Diagnosis and Management of Autoimmune Hepatitis: A Clinical Guide

Contents
Contributors
Chapter 1: Epidemiology and Burden of Disease
	Introduction
	Epidemiology and Burden of Disease
		Incidence and Prevalence in North America, Europe, and Asia
			Risk Factors: Gender, Race, and Age
			Autoantibodies
			Concurrent Nonhepatic Autoimmune Diseases
		Burden of Disease
	References
Chapter 2: The Pathogenesis of Autoimmune Liver Diseases
	Overview
	Pathogenesis of Classic Autoimmune Hepatitis
		Environmental Factors That Contribute to the Pathogenesis of AIH
	Pathogenesis of Primary Biliary Cholangitis, Primary Sclerosing Cholangitis, and Overlap Syndromes
		Primary Biliary Cholangitis
		Cholangiocyte Physiology and Pathophysiology
			Normal Functions of Healthy Cholangiocytes
			Defective Biliary Bicarbonate Umbrella
			Cholangiocyte Injury and Apoptosis
			Cholangiocyte Senescence
		Environmental Factors
			Molecular Mimicry
			Xenobiotics
			Smoking as a Risk Factor
			Intestinal Dysbiosis
		Hepatic Immune Dysregulation
			Autocrine and Paracrine Signaling from Activated Cholangiocytes
			Dysregulation of Innate Immunity
			Aberrancies of Adaptive Immunity
			Histologic Hallmarks of PBC
		Autoantibodies
		Genetic and Epigenetic Influences
			Genetic Factors
			Epigenetic Influences
		Primary Sclerosing Cholangitis
		Cholangiocyte Pathophysiology
			Proposed Mechanisms for Cholangiocyte Injury and Activation
		Environmental Factors
			Protective Effect of Smoking
			Intestinal Dysbiosis
		Immune Dysregulation in PSC
		Autoantibodies
		Considerations in PSC-IBD
			IBD Disease and Its Effect on PSC
			Status of PSC-IBD After Colectomy
			Malignancy Risk
		PSC Recurrence Post Liver Transplantation
		Genetic and Epigenetic Influences
			Genetic Factors
			Epigenetic Influences
		Overlap Syndromes
	References
Chapter 3: Clinical Presentation and Diagnosis
	Introduction
	Classification of AIH
		Type 1
		Type 2 AIH
	Clinical Presentations
		Acute AIH
		Acute Liver Failure
		Chronic Liver Disease
	Cirrhosis
	Diagnosis and Clinical Presentation
	Auto-Antibodies
	Practical Clinical Considerations
	Histology
	Scoring Systems
	References
Chapter 4: The Pathology of Autoimmune Hepatitis
	Introduction
	Autoimmune Hepatitis Types
	Autoimmune Hepatitis Scoring Systems
	Newly Discovered Problems with Classic Autoimmune Hepatitis Histology
	Acute, Acute on Chronic, and Chronic Presentation of Autoimmune Hepatitits
	Differentiating Autoimmune Hepatitis from Infection and Drug Induced Liver Injury
	Autoimmune Hepatitis with PBC and PSC Overlap
	References
Chapter 5: Treating the Adult Patient: First Line Therapy
	Introduction
	Treatment of Mild AIH
	Induction Therapy
	Maintenance Treatment
	Budesonide as an Alternative First-Line Agent
	Acute Severe Autoimmune Hepatitis
	Treatment Duration and Withdrawal
	Therapeutic Drug Monitoring
	Conclusion
	References
Chapter 6: Treating the Adult Patient: Alternative Drug Therapies
	Introduction
	Predictors of Poor Response
	Evaluating the Nonresponder
	Budesonide
	Thiopurines
	Mycophenolate Mofetil
	Calcineurin Inhibitors
	mTOR Inhibitors
	Rituximab
	Infliximab
	Investigational Agents
	References
Chapter 7: The Approach to the Pediatric Patient
	Autoimmune Hepatitis
	Pathologic Features
	Treatment
	When to Treat
	How to Treat (Fig. 7.1)
	Standard Treatment
	Alternative Treatments: For Induction of Remission
	Alternative Treatments: For Refractory Cases
	Autoimmune Sclerosing Cholangitis
	Liver Transplantation for Pediatric Autoimmune Liver Disease
	References
Chapter 8: Autoimmune Hepatitis and Pregnancy
	Introduction
	Pre-conception Counseling
	Management During Pregnancy
		Disease Course During Pregnancy
		Immunosuppressive Medications During Pregnancy
		Pregnancy Outcomes
	Post-partum Management
	References
Chapter 9: Liver Transplantation for Autoimmune Hepatitis
	Introduction
	Presentations of Autoimmune Hepatitis Leading to Transplant
		Acute Severe Autoimmune Hepatitis
		Laboratory and Imaging Features of Acute Severe Autoimmune Hepatitis
		Histology of Acute Severe Autoimmune Hepatitis
		Steroid Use and Response in Acute Severe Autoimmune Hepatitis
		Other Immunosuppressants and Response
		Appropriate Timing of Liver Transplantation for Acute Severe Autoimmune Hepatitis
		Liver Transplant for Chronic Autoimmune Hepatitis
	Presentations of Autoimmune Hepatitis After Liver Transplant
		Recurrent Autoimmune Hepatitis After Liver Transplant
		Prevention and Treatment of Recurrent Autoimmune Hepatitis
		Autoantibodies After Liver Transplantation
		De Novo Autoimmune Hepatitis After Liver Transplantation
	Chapter Summary
	Useful Tips for Practitioners
	Common Pitfalls in Practice
	References
Chapter 10: Autoimmune Overlap Syndromes
	Introduction
	Autoimmune Hepatitis and Primary Biliary Cholangitis Overlap
	Diagnosis
	Clinical Presentation
	Management
	Autoimmune Hepatitis and Primary Sclerosing Cholangitis (AIH-PSC Overlap)
	Clinical Features
	Treatment
	Liver Transplantation for Overlap Syndromes
	References
Chapter 11: Drug-Induced Liver Injury with Autoimmune Features
	Introduction
	Diagnosis of Drug-Induced Autoimmune Liver Disease
	Risk Factors for DIAILD
	Clinical and Laboratory Features of DIAILD Versus iAIH
	Histology
	Treatment and Prognosis
	Nitrofurantoin
	Minocycline
	HMG-CoA Reductase Inhibitors (Statins)
	Tumor Necrosis Factor (TNF)-α Antagonists
	References
Index