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    Autoimmune diseases (Rose)
    The autoimmune diseases / edited by Noel Richard Rose, Ian R. MacKay.

    • Title:[Autoimmune diseases (Rose)]
      The autoimmune diseases / edited by Noel Richard Rose, Ian R. MacKay.
    •    
    • Other Contributors/Collections:Rose, Noel R., editor.
      Mackay, Ian R., editor.
    • Published/Created:Amsterdam : Elsevier/Academic Press, 2014.
    • Holdings

       
    • Library of Congress Subjects:Autoimmune diseases.
    • Medical Subjects: Autoimmune Diseases.
    • Edition:Fifth edition.
    • Description:xxxiii, 1, 267 pages : illustrations (some colored) ; 29 cm
    • Summary:This edition of 'Autoimmune Diseases' incorporates new material and combines common themes underlying inductive and effector mechanisms and therapies that relate generally to the autoimmune disorders. It discusses the biological basis of disease at genetic, molecular, cellular, and epidemiologic levels.
    • Notes:Previous edition: 2006.
      Includes bibliographical references and index.
    • ISBN:9780123849298 (hbk.)
      0123849292 (hbk.)
      9780123849304 (PDF ebook)
    • Contents:Machine generated contents note: pt. 1 Immunologic Basis of Autoimmunity
      1. Autoimmune Disease: The Consequence of Disturbed Homeostasis / Ian R. Mackay
      Evolution of the Autoimmune Response
      Phylogenetic Perspective
      Innate Immune System
      Adaptive Immune System
      Self and Non-Self, and the Nature of Autoantigens
      Autoimmunity and Autoimmune Disease
      Common Threads
      "Proper Study..."
      Towards the Future
      Last Word
      References
      2. Autoimmunity: A History of the Early Struggle for Recognition / Arthur M. Silverstein
      Search for Autoantibodies
      Horror Autotoxicus
      Nature of Ehrlich's "Contrivances"
      Challenges to the Thesis
      Lens Autoantibodies
      Paroxysmal Cold Hemoglobinuria
      Sympathetic Ophthalmia
      Wassermann Antibody
      Shift to Immunochemistry
      Return of Immunobiology
      Concluding Remarks
      References
      3. General Features of Autoimmune Disease / Betty Diamond
      Innate Immune Activation
      Cells of the Adaptive Immune System
      Defining Autoimmune Disease
      Prevalence of Autoimmunity
      Genetics of Autoimmunity
      Association of Single Gene Defects with Autoimmunity
      Genome-wide Association Studies (GWAS) Identify Multiple Gene Loci that are Associated with Autoimmunity
      Hormones and Autoimmunity
      Autoimmunity and Central Tolerance
      Autoimmunity and Peripheral Tolerance
      Triggers of Autommunity
      Activation of the Immune System
      Role of Antigen as a Driver of Autoimmunity
      Defective Downregulation of an Immune Response
      Regulatory Lymphocytes
      Role of the Gut Microbiota in Autoimmunity
      Mechanisms of Tissue Damage
      Flares and Remissions During Disease
      Therapeutic Advances
      Goals for the Future
      Concluding Remarks
      Acknowledgments
      References
      4. Concept of Autoinflammatory Diseases / Anna Simon
      Historical Perspective
      Definition
      Spectrum from Autoimmune to Autoinflammatory Disease
      Mechanisms in Autoinflammation
      Classical Hereditary Autoinflammatory Disorders
      Common Phenotype
      Cryopyrin-Associated Periodic Syndrome
      Familial Mediterranean Fever
      TNF Receptor-Associated Periodic Syndrome
      HIDS (also Known as Mevalonate Kinase Deficiency)
      Polygenic or Acquired Autoinflammatory Disorders
      Autoinflammatory Mechanisms of Disease in Common Disorders
      Type 2 Diabetes Mellitus
      Atherosclerosis
      Newest Developments: Rare Hereditary Disorders with Autoinflammatory Aspects
      Proteasome Defects
      PLCγ2 Deficiency
      Heme-Oxidized IRP2 Ubiquitin Ligase
      Conclusion
      References
      pt. 2 Immune Cells and Immune Responses
      5. Innate and Adaptive Systems of Immunity / Peter J. Delves
      Introduction
      Innate and Adaptive Responses
      Innate Responses
      Cellular Components
      Soluble Mediators
      Adaptive Immune Responses
      T Cell Development
      Functional Activities of T Cells
      B Cell Development and Functions
      Antibodies
      Secondary Lymphoid Tissues
      Resolution of the Immune Response
      References
      6. T Cells and their Subsets in Autoimmunity / Vijay K. Kuchroo
      Introduction
      TH1 Cells
      TH17 Cells
      Discovery and Differentiation
      Function
      Reciprocal Relationship with Tregs
      Role of IL-23 in the Generation of Th17 Cells
      Th17 Pathogenicity and Plasticity
      Th17 Regulation in the Intestine
      Regulatory CD4+ T Cells
      Tr1 Cells
      TFH Cells
      Th2 Cells
      Th9 Cells
      Concluding Remarks
      References
      7. Immunological Tolerance
      -T Cells / Pamela S. Ohashi
      Early Studies Supporting the Induction of Tolerance
      Thymic Tolerance
      Brief Overview of Thymocyte Development
      Mechanisms of Thymic Tolerance
      Expression of Tissue-Restricted Antigens in the Thymus
      Peripheral Tolerance
      Impact of Dendritic Cells
      Mechanisms of Peripheral Tolerance
      Expression of Tissue-Specific Antigens by Lymph Node Stromal Cells
      Concluding Remarks
      Acknowledgments
      References
      8. Role of Invariant Natural Killer T Cells in Autoimmune Diseases / Mitchell Kronenberg
      Curious Case of iNKT Cells
      Many Names of NKT Cells
      Many Faces of iNKT Cells
      Many Effector Functions of iNKT Cells
      Many Kinds of iNKT Cells
      Technical Problems and the Species Divide
      Janus-Like Character of iNKT Cells in Autoimmunity
      Too Much of a Good Thing: Detrimental Roles of iNKT Cells
      Missed so Sadly: Beneficial Roles of iNKT Cells
      Good or Bad Performers?
      Far End of the Question?
      Conclusion
      Acknowledgments
      Abbreviations
      References
      9. B Cell Development: How to Become One of the Chosen Ones / Fritz Melchers
      Introduction
      -What has to be Generated in B Cell Development to Make it to Maturity?
      Follicular B Cells
      Intraepithelial B Cells
      Two Types of Memory B Cells
      B Lymphopoiesis Before Ig Repertoire Generation
      -Development of Progenitor and Precursor Cells
      Development in Waves During Ontogeny, and in Niches Throughout Life
      Cellular Environments of the First Phase of Early, Antigen-Independent B Cell Development
      Early Commitments to Antigen-Independent B Cell Development
      Second, Eventually Autoantigen-Sensitive, Phase of B Cell Development to slgM+ Immature B Cells
      First Checkpoint for the Emerging B Cell Repertoire
      -Probing the Fitness for a Good BCR
      Expression of IgL Chains
      Second Checkpoint: Sites and Mechanisms of Selection of Newly Generated slgM+ B Cells
      Future Approaches to Understanding Central B Cell Tolerance
      Acknowledgments
      References
      10. B Cell Activation and B Cell Tolerance / Paul A. Blair
      B Cell Activation
      Antigen-Triggered Activation
      Secondary Signals for B Cell Activation
      Immediate Consequences of B Cell Activation
      B Cell Activation Requires Interaction with Helper T cells
      Cell Surface Molecules Involved in B Cell-T Cell Interactions
      Cytokines Involved in B Cell-T Cell Interactions
      Activation and Maturation of B Cells Occurs in Lymphoid Organs
      Where Do B Cells become Antigen Activated?
      Antigen Activation of B Cells Leads to the Selection of High Affinity Class-Switched Antibody
      Germinal Center
      T Cell-Independent Antibody Responses
      B Cell Tolerance: A Traditional and New Concept
      B Cell Tolerance is Acquired by at Least Three Different Processes
      Receptor Editing and Clonal Deletion
      Defective Receptor Editing Can Promote Autoimmunity
      Anergy
      Characteristics of Anergic B Cells
      Survival Factors and Tonic Signals Modulate B Cell Tolerance
      Tonic Signaling in B Cell Development
      Tonic Signaling and Autoimmunity
      BAFF and Autoimmunity
      Regulatory T cells
      Antibody-Independent Activity of B cells in Tolerance
      Cytokine Production by B Cells
      Regulatory B Cells
      Future Directions
      References
      pt. 3 Non-Antigen-Specific Recognition
      11. Role of Macrophages in Autoimmunity / Annette Pluddemann
      Introduction
      Origin and Distribution of Monocytes and Macrophages
      Recognition, Sensing, and Responses
      Phagocytosis, Antigen Presentation, and Secretion
      Activation and Downregulation: Interactions with T and B Lymphocytes
      Role of Macrophages in Adaptive Immunity and Tolerance
      Role of Macrophages in Autoimmune Models and Diseases
      Generation of Autoantigens
      Modulation of Macrophage Activation
      Conclusions and Questions
      Acknowledgments
      References
      12. Dendritic Cells in Autoimmune Disease / Meredith O'Keeffe
      Antigen Processing by Dendritic Cells
      Pattern Recognition Receptors
      Dendritic Cell Activation
      Dendritic Cell Subsets
      Mouse Dendritic Cells
      Dendritic Cells in the Mouse Thymus
      Dendritic Cell Subsets and Tolerance
      Human Dendritic Cell Subsets in Steady State
      Dendritic Cell Subsets in Human Skin: Epidermal Langerhans Cells and dermal Dendritic Cells
      Dendritic Cells and Autoimmune Disease
      Systemic Lupus Erythematosus
      IBD
      -Crohn's Disease and Ulcerative Colitis
      Dendritic Cell Immunotherapy as a Treatment for Autoimmune Diseases
      Targeting of Dendritic Cells in Autoimmune Disease
      Conclusions and Future Prospects
      References
      13. Natural Killer Cells / Hans-Gustaf Ljunggren
      Introduction to NK Cells
      NK Cell Development and Differentiation
      Phenotype and Tissue Localization of NK Cells
      Functional Responses by NK Cells
      NK Cell Receptor Signaling and Effector Functions
      NK Cell Contact and Adhesion to Target Cells
      NK Cell Lytic Granule Polarization and Maturation
      NK Cell Cytolytic Granule Exocytosis
      NK Cell Chemokine and Cytokine Production
      NK Cells and Human Autoimmunity
      Defective Control of other Immune Cells Links NK Cells to Autoimmune Diseases
      Genetic Association Studies Revealing Links between NK Cells and Autoimmune Diseases
      Conclusions
      References
      14. Granulocytes: Neutrophils, Basophils, Eosinophils / Manuel Ramos-Casals
      Neutrophils
      Basic Biology and Role in Immunity
      Neutrophil Extracellular Traps and NETosis: A Novel Form of Cell Death
      Pathogenic Role of Neutrophils in Autoimmune Diseases
      Basophils
      Basic Biology and Role in Immunity
      Basophils in Autoimmune Diseases
      Eosinophils
      Basic Biology and Role in Immunity
      Eosinophils in Churg-Strauss Syndrome
      Conclusions and Therapeutic Implications
      References
      15. Roles and Contributions of the Complement System in the Pathophysiology of Autoimmune Diseases / Robert B. Sim
      Complement System and Complement Activation Pathways
      Classical Pathway
      Lectin Pathway
      Alternative Pathway
      Membrane Attack Complex
      Contents note continued: Control of Complement Activation
      Fluid Phase Regulators
      Membrane-Bound Regulators
      Biological Effects of Complement Activation
      Complement Involvement in the Pathophysiology of Diverse Autoimmune Diseases
      References
      16. Cytokines, their Receptors and Signals / Joost J. Oppenheim
      Historical Perspective
      Cytokines and Immunity
      Cytokine Receptor Subsets
      Common γc Chain Subset
      βc Utilizing Subset
      gp130 Utilizing Subset (IL-6 Family)
      Cytokines Sharing either a p35 or p40 Ligand Chain
      Th17 Cytokines and Receptors
      Class II Cytokine Receptor Family
      Type I Interferons α and β
      Type II Interferon Gamma
      Type III Interferon Lambda
      Non-Interferon Members
      TNF Receptor Family
      IL-1/TLR Family of Receptors
      Immunosuppressive Cytokines/Growth Factors
      Chemokines
      Alarmins
      Conclusions
      Acknowledgments
      References
      pt. 4 Initiation of Autoimmunity
      17. Cellular Injury and Apoptosis / Philip L. Cohen
      Apoptosis
      History of Apoptosis
      Molecular Pathways of Apoptosis
      Apoptotic Cell Morphology
      Apoptosis in Autoimmunity
      Defective Apoptosis
      Excessive Apoptosis and Apoptotic Cells as Sources of Autoantigen
      Apotopes
      NETosis
      Necrosis
      Necroptosis
      Parthanatos {Par[poly(ADP-ribosyl)ation] and thanatos (death)}
      Clearance of Dead Cells
      Find-me Signals
      Eat-me Signals and their Receptors
      Receptors for Necrotic Cells
      Anti-Inflammatory Effects of Apoptotic Cells
      Immuno-Stimulatory Effects of Necrotic Cells
      Glimpse into the Future
      References
      18. Autophagy in Autoimmunity / Christian Munz
      Autophagy Pathways
      Autophagy in Innate Immunity
      Autophagy in Lymphocyte Development and Activation
      Antigen Presentation for CD4+ and CD8+ T Cell Recognition
      Autophagy in Tolerance and Autoimmunity
      Future Prospects
      References
      19. Infectious Triggers of T Cell Autoimmunity / Stephen D. Miller
      Introduction
      Role of Infections in Priming of Autoreactive Immune Responses
      Potential Mechanisms of Infection Triggering Autoimmunity
      Molecular Mimicry
      Bystander Activation of Autoreactive Cells and Epitope Spreading
      Emerging Mechanisms of Infection-Induced Autoimmune Disease
      Reciprocal Relationships of Pathogen-Derived Mechanisms of Autoimmunity
      How do these Mechanisms Lead to Autoimmune Disease?
      Autoimmunity Can Occur at a Site Distal to the Initiating Infection
      Conclusions
      Acknowledgments
      References
      20. Autoimmune Diseases: The Role for Vaccines / Paul-Henri Lambert
      Introduction
      Theoretical Concerns for Autoimmune Diseases in the Context of Vaccination
      Crossfire and Coincidence
      Challenges using Animal Models
      One Size does "not" Fit All
      Induced Autoimmune Disease in Animals
      Spontaneous Autoimmune Disease in Animals
      "Of Mice and Men"
      -The Correct Application of Human Epidemiology and Translation of Human Immunology to Rodent Immunology
      Practical Approach to Vaccination in Patients with Autoimmune Disease
      Reality Facing Clinicians Currently
      Certainty about Vaccines, Uncertainty about Compatibility of Administration in Certain Settings
      Search and You Will Find
      Conclusion
      References
      21. Non-infectious Environmental Agents and Autoimmunity / Frederick W. Miller
      Introduction
      Evidence Supporting the Role of Environmental Agents in Autoimmune Disease
      Identifying and Defining Environmentally Associated Autoimmune Diseases
      Non-Infectious Agents Associated with Autoimmune Diseases
      Drugs
      Occupational Exposures
      Others
      Possible Mechanisms by Which Environmental Agents May Induce Autoimmune Diseases
      Overview and Future Directions
      References
      22. Adhesion Molecules and Chemoattractants in Autoimmunity / Ulrich H. von Andrian
      Microvascular Determinants of T Cell Recruitment
      Adhesion Molecules
      Chemoattractants and their Receptors
      Multistep Adhesion Cascades
      Organized Lymphoid Tissues: Venues for Naive T Cell Homing and Dendritic Cell Interactions
      Effector T Cell Migration
      Homing to Non-Lymphoid Tissues
      Some Clinical Applications
      Conclusions and Future Directions
      Acknowledgments
      References
      pt. 5 Facilitation of Autoimmunity
      23. Effector Mechanisms in Autoimmunity / Martin Aringer
      Introduction
      Autoantibodies
      Direct Antibody-Mediated Disease
      Immune Complex Disease
      Complement Cascades
      Macrophages
      Neutrophils
      Mast Cells
      Natural Killer Cells and Cytotoxic T Cells
      Effector T Helper Cell-Mediated Autoimmune Disease
      Effector Cytokines and their Targets
      Conclusions
      References
      24. Sexual Dimorphism in the Immune System / Judith M. Greer
      Introduction
      Overview of Sexual Dimorphism
      Sexual Dimorphism in the Immune System
      Effects of Hormones on the Immune System
      Estrogens
      Progesterone
      Androgens
      Role of the Sex Chromosomes in Immunity
      X Chromosome
      Y Chromosome
      Environmental Effects on Sex Differences in Immunity
      Consequences for Autoimmunity of Sexual Dimorphism in the Immune System
      Concluding Remarks
      References
      25. Microbiome and Autoimmunity / Alicia Perez-Arroyo
      Introduction
      Intestinal Microbiome
      Intestinal Microbiome Composition and Genotyping Techniques
      Microbiome Changes Over a Lifetime
      Intestinal Microbiome Depends on the Environment
      Germ-Free (GF) Animals
      Effect of a GF Environment on Experimental Diseases
      Non-Obese Diabetic Mouse
      Experimental Allergic Encephalomyelitis
      Experimental Arthritis
      Murine Lupus
      Microbiome Composition and Autoimmune Diseases
      Type 1 Diabetes
      Rheumatoid Arthritis
      Effect of Probiotics on Autoimmune Diseases
      Probiotic Mixtures
      Specific Probiotics
      Role of Toll-like Receptor Stimulation
      Relationship Between the Intestinal Microbiome and the Hygiene Hypothesis
      Brief Overview of the Hygiene Hypothesis
      Clean Environment Promotes the Onset of Autoimmune Diabetes
      Autoimmune Diseases Can be Prevented by Infections With a Pathogen
      Synthesis and Conclusions
      Strength of the Protective Effect of Pathogens
      Ambiguous Effect on Autoimmune Disease of the GF or Axenic Status
      Delicate Interpretation of the Reduction in the Diversity of the Intestinal Microbiome
      Perspectives
      Acknowledgments
      References
      26. Genetic Predisposition, Humans / Alan G. Baxter
      Introduction
      Diseases of Interest
      Type 1 Diabetes
      Multiple Sclerosis
      Systemic Lupus Erythematosus
      Human Leukocyte Antigen (HLA) Complex and other Candidate genes
      Association of Type 1 Diabetes with HLA and other Candidate Genes
      Association of Multiple Sclerosis with HLA and other Candidate Genes
      Association of Lupus with HLA and other Candidate Genes
      Mechanisms of Complement and Fc Associations with Autoimmunity
      Mechanisms of HLA Association with Autoimmunity
      Gene Linkage Studies of Autoimmunity
      Linkage Studies of Type 1 Diabetes
      Linkage Studies in Multiple Sclerosis
      Linkage Studies in Lupus
      Genome-Wide Association Studies of Autoimmunity
      Genome-Wide Association Studies of Type 1 Diabetes
      Genome-Wide Association Studies of Multiple Sclerosis
      Genome-Wide Association Studies of Systemic Lupus Erythematosus
      From Location to Molecular Mechanisms
      Concluding Comments
      Acknowledgments
      References
      27. Genetic Predisposition to Autoimmune Diseases Conferred by the Major Histocompatibility Complex: Utility of Animal Models / Chella S. David
      Major Histocompatibility Complex
      MHC and Autoimmunity
      Mystery of HLA-B27 and Spondyloarthropathies
      HLA Class II Association with Autoimmune Diseases
      Predisposition
      Onset
      Environmental Factors
      Infectious Agent
      Porphyromonas Gingivalis and Rheumatoid Arthritis
      Smoking and Autoimmunity
      Vitamin D in Autoimmune Diseases
      Post-Translational Modifications in Autoimmunity
      Deimination
      Deamidation
      Humanized Animal Models of Autoimmunity
      Collagen-Induced Arthritis
      Non-RA-Associated HLA Alleles Can Predispose to Autoimmunity
      HLA-DR Transgenic Mice with EAE as an Animal Model of MS
      Role of DQ Molecules in Predisposition to MS
      Animal Model of Celiac Disease
      Animal Model for Type 1 Diabetes
      HLA Class II Molecules Regulate Infection Through Modulation of Cytokine Networks
      Concluding Remarks
      References
      28. Epigenetics and Autoimmune Diseases / Moncef Zouali
      Epigenetic Modifications
      DNA Methylation
      Histone Post-Translational Modifications
      Acetylation and Deacetylation
      Histone Methylation
      Arginine Methylation
      Ubiquitination
      MicroRNAs
      Epigenetic Stability
      Epigenetics of Immune Tolerance to Self
      Epigenetic Regulators of Tolerant T Cells
      Role of CpG DNA Methylation in Treg Development and Function
      Impacts of Histone Acetylation on Development and Function of Tregs
      Epigenetic Modulation of Treg Stability
      Systemic Lupus Erythematosus
      DNA Methylation
      DNA Methylation Control by miRNA
      Histone Acetylation
      Environmental Epigenetics in SLE
      Epigenetic Disruption of B Cell Tolerance in SLE
      Rheumatoid Arthritis
      Acetylation Marks
      Genomic DNA Hypomethylation and the Activated Phenotype of RASFs
      miRNA and the Destructive Potential of RASFs
      Aberrant SUMOylation
      Systemic Sclerosis
      Sjogren's Syndrome
      Antineutrophil Cytoplasmic Autoantibodies-Associated Vasculitis
      Contents note continued: Type 1 Diabetes
      DNA Methylation Profiling
      Chromatin Remodeling and Histone Acetylation
      Histone Deacetylase Inhibitors in Preclinical Studies
      Multiple Sclerosis
      Generation of Neo-Epitopes
      DNA Hypomethylation
      Deregulation of Acetylation Homeostasis
      Epigenetic Therapy
      Targeting DNA Methylation
      Histone Deacetylase Inhibitors
      Epigenetic Generation of Tregs
      Future Prospects
      References
      29. Autoimmunity in Primary Immunodeficiency Disorders / Arnold I. Levinson
      Introduction
      T Cell Developmental Defects
      Severe Combined Immunodeficiency
      Combined Immune Deficiencies
      DiGeorge Syndrome
      Wiskott-Aldrich Syndrome
      Antibody Production Defects
      X-Linked Agammaglobulinemia
      Common Variable Immunodeficiency
      Selective IgA Deficiency
      Immunodeficiency with Hyper-IgM Syndrome
      Innate Immune Defects
      Chronic Granulomatous Disease
      Early Complement Component Deficiency
      Toll-Like Receptor 3 (TLR3) Polymorphism and Autoimmunity
      Monogenic Defects Affecting Immune Homeostasis and/or Tolerance
      Autoimmune Polyendocrinopathy, Candidiasis, Ectodermal Dystrophy
      Immunodysregulation, Polyendocrinopathy, Enteropathy X-Linked
      Autoimmune Lymphoproliferative Syndrome
      Conclusion
      Acknowledgment
      References
      pt. 6 Experimental Models of Autoimmunity
      30. Animal Models: Systemic Autoimmune Diseases / George C. Tsokos
      Introduction
      Spontaneous Models of Systemic Autoimmunity
      Genetically Manipulated Models of Systemic Autoimmunity
      Lymphocyte Activation Molecules
      Ubiquitination-Protein Ligases
      Cytokines and their Receptors
      Complement and Complement Receptor Proteins
      Clearance of Dead Cells
      Innate Immune Cell Signaling
      Induced Models of Systemic Autoimmunity
      Concluding Comments
      References
      31. Animal Models of Organ-Specific Autoimmune Disease / Matthias von Herrath
      What Can Animal Models Teach us about Organ-Specific Autoimmunity?
      Inciting Autoantigen
      Antigen-specific Tolerization Strategies
      Other Immune Therapies
      Gene Function
      Understanding the Complexity of Organ-Specific Autoimmunity
      Animal Models
      -Advantages and Disadvantages
      Animal Models for Organ-Specific Autoimmune diseases
      Spontaneous Models of Organ-Specific Autoimmunity
      Genetically Engineered Animal Models for Organ-Specific Autoimmunity
      Induced Animal Models for Organ-Specific Autoimmune Diseases
      Protein/Peptide and Adjuvant
      Cell Transfer or Depletion
      Comparison of Organ-Specific Autoimmune Disease in Animal Models and Humans
      Conclusions
      References
      pt. 7 Multisystem Autoimmune Diseases
      32. Systemic Lupus Erythematosus / Robert G. Lahita
      Introduction
      Pathogenesis
      Genetics
      Epidemiology
      Autoantibody
      Clinical
      Measurement of Clinical Activity
      Musculoskeletal
      Renal
      Neuropsychiatric
      Cardiac
      Pulmonary
      Hematology
      Dermatology
      Gastroenterological
      ENT and Eye
      Lupus Therapeutics
      References
      33. Systemic Sclerosis, Scleroderma / Daniel E. Furst
      Introduction
      Clinical, Pathologic, and Epidemiologic Features
      Clinical Features
      Pathologic Features
      Epidemiologic Features
      Autoimmune Features and Immunologic Markers in Disease
      Potential Pathogenetic Antibodies
      Diagnostic and Prognostic Antibodies
      Genetic Features
      Environmental Influences
      Animal Models of SSc
      UCD200 Chickens
      Tsk1 and Tsk2 Mice
      Scl-GvHD
      TGF-β, CTGF, and bFGF
      Bleomycin-Induced Fibrosis
      Fra2-Transgenic Mice
      TβRIIΔk Mice
      Wnt-10b-Transgenic Mice
      Pathogenic Mechanisms
      Vasculopathy
      Immunological Abnormalities
      Fibrosis
      Concluding Remarks and Future Prospects
      References
      34. Antiphospholipid Syndrome / Yehuda Shoenfeld
      Introduction
      Clinical Spectrum of Antiphospholipid Syndrome
      Obstetric APS
      Thrombotic APS
      Neurological APS
      Hematologic APS
      Dermatologic APS
      Cardiac APS
      Pulmonary APS
      Renal APS
      Catastrophic Antiphospholipid Syndrome
      Antiphospholipid Antibodies
      b2GPI-Anti-b2GPI Complex in APS
      Non-Criteria aPL Antibodies
      aPL of the IGA Isotype
      Low-level aPL
      Mechanisms of aPL-Mediated Disease Expressions
      Thrombotic Manifestations
      Obstetric Manifestations
      Neurologic Manifestations
      Complement System in APS
      Treatment of APS
      Conclusions and Future Aspects
      References
      35. Sjogren's Syndrome / Haralampos M. Moutsopoulos
      Introduction
      Clinical Features
      Local Manifestations (Salivary and Lachrymal Glands)
      Systemic Manifestations (Beyond Salivary and Lachrymal Glands)
      Overlapping Autoimmune Entities
      Diagnosis and Differential Diagnosis
      Autoimmune Features and Pathogenic Mechanisms
      Immunopathology
      Autoantibodies
      Etiopathogenesis
      Genetics
      Epigenetics
      Environmental Factors
      Exocrine Gland Dysfunction
      Apoptosis
      Neurotransmission
      Aquaporins
      Structural Abnormalities
      Therapy
      Future Prospects
      References
      36. Rheumatoid Arthritis / Kurt Redlich
      Introduction
      Clinical, Pathologic, and Epidemiologic Features
      Autoimmune Features
      Genetic Features
      In Vivo Models
      Pathologic Effector Mechanisms
      Autoantibodies as Potential Immunologic Markers
      Concluding Remarks
      -Future Prospects
      Acknowledgment
      References
      37. Juvenile Idiopathic Arthritis / Alberto Martini
      Epidemiology
      Clinical Features
      Systemic Arthritis
      Rheumatoid Factor-Positive Polyarthritis
      Enthesitis-Related Arthritis
      Oligoarthritis
      Rheumatoid Factor-Negative Polyarthritis
      Psoriatic Arthritis
      Undifferentiated Arthritis
      Perspectives
      Etiology and Pathogenesis
      Systemic Juvenile Idiopathic Arthritis
      Oligoarticular Juvenile Idiopathic Arthritis
      Treatment
      References
      38. Spondyloarthritides / Joachim Sieper
      Definition, Epidemiology, Clinical Manifestations, and Treatment
      Reactive Arthritis
      Arthritis with IBD
      Psoriatic Arthritis
      Role of HLA-B27 in the Pathogenesis of Spondyloarthritis
      Arthritogenic Peptide Hypothesis
      Misfolded HLA-B27 Hypothesis
      Role of Non-MHC Genes in Spondyloarthritis
      Bacterial Trigger and Autoimmunity in the Pathogenesis of the Spondyloarthritides
      Cytokines in the Pathogenesis of Reactive Arthritis
      Cytokines in the Pathogenesis of Ankylosing Spondylitis
      What is the Immune Target in Ankylosing Spondylitis?
      Inflammation and Bone Formation
      Concluding Remarks
      -Future Prospects
      References
      39. Autoimmune Myopathies / Antony Rosen
      Defining Autoimmune Myopathies
      Clinical and Pathological Descriptions of Different Phenotypes, including IMNM
      Characteristic Pathology, but Significant Overlap between Phenotypes
      Epidemiological Clues into Mechanism
      Specific Autoantibodies are Strongly Associated with Phenotype, Making them Useful Probes of Disease Mechanism
      Myositis-Specific Autoantibodies
      HMG CoA Reductase Autoantibodies in Statin-Associated Immune-Mediated Necrotizing Myopathy
      Mechanisms of Disease
      Association of Malignancy with Myositis: Insights into Disease Initiation
      Enhanced Expression of Myositis Autoantigens in Regenerating Muscle Cells to Focus Propagation on Muscle
      Modification of Autoantigen Expression or Structure by Immune Effector Pathways to Generate a Self-Sustaining Phenotype
      Therapeutic Insights
      Concluding Remarks
      References
      pt. 8 Endocrine System
      40. Thyroid Disease / Anthony P. Weetman
      Autoimmune Thyroiditis
      Historic Background
      Clinical, Pathologic, and Epidemiologic Features
      Autoimmune Features
      Genetic Features
      Environmental Influences
      In Vivo Models
      Pathologic Effector Mechanisms
      Autoantibodies as Potential Immunological Markers
      Treatment and Outcome
      Concluding Remarks
      -Future Prospects
      Graves' Disease
      Historic Background
      Clinical, Pathologic, and Epidemiologic Features
      Autoimmune Features
      Genetic Features
      Environmental Influences
      In Vivo Models
      Pathologic Effector Mechanisms
      Autoantibodies as Potential Immunological Markers
      Treatment and Outcome
      Thyroid-Associated Ophthalmopathy and Dermopathy
      Concluding Remarks
      -Future Prospects
      References
      41. Autoimmune (Type 1) Diabetes / Ake Lernmark
      Introduction
      Clinical and Pathologic Features
      Epidemiologic Features
      Genetic Features
      HLA Genetic Factors: The DR-DQ Alleles
      Non-HLA Genetic Factors
      Autoimmune Features
      Pathologic Effector Mechanisms
      Triggering Autoimmunity
      APCs in Genetically Predisposed Subjects
      In Vivo and In Vitro Models
      Autoantibodies as Potential Immunologic Markers
      Concluding Remarks
      -Future Prospects
      References
      42. Adrenalitis / Renato Zanchetta
      Introduction
      Anatomy and Physiology of the Adrenals
      Epidemiology of Addison's Disease and Autoimmune Adrenalitis
      Autoimmune Addison's Disease (AAD)
      Histopathology
      Focal Lymphocytic Adrenalitis
      Diffuse Lymphocytic Adrenalitis
      Animal Models
      Induced Immunity
      Spontaneous Animal Models of AD
      Immunologic Studies
      Genetic Predisposition
      Cellular Immunity
      Humoral Immunity
      Natural History of AAD
      Diagnosis of AAD
      Clinical Manifestations
      Hormonal Tests
      Imaging
      Different Clinical Presentations of AAD
      Association with other Autoimmune Disorders
      Therapy
      Contents note continued: General Information
      Acknowledgments
      References
      43. Polyendocrine Syndromes / Eystein S. Husebye
      Historical Background
      Clinical, Pathologic and Epidemiologic Features
      Autoimmune Features
      Genetic Features
      Environmental Features
      Animal Models
      AIRE-Deficient Mouse as a Model for APS-1
      Spontaneous Animal Models
      Thymectomy Animal Model
      Pathogenic Mechanisms
      Immunologic Markers in Diagnosis
      Treatment and Outcome
      Concluding Remarks
      -Future Prospects
      Acknowledgments
      References
      44. Autoimmune Gastritis and Pernicious Anemia / Paul A. Gleeson
      Clinical, Pathologic, and Epidemiologic Features
      Autoimmune Features
      Autoantibodies
      T Cell Immunity
      Genetic Features
      In Vivo and In Vitro Models
      Pathologic Effector Mechanisms
      Autoantibodies as Potential Immunologic Markers
      Concluding Remarks
      -Future Prospects
      References
      45. Autoimmune Hypophysitis / Angelika Gutenberg
      Definition and Classification of Autoimmune Hypophysitis
      Historical Background
      Epidemiology and Body of Literature
      Clinical Features
      Pathological Features
      Autoimmune Features
      Genetic and Environmental Influences
      Animal Models
      Diagnosis
      Treatment
      Outcome
      Hypophysitis Secondary to CTLA-4 Blockade
      Concluding Remarks
      -Future Perspectives
      Acknowledgments
      References
      pt. 9 Blood Disorders
      46. Autoimmune Hemolytic Anemia / Robert N. Barker
      Historical Background
      Classification of AIHA
      Animal Models of AIHA
      Mechanisms of RBC Destruction in AIHA
      Cold Reactive Antibodies
      Warm Reactive Antibodies
      Pathogenicity of Warm Reactive IgG Antibodies
      Additional Mechanisms of Hemolysis by Warm Antibodies
      RBC Autoantigens
      Clinical Signs of AIHA
      Laboratory Diagnosis of AIHA
      Treatment of AIHA
      Etiology of AIHA and Predisposing Factors
      Genetic Predisposition
      Gender and Age
      Infectious Agents
      Drugs
      Neoplasia
      Immune Mechanisms Underlying Loss of Self Tolerance in Warm AIHA
      B Cells and Tolerance
      T Helper (Th) Cells and Tolerance
      Concluding Remarks
      References
      47. Immune Thrombocytopenia / James B. Bussel
      Clinical, Pathologic, and Epidemiologic Features
      General Features and Definitions
      Diagnosis
      Epidemiology and Clinical Presentation in Children
      Epidemiology and Clinical Presentation in Adults
      Autoimmune Features
      Autoimmune Markers in Primary ITP
      Evans Syndrome
      ITP Secondary to Systemic Lupus Erythematosus
      ITP Secondary to Primary Immunodeficiencies
      Genetics Features
      Familial ITP
      Genetic Markers of ITP
      In Vivo Models
      Pathologic Effector Mechanisms
      Increased Platelet Destruction
      Insufficient Platelet Production
      Infection-related Thrombocytopenia
      Autoantibodies as Potential Immunologic Markers
      Concluding Remarks
      -Future Prospects
      References
      48. Autoimmune Neutropenia / Parviz Lalezari
      Historical Background
      Clinical and Pathologic Features
      Autoimmune Neutropenia of Infancy
      Primary Autoimmune Neutropenia in Adolescents and Adults
      Neutrophil-Specific Antigens in Primary Autoimmune Neutropenias
      Secondary Autoimmune Neutropenias
      Differential Diagnosis
      Mechanisms of Cell Destruction
      Laboratory Diagnosis
      Treatment
      Perspectives and Future Directions
      References
      49. Acquired Aplastic Anemia / Richard J. Jones
      Historical Background
      Genetic Features
      Clinical, Pathologic, and Epidemiologic Features
      Autoimmune Features and Pathogenic Mechanisms
      Environmental Features
      Animal Models
      Therapy for Aplastic Anemia
      Bone Marrow Transplantation (BMT)
      BMT from Unrelated Donors
      Immunosuppressive Therapy
      High-Dose Cyclophosphamide without BMT
      Aplastic Anemia and Clonality
      Concluding Remarks
      -Future Prospects
      References
      50. Monogenic Autoimmune Lymphoproliferative Syndromes / Michael Lenardo
      Introduction
      -Apoptosis and the Immune System
      Clinical and Pathological Features
      Autoimmune Lymphoproliferative Syndrome
      Clinical Presentation
      Laboratory Evaluation
      Imaging Studies
      Treatment
      Prognosis
      ALPS-Related Disorders
      Caspase-8 and FADD Deficiencies
      RAS-Associated Autoimmune Leukoproliferative Disorder
      Genetic Features
      Autoimmune Lymphoproliferative Syndrome (ALPS)
      Germline FAS Mutations
      Somatic FAS Mutations
      Additional Genetic Etiologies
      RAS-Associated Autoimmune Leukoproliferative Disorder
      In Vivo and In Vitro Models of Disease
      Pathogenic Effector Mechanisms
      Conclusion
      Acknowledgments
      References
      51. Autoimmune Clotting Dysfunction / Christoph Konigs
      Introduction
      Procoagulant Thrombotic Diseases
      Autoimmune Inhibitors to ADAMTS13
      Anticoagulant (Bleeding) Diseases
      Autoimmune Antibody Inhibitors to Fibrinogen (Factor I) and Fibrin
      Autoimmune Inhibitors to Prothrombin (Factor II) and Thrombin
      Autoimmune Inhibitors to Factor V
      Autoimmune Inhibitors to Factor VII
      Autoimmune Inhibitors to Factor VIII
      Autoimmune Inhibitors to Factor IX
      Autoimmune Inhibitors to Factor X
      Autoimmune Inhibitors to Factor XI
      Autoimmune Inhibitors to Factor XII
      Autoimmune Inhibitors to Factor XIII
      Autoimmune Inhibitors to Von Willebrand Factor
      Autoimmune Inhibitors to Further Proteins
      Conclusions and Future Prospects
      Acknowledgments
      References
      pt. 10 Central and Peripheral Nervous System
      52. Multiple Sclerosis / David A. Hafler
      Historical Background
      Clinical Features
      Imaging
      Immunological Markers in Diagnosis
      Pathology
      Epidemiology of MS
      Genetic Factors
      Environmental Factors
      Immune Pathogenesis
      T Cell Pathogenesis
      Immune Dysregulation
      Autoantigens
      Meningeal Ectopic B Cell Follicles
      Treatment
      Interferons
      Glatiramer Acetate (Copaxone®)
      Natalizumab (Tysabri®)
      Mitoxantrone (Novantrone®)
      Fingolimod (Gilenya®)
      Teriflunomide (Aubagio®)
      Dimethyl Fumarate, BG-12 (Tecfidera®)
      Concluding Remarks
      References
      53. Peripheral Neuropathies / Kazim A. Sheikh
      Introduction
      Acute Neuropathies: The Guillain
      Barre Syndrome
      Historical Background
      Epidemiology
      Clinical Features and Subtypes of GBS
      Autoimmune Features
      Environmental Effects
      Animal Models of Disease
      Cellular Mechanisms
      Cellular and Humoral Immune Elements are Synergistic
      Genetic Aspects of GBS
      Treatment and Outcomes
      Chronic Neuropathies: Chronic Inflammatory Demyelinating Polyradiculoneuropathy
      History
      Epidemiology and Clinical Features
      Autoimmune Features
      Immunogenetic Features
      Environmental Influences
      Animal Models
      Pathogenic Mechanisms
      Treatment and Outcome
      Concluding Remarks and Future Prospects
      Acknowledgments
      References
      54. Myasthenia Gravis and Related Disorders / Angela Vincent
      Introduction
      Neuromuscular Junction
      AChR, MuSK, and Lrp4
      Neuromuscular Transmission
      Myasthenia Gravis
      Epidemiology
      Etiology
      General Aspects
      Clinical Heterogeneity
      Early-onset AChR Antibody Positive MG (AChRab+ MG)
      Late-onset AChR Antibody Positive MG (AChRab+ MG)
      Thymoma-Associated MG (Thymoma MG)
      MuSK Antibody-Positive MG (MuSKab+ MG)
      Neonatal MG
      Antibodies in Myasthenia
      Serological Testing
      AChR Antibody Characteristics
      MuSK and Other Antibodies
      Pathogenic Mechanisms
      Evidence for Pathogenicity of AChR and MuSK Antibodies
      AChR Antibody-Positive MG
      MuSK Antibody-Positive MG
      Thymus and Cellular Immunity in MG
      Role of T Lymphocytes in MG
      Thymus and MG
      Lambert
      Eaton Myasthenic Syndrome
      Epidemiology and Etiology
      Clinical Features
      Investigation and Treatment
      Pathophysiology
      Conclusions and Future Prospects
      References
      55. Ocular Disease / Robert B. Nussenblatt
      Historical Background
      Clinical Features
      Pathologic Features
      Epidemiologic Features
      Autoimmune Features
      Hormonal Influences
      Genetic Factors
      Animal Models
      Pathogenic Mechanisms
      Ocular Immune Responses
      Tissue Destruction
      Immunologic Markers
      Treatment and Outcomes
      Concluding Remarks and Future Prospects
      References
      56. Immune-Mediated Inner Ear Disease / Antonio Puccetti
      Introduction
      Clinical Features
      IMIED Associated with Systemic Autoimmune Diseases
      IMIED Associated with Primary Vasculitides
      Evidence of Autoimmunity
      Genetic Susceptibility
      Animal Models
      Treatment
      Concluding Remarks and Future Perspectives
      Acknowledgments
      References
      57. Encephalomyelopathies / Eric Lancaster
      Introduction
      Neurological Syndromes of Autoimmune Causation
      Cerebral Syndromes
      Ataxia
      Spinal Myelitis
      Stiff Person Syndrome
      Systemic Immunopathic Disorders with Encephalitis and Myelitis
      Systemic Lupus Erythematosus
      Sarcoidosis
      Sjogren's Syndrome
      Behcet's Disease
      Diseases with Autoantibodies to Cell-surface Channels, Receptors
      Neuromyelitis Optica (NMO; Devic's disease)
      Encephalitis with Antibodies to N-methyl-d-aspartate receptor (NMDAR)
      Anti-AMPAR
      Anti-GABA-B-R
      Group 1 Metabotropic Glutamate Receptors (mGluR1, mGluR5)
      Glycine Receptor (GlyR)
      Voltage-Gated Potassium Channel Complex (VGKC)
      LGI1
      Caspr2
      PCA-Tr
      CNS Diseases with Autoantibodies to Intracellular Antigens
      GAD65
      Contents note continued: Amphiphysin I
      Neuronal nuclear antigens (NNA)
      Conclusions and Future Prospects
      Acknowledgments
      Abbreviations
      References
      58. Paraneoplastic Neurologic Syndromes / Jerome B. Posner
      Introduction
      Pathogenesis
      Diagnosis
      Antibodies
      Nuclear/Nucleolar Antibodies
      Cytoplasmic Antibodies
      Synaptic/Cell Surface Antibodies
      Ion Channel Antibodies
      Treatment
      Clinical Syndromes
      Paraneoplastic Cerebellar Degeneration
      Paraneoplastic Encephalomyelitis
      Paraneoplastic Limbic Encephalitis
      Brainstem Encephalitis
      Myelitis
      Subacute Sensory Neuronopathy
      Autonomic Neuropathy
      Vision Loss
      Retinopathy
      Optic Neuropathy
      Stiff Person Syndrome
      Lambert-Eaton Myasthenic Syndrome
      References
      pt. 11 Gastrointestinal System
      59. Celiac Disease / Knut E.A. Lundin
      Clinical, Pathologic, and Epidemiologic Features
      Clinical Features and Associated Disorders
      Pathology of the Intestinal Lesion
      Epidemiology
      Autoimmune Features
      Autoantibodies
      Autoreactive Intraepithelial Lymphocytes (IELs)
      Genetic Features
      HLA Genes
      Non-HLA Genes
      Environmental Influences
      Gluten Proteins
      Other Environmental Factors
      In Vivo and In Vitro Models
      Animal Models
      Organ Culture Assays
      Pathogenic Mechanisms
      Gluten-reactive CD4+ T Cells
      Role of Transglutaminase 2
      Gluten Antigen Presentation by Disease-associated DQ Molecules
      Mucosal Antigen-presenting Cells
      Effector Mechanisms Leading to Mucosal Alterations
      Autoantibodies as Potential Immunologic Markers
      Serology
      Staining of Immune Complexes
      Treatment and Outcome
      Current Treatment and Outcome
      Novel Therapeutic Options
      Concluding Remarks
      -Future Prospects
      Acknowledgments
      References
      60. Inflammatory Bowel Diseases / Lloyd Mayer
      Introduction
      History
      Epidemiology and Environmental Factors
      Clinical and Pathologic Features
      Disease Presentation
      Pathology
      Genetics
      Immunopathogenesis
      Epithelial Barrier and Innate Immunity
      Adaptive Immunity
      Host-Microbial Interactions
      Biomarkers
      Fecal Markers
      Serologic Markers
      Treatment
      Medical
      Surgical
      Future Prospects
      References
      61. Hepatitis / Giorgina Mieli-Vergani
      Clinical, Pathologic, and Epidemiologic Features
      Clinical Features
      Pathologic Features
      Epidemiologic Features
      Autoimmune Features
      Autoantibodies as Potential Immunologic Markers
      Genetic Features
      Animal Models
      Pathologic Effector Mechanisms
      Treatment and Outcome
      Standard Treatment
      Alternative Treatments
      Duration of Treatment
      Liver Transplantation
      Future Treatment Approaches
      Concluding Remarks
      -Future Prospects
      References
      62. Primary Biliary Cirrhosis / M. Eric Gershwin
      Clinical, Pathologic, and Epidemiologic Features
      History
      Diagnosis
      Pathology
      Clinical Features
      Epidemiology and Natural History
      Treatment
      Autoimmune Features
      Genetic Features
      Familial PBC: Twins and Relatives
      HLA Association
      Genome-wide Association Studies (GWAS)
      Epigenetic Effects
      Fetal Microchimerism
      Genes on the X Chromosome
      Environmental Provocation of PBC
      Infections
      Xenobiotics
      Experimental Animal Models
      Pathologic Effector Mechanisms
      Autoantibodies as Potential Immunologic Markers
      Concluding Remarks
      -Future Prospects
      References
      63. Primary Sclerosing Cholangitis / Keith D. Lindor
      Epidemiology and Risk Factors
      Epidemiologic Features
      Risk Factors
      Natural History, Clinical Features, and PSC-IBD
      Natural History and Clinical Features
      PSC-IBD
      Diagnosis
      Biochemical Features
      Cholangiography
      Histology
      PSC Subtypes and Pediatric PSC
      Small Duct PSC
      PSC-AIH
      Pediatric PSC
      PSC-Associated Malignancies
      Colorectal Neoplasia
      Cholangiocarcinoma (CCA)
      Gallbladder Neoplasia
      Hepatocellular Carcinoma
      Genetics
      Animal Models
      Overview
      Summary of PSC Models
      Potential Pathogenic Mechanisms
      Overview
      Bacterial Translocation, Pathogen-associated Molecular Patterns, and Innate Immune Response
      Adhesion Molecules, Lymphocyte Homing, and the Liver-Gut Axis
      Antibodies, Memory, and Regulatory T cells
      Transporter Defects and Bile Acids
      Autoimmune Features
      Treatment
      Medical Therapy
      Therapeutic Endoscopy
      Liver Transplantation
      Concluding Remarks
      References
      64. Autoimmune Pancreatitis and IgG4-related Disease / Kendo Kiyosawa
      Historical Background
      Autoimmune Pancreatitis - Clinical, Pathologic, and Epidemiological Features
      Clinical Features
      Extra-pancreatic Lesions
      Pathological Features
      Epidemiologic Features
      Autoimmune Features
      Genetic Features
      Animal Models
      Pathological Mechanisms
      Immunological Markers in Diagnosis
      Treatment and Outcome
      IgG4-related Disease
      Definition
      Historical Background
      Epidemiology
      Clinical Features of IgG4-relate4 Disease
      Definite IgG4-related Diseases
      Possible IgG4-related Diseases
      Pathological Features
      Autoimmune Features, Genetic Features, Animal Models, and Pathological Mechanisms
      Diagnosis
      Treatment
      Concluding Remarks: Future Prospects
      References
      pt. 12 Skin Diseases
      65. Autoimmune Bullous Skin Diseases-Pemphigus and Pemphigoid / Luis A. Diaz
      Introduction
      Pemphigus Vulgaris
      Clinical, Pathologic, and Epidemiologic Features
      Autoimmune Features
      Genetic Features
      In Vivo and In Vitro Models
      Pathologic Effector Mechanisms
      Autoantibodies as Potential Immunologic Markers
      Pemphigus Foliaceus
      Clinical, Pathologic, and Epidemiologic Features
      Autoimmune Features
      Genetic Features
      In Vivo and In Vitro Models
      Pathologic Effector Mechanisms
      Autoantibodies as Potential Immunologic Markers
      Environmental Factors involved in Fogo Selvagem
      Other Types of Pemphigus
      Paraneoplastic Pemphigus
      Drug-induced Pemphigus
      IgA Pemphigus
      Bullous Pemphigoid
      Clinical, Pathologic, and Epidemiologic Features
      Autoimmune Features
      Genetic Features
      In Vivo and In Vitro Models
      Pathologic Effector Mechanisms
      Autoantibodies as Potential Immunologic Markers
      Other Subepidermal Bullous Diseases
      Herpes Gestationis (Pemphigoid Gestationis)
      Cicatricial Pemphigoid
      Linear IgA Disease
      Epidermolysis Bullosa Acquisita
      Dermatitis Herpetiformis
      Treatment of Autoimmune Bullous Diseases
      Concluding Remarks
      References
      66. Non-bullous Skin Diseases: Alopecia Areata, Vitiligo, Psoriasis, and Urticaria / David A. Norris
      Alopecia Areata
      Clinical, Pathologic, and Epidemiologic Features
      Autoimmune Features
      Genetic Features
      In Vivo and In Vitro Models
      Pathologic Effector Mechanisms
      Autoantibodies as Potential Immunologic Markers
      Concluding Remarks
      -Future Prospects
      Vitiligo
      Clinical, Pathologic, and Epidemiologic Features
      Autoimmune Features
      Genetic Features
      In Vivo and In Vitro Models
      Pathogenetic Mechanism
      Autoantibodies as Potential Immunologic Markers
      Concluding Remarks
      -Future Prospects
      Psoriasis
      Clinical, Pathologic, and Epidemiologic Features
      Autoimmune Features
      Genetic Features
      In Vivo and In Vitro Models
      Pathogenic Mechanism
      Autoantibodies as Potential Immunologic Markers
      Concluding Remarks
      -Future Prospects
      Chronic Urticaria
      Clinical, Pathologic, and Epidemiologic Features
      Autoimmune Features
      Genetic Features
      In Vivo and In Vitro Models
      Pathologic Effector Mechanisms
      Autoantibodies as Potential Immunologic Markers
      Concluding Remarks
      -Future Prospects
      References
      pt. 13 Nephropathies and Reproductive System
      67. Autoimmune Disease in the Kidney / Ronald J. Falk
      Introduction
      Are there Hallmarks of Autoimmune Disease?
      Autoantibodies and Their Antigens
      Hallmarks of Autoimmune Diseases of the Kidney
      Autoreactive T and B Cells Evade Deletion
      Pre-existence of Asymptomatic "Normal" Autoantibodies
      Hyperactivity of Fc-FcR Pathway
      Antigenic Alterations of "Self" Proteins
      Susceptibility to Environmental Impacts
      Microbial Infections
      Summary
      Future Directions
      References
      68. Autoimmune Orchitis and Autoimmune Oophoritis / Kenneth S.K. Tung
      Introduction
      Experimental Autoimmune Disease of the Testis
      Autoimmune Orchitis in the Dark Mink
      Autoimmune Orchitis in Rats Expressing Transgenic Human HLA B27 and Human β2 Microglobulin
      Post-vasectomy Autoimmune Orchitis in Mice with Treg Depletion and Post-vasectomy Tolerance to Testis Antigens
      Autoimmune Orchitis Associated with Viral Infection
      Autoimmune Orchitis in Day 3 Thymectomized (d3tx) Mice
      Classical Experimental Autoimmune Orchitis Induced by Immunization with Testis Antigen in Adjuvant
      Clinical Autoimmune Disease of the Testis
      Idiopathic Male Infertility
      Infertility and ASA Coexist with Other Autoimmune Diseases
      Vasectomy, Sperm Granuloma, and Cystic Fibrosis
      Orchitis Associated with Virus Infections
      Experimental Autoimmune Disease of the Ovary
      Spontaneous Autoimmune Ovarian Disease (AOD) in AIRE Null Mice
      AOD in Day 3 Thymectomized (d3tx) Mice
      AOD in Neonatal Mice by Maternal Antibody to Murine ZP3
      Classical Experimental Autoimmune Oophoritis
      Clinical Autoimmune Disease of the Ovary
      Contents note continued: Concluding Remarks
      Acknowledgments
      References
      pt. 14 Cardiovascular System and Lungs
      69. Rheumatic Fever and Rheumatic Heart Disease / J. Kalil
      Clinical, Pathological, and Epidemiologic Features
      Autoimmune Features
      Genetic Features
      Innate Immune Response
      Adaptive Immune Response
      Both Innate and Adaptive Immune Response
      In Vivo and In Vitro Models
      In Vivo Model of Myocarditis and Valvulitis
      In Vitro Model of Rheumatic Heart Disease Autoimmune Reactions
      Pathologic Effector Mechanisms
      Autoantibodies as Potential Immunologic Markers
      Concluding Remarks
      -Future Prospects
      References
      70. Myocarditis and Dilated Cardiomyopathy / Ziya Kaya
      Historical Background
      Clinical, Pathologic, and Epidemiologic Features
      Myocarditis
      Dilated Cardiomyopathy
      Autoimmune Features and Immunologic Markers
      Circulating Antibodies
      Immunofluorescence
      Western Immunoblot
      Immunoassay with Defined Antigens
      Immunologic Assessment of Biopsies
      Genetic Features
      Environmental Features
      Animal Models and Pathogenic Mechanisms
      Treatment and Outcome
      Personal Thoughts
      Acknowledgments
      References
      71. Atherosclerosis / Alex Bobik
      Introduction
      Development of Atherosclerotic Lesions
      Mouse Models of Atherosclerosis
      Culprit Autoantigens
      Oxidized Low-density Lipoprotein (oxLDL)
      Heat Shock Protein 60 (HSP60)
      β2 Glycoprotein 1 (β2-GP1)
      Immune Responses in Atherosclerosis
      Innate Immunity in Atherosclerosis
      Adaptive Immunity in Atherosclerosis
      Other Key Cellular Players
      Immune System Activation
      Experimental Therapeutics: A Protective "Vaccine" for Atherosclerosis
      Implications for Clinical Translation
      References
      72. Necrotizing Arteritis and Small Vessel Vasculitis / Ronald J. Falk
      Historical Background
      Necrotizing Arteritis
      Purpura and Small Vessel Vasculitis
      Polyarteritis Nodosa
      Clinical, Epidemiologic, and Pathologic Features
      Autoimmune Features
      Genetic Features and Environmental Influences
      In Vivo Models
      Pathologic Effector Mechanisms
      Autoantibodies as Potential Immunologic Markers
      Kawasaki's Disease
      Clinical, Pathologic, and Epidemiologic Features
      Autoimmune Features
      Genetic Features and Environmental Influences
      In Vivo Models
      Pathologic Effector Mechanisms
      Autoantibodies as Potential Immunologic Markers
      ANCA-Associated Vasculitis (AAV)
      Clinical, Pathologic, and Epidemiologic Features
      Autoimmune Features
      Genetic Features
      Environmental Influences
      In Vivo Models
      Pathologic Effector Mechanisms
      Autoantibodies as Immunologic Markers
      Cryoglobulinemic Vasculitis
      Clinical, Pathologic, and Epidemiologic Features
      Autoimmune Features
      Genetic Features and Environmental Influences
      In Vivo Models
      Pathologic Effector Mechanisms
      Autoantibodies as Immunologic Markers
      IgA Vasculitis (Henoch
      -Schonlein Purpura)
      Clinical, Pathologic, and Epidemiologic Features
      Autoimmune Features
      Genetic Features and Environmental Influences
      In Vivo Models
      Pathologic Effector Mechanisms
      Autoantibodies as Potential Immunologic Markers
      Concluding Remarks
      -Future Prospects
      References
      73. Large and Medium Vessel Vasculitides / Jorg J. Goronzy
      Vasculitides of Large and Medium-sized Blood Vessels
      Giant Cell Arteritis
      Historical Background
      Clinical, Pathologic, and Epidemiologic Features
      Vascular Legion
      Epidemiology
      Genetic Features
      Pathogenic Mechanisms
      T Cells and Antigen-Presenting Cells in Giant Cell Arteritis
      Macrophages in Giant Cell Arteritis
      Intimal Hyperplasia
      Immuno-stromal Interactions Promoting Vasculitis
      Systemic Inflammatory Syndrome
      Treatment, Monitoring, and Outcome
      Takayasu's Arteritis
      Historical Background
      Clinical, Pathologic, and Epidemiologic Features
      Genetic Features
      Pathogenic Mechanisms
      Treatment and Outcome
      Concluding Remarks
      -Future Perspectives
      Acknowledgment
      References
      74. Idiopathic and Autoimmune Interstitial Lung Disease / Ronald G. Crystal
      Introduction
      History
      Cryptogenic Organizing Pneumonia
      Idiopathic Pulmonary Fibrosis
      Clinical, Pathological, and Epidemiological Features
      Cryptogenic Organizing Pneumonia
      Idiopathic Pulmonary Fibrosis
      Autoimmune Features
      Cryptogenic Organizing Pneumonia
      Idiopathic Pulmonary Fibrosis
      Genetic Features
      Cryptogenic Organizing Pneumonia
      Idiopathic Pulmonary Fibrosis
      In Vivo and In Vitro Models
      Cryptogenic Organizing Pneumonia
      Idiopathic Pulmonary Fibrosis
      Pathologic Effector Mechanisms
      Cryptogenic Organizing Pneumonia
      Idiopathic Pulmonary Fibrosis
      Treatment and Outcome
      Cryptogenic Organizing Pneumonia
      Idiopathic Pulmonary Fibrosis
      Conclusions
      Acknowledgments
      References
      pt. 15 Unclassified Expressions of Autoimmunity
      75. Cameos: Candidates and Curiosities / Ian R. Mackay
      Introduction
      Autoimmune/Inflammatory Syndrome Induced by Adjuvants
      Autonomic Neuropathy
      Birdshot Retinopathy
      Cystitis, Interstitial
      Endometriosis
      Epilepsy
      Fatigue Syndrome
      Folate Deficiency
      Lichen Sclerosus
      Lymphocytic Mastitis
      Metabolic-Genetic Storage Diseases
      Movement Disorders
      Narcolepsy
      Osteoarthritis
      Parathyroid Disease
      Polychondritis, Relapsing
      Prostatitis
      Sarcoidosis
      References
      76. Autoantibodies Against Cytokines / James W. Goding
      Introduction
      Autoantibodies Against Cytokines in Humans
      Autoantibodies Against Type I and Type II Interferon
      Autoantibodies Against IL-1 α
      Autoantibodies Against Tumor Necrosis Factor (TNF)
      Autoantibodies Against IL-6
      Autoantibodies against Granulocyte-Macrophage Colony-Stimulating Factor
      Pathogenicity of Autoantibodies Against Cytokines
      -General Comments
      Autoantibodies Against GM-CSF Cause Idiopathic Pulmonary Alveolar Proteinosis
      Autoantibodies Against Erythropoietin Cause Pure Red Cell Aplasia
      Autoantibodies Against IFN-γ Cause Mycobacteria Infections
      Autoantibodies Against IL-17A, IL-17F, and IL-22 Correlate with Mucocutaneous Candidiasis in Autoimmune-Polyendocrinopathy-Candidiasis-Ectodermal Dystrophy
      Opportunistic Infections in Patients with a Thymoma
      Autoantibodies Against the Bioactivity of Osteoprotegerin
      Autoantibodies Against IL-8
      Autoantibodies Against IL-1 α
      Autoantibodies Against IL-6
      What Is the Therapeutic Benefit of Autoantibodies against Cytokines from Pharmaceutically Prepared Immunoglobulin?
      Are Anti-Cytokine Autoantibodies Against a Range of Cytokines in all Healthy Humans?
      Analysis of a Panel of Neutralizing Monoclonal Antibodies to GM-CSF
      Advantage of Studying Monoclonal Autoantibodies with Natural and Authentic H and L Chain Pairing
      Autoantibodies Against GM-CSF Were all Polyclonal, Excluding a "Forbidden B Cell Clone"
      No Preferred V-gene Usage in Autoantibodies to GM-CSF
      Multiple Epitopes Recognized by Pathogenic Monoclonal Autoantibodies to GM-CSF
      Mechanism of Pathogenicity of Monoclonal Autoantibodies to GM-CSF
      Inhibitory Activity Is Strengthened by Formation of Stable High-avidity Complexes Comprising Multiple Antibodies Binding to Multiple Epitopes
      Somatic Mutation of Autoantibodies to GM-CSF
      Is Antibody Autoreactivity a Consequence of Somatic Mutation?
      Could Autoantibodies Arise in Response to a B Cell Epitope on a Pathogen Antigen that Mimics a Self-antigen GM-CSF?
      Autoantibodies to GM-CSF: Implications for B Cell Tolerance to Cytokines
      General B Cell Tolerance
      Antibodies Binding to Multiple Epitopes on GM-CSF Suggest that B Cells Lack Tolerance to Cytokines
      Role of T Cells and the Thymus in Pathogenesis of Autoantibodies to Cytokines
      General T Cell Tolerance
      Role of the AIRE Gene in T Cell Tolerance
      T Cell Tolerance to Cytokines
      T Cell Tolerance Is Incomplete and Breakable
      Autoantibodies against Cytokines in APECED
      Autoantibodies against Cytokines in Patients with Thymoma
      Induction of Autoantibodies as a Consequence o Therapy with Recombinant Cytokines
      Conclusions and Future Prospects
      References
      pt. 16 Diagnosis, Prevention, and Therapy
      77. Autoantibody Assays: Performance, Interpretation, and Standardization / Marvin J. Fritzler
      Introduction
      Spectrum of Autoantibodies
      Assays and Technologies for Autoantibody Testing
      Clinical Interpretation and Application of Autoantibody Testing
      Clinical Practice Guidelines
      Laboratory Reports, Electronic Medical Records, and Cost Analysis
      Standardization and Quality Assurance
      Conclusions and Future Prospects
      References
      78. Prediction of Autoimmune Disease / Roberto Gianani
      Type 1 Diabetes Mellitus as a Model for Prediction of Autoimmune Disease
      Pancreatic Pathology in Type 1 Diabetes Mellitus and Islet Autoimmunity
      Genetics
      Laboratory Markers of Autoimmunity (Including Autoantibodies and T Cell Assays)
      Metabolic Studies
      Organ-Specific Autoimmune Diseases
      Thyroid
      Addison's Disease
      Celiac Disease
      Multiple Sclerosis
      Non-Organ Specific Disease
      Rheumatoid Arthritis
      Systemic Lupus Erythematosus
      Conclusions
      Acknowledgments
      References
      79. Prevention of Autoimmune Disease: The Type 1 Diabetes Paradigm / John M. Wentworth
      Introduction
      People at Risk for Type 1 Diabetes
      Primary Prevention
      Contents note continued: Diet and the Intestinal Environment
      Viruses
      Secondary Prevention
      Mucosa-mediated Antigen-specific Tolerance
      Trials of Islet Autoantigen-specific Vacc inationinHumans
      Epilogue
      Acknowledgments
      References
      80. Treatment of Autoimmune Disease: Established Therapies / Jennifer K. King
      Principles of Immune Suppression
      General Considerations
      Non-Specific Anti-Inflammatory Drugs
      NSAIDs
      Glucocorticoids
      Established Treatments of Rheumatic Diseases
      Antimalarials
      Sulfasalazine
      Leflunomide
      Methotrexate
      Cyclophosphamide
      Mycophenolate Mofetil (Cellcept)
      Azathioprine (Imuran)
      Cyclosporin A
      Other Treatment Options
      B Cell Suppressive Therapies
      IVIG
      Moving Towards Biological and Molecular Therapies
      References
      81. Treatment of Autoimmune Disease: Biological and Molecular Therapies / Lucienne Chatenoud
      Introduction
      Therapeutic Armamentarium Derived from Biotechnology
      Monoclonal Antibodies (mAbs)
      Other Soluble Receptors and Protein Fusion Conjugates
      Soluble Autoantigens
      Bone Marrow Transplantation
      Cell Therapy and Gene Therapy
      Cell Therapy
      Gene Therapy
      Perspectives for the Future
      References.
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