medication in Pregnancy
210(3)
Summary
213(1)
References
214(5)
12 Bone Health in Patients with Autoimmune Liver Diseases
219(14)
Albert Pares
Nuria Guanabens
Key Points
219(1)
Introduction
220(2)
Prevalence of Osteoporosis and Fractures
222(2)
Primary Biliary Cholangitis
222(1)
Primary Sclerosing Cholangitis
223(1)
Autoimmune Hepatitis
223(1)
Pathogenesis
224(2)
Assessment of Bone Disease
226(1)
Prevention and Treatment of Bone Loss
227(3)
General Measures
227(1)
Pharmacologic Agents
227(1)
Bisphosphonates
227(1)
Other Agents
229(1)
Summary
230(1)
References
231(2)
Section IV Transplantation and Its Role in Autoimmune Liver Disease 233(30)
13 Recurrent Autoimmune Liver Disease and Its Impact on Clinical Practice
235(12)
Carlos Moctezuma-Velazquez
Aldo J. Montano-Loza
Key Points
235(1)
Introduction
236(1)
Primary Biliary Cholangitis
236(3)
Recurrence of PBC After LT
236(1)
Diagnosis of PBC After LT
236(1)
Risk Factors Associated with PBC Recurrence After LT
237(1)
Treatment of PBC Recurrence After LT
238(1)
Prognostic Impact of PBC Recurrence After LT
239(1)
Primary Sclerosing Cholangitis
239(2)
Recurrence of PSC After LT
240(1)
Diagnosis of Recurrence of PSC After LT
240(1)
Risk Factors Associated with PSC Recurrence After LT
240(1)
Treatment of PSC Recurrence After LT
241(1)
Prognostic Impact of PSC Recurrence After LT
241(1)
Autoimmune Hepatitis
241(2)
Recurrence of AIH After LT
242(1)
Risk Factors Associated with AIH Recurrence After LT
242(1)
Treatment of AIH Recurrence After LT
243(1)
Prognostic Impact of AIH Recurrence After LT
243(1)
Conclusions
243(1)
References
244(3)
14 Recurrent Autoimmune Liver Disease and its Scientific Significance
247(16)
Atsushi Tanaka
Patrick S.C. Leung
M. Eric Gershwin
Key Points
247(1)
Introduction
247(1)
Recurrence of PBC
248(3)
Incidence and Diagnosis of Recurrent PBC
249(1)
Risk Factors of Recurrent PBC
250(1)
Impact of Recurrent PBC on Long-term Outcomes
250(1)
Recurrence of PSC
251(3)
Incidence and Diagnosis of Recurrent PSC
252(1)
Risk Factors of Recurrent PSC
253(1)
Impact of Recurrent PSC on Long-term Outcomes
254(1)
Recurrence of AIH
254(2)
Incidence and Diagnosis of Recurrent AIH
254(1)
Risk Factors of Recurrent AIH
255(1)
Impact of Recurrent AIH on Long-term Outcomes
255(1)
Concluding Remarks
256(1)
References
257(6)
Section V Controversies in Autoimmune Liver Diseases 263(38)
15 Making Sense of Overlap and Crossover Syndromes
265(12)
Olivier Chazouilleres
Key Points
265(1)
Introduction
266(1)
General Considerations
267(2)
Liver Biopsy
268(1)
AIH Scores
269(1)
Genetics
269(1)
PBC/AIH Overlap Syndrome
269(3)
PSC/AIH Overlap Syndrome
272(1)
Liver Transplantation
273(1)
Conclusions
274(1)
References
275(2)
16 The Role of Extrahepatic Autoimmunity in Autoimmune Liver Disease
277(12)
Ewa Wunsch
Piotr Milkiewicz
Key Points
277(1)
Introduction
278(1)
Epidemiology
278(1)
Autoimmune Thyroid Disease
279(1)
Sjogren Syndrome
279(1)
Systemic Sclerosis
280(1)
Systemic Lupus Erythematosus
281(1)
Rheumatoid Arthritis
282(1)
Celiac Disease
283(1)
Inflammatory Bowel Disease
284(2)
Conclusions
286(1)
Guidelines for Clinicians
287(1)
References
288(1)
17 Symptoms, Chronic Disease, and Patient Management
289(12)
David Jones
Key Points
289(1)
Background
290(1)
Goals of Treatment
290(1)
Symptoms, Quality of Life, and Health Utility: Key Concepts
291(1)
Symptoms
291(1)
Health-related Quality of Life
292(1)
Health Utility
292(1)
Symptoms and Their Management in AILD
292(7)
Impact of Disease-modifying Therapy on Symptoms in AILD
292(1)
Stage-associated Symptoms
293(1)
Stage-independent Symptoms
293(1)
Pruritus
293(1)
Fatigue and Cognitive Symptoms
295(1)
Social Isolation Symptoms
298(1)
Effective Care Delivery in AILD
299(1)
References
300(1)
Index 301
JAMES NEUBERGER studied at Oxford and trained at the Liver Unit, Kings College Hospital, in London. He has researched and written about liver disease and liver transplantation for over 35 years, including editing five books and serving as the editor of Transplantation and associate editor of The Journal of Hepatology. He is currently a consultant physician in the Liver Unit, Queen Elizabeth Hospital, Birmingham, UK.

GIDEON M. HIRSCHFIELD is a clinician-scientist and expert in autoimmune liver disease practice and science. His experience spans many years in transplant Hepatology in North America and the United Kingdom, including numerous published works. He currently has a practice at the University of Toronto, Ontario, Canada.