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Table of Contents
Cover
Title
Copyright
Dedication
Contents
Contributors
Introduction
Introduction to the second edition
Part I - Epidemiology and diagnosis
Chapter 1 - History of systemic lupus erythematosus with an emphasis on certain recent major issues
History of clinical observations
The age of dermatology
Transition of lupus erythematosus to systemic lupus erythematosus
History of laboratory investigations
Development of clinical tests
History of genetics of SLE
History of therapy in SLE with an emphasis on the use of steroids
History of classification criteria for SLE
References
Chapter 2 - The patient
Improve the interactions with your patients
Do not make your patient wait
Smile as you enter the patient's room
Make a visible show of hand washing
Shake hands
Acknowledge and greet others in the room
Ask open-ended questions
Provide a mechanism for your patient to set the agenda
Speak in nonmedical terms
Be honest
Learn to be empathetic
Always examine your patient
End the visit with, "Do you have any other concerns or questions?"
Consulting in the hospital
Improving adherence
Disability
Patient education
The lupus secrets
References
Chapter 3 - A plea of a young patient to the lupus experts
Reference
Chapter 4 - Epidemiology
Introduction
Incidence and prevalence
Distribution according to gender
Distribution according to age
Distribution according to ethnic/racial group
Distribution around the world
Factors that affect the course of SLE
Impact of race/ethnicity
Impact of gender
Mortality and survival in SLE
References
Chapter 5 - Measuring disease activity
References
Chapter 6 - Disease development and outcome
Historical perspective
Clinical manifestations
Assessment of disease activity.
Disease damage
Patient reported outcomes
Mortality
Causes of death
Treatment guidelines and quality indicators
Conclusions
References
Chapter 7 - Socioeconomic aspects of SLE
Introduction
Sociodemographic determinants of health in SLE
Gender as a construct in risk and morbidity
Race/ethnicity as a multidimensional determinant of outcomes
Age and aging
Income, poverty, and educational attainment
Healthcare access and utilization
Health literacy
Health insurance
The economic burden of SLE
Work and disability
Individual and societal costs
Conclusion
References
Chapter 8 - Biomarkers in systemic lupus erythematosus
Introduction
Biomarker definition and validation
Biomarkers for diagnosis of SLE
Cell-bound complement activation products
Interferon-α and interferon-α-inducible genes
T-cell gene expression assays
DNA methylation
Plasma thermograms
Biomarkers for measuring SLE disease activity
Cell-bound complement activation proteins
Interferon-α and interferon-α-inducible genes
B-lymphocyte stimulating factor/B-cell activating factor
MicroRNAs
Tumor necrosis factor α
CD27high plasma cells
CD44+ T cells
Biomarkers to detect specific organ involvement
Lupus nephritis biomarkers
Antibodies to complement C1q
Monocyte chemoattractant protein-1
Neutrophil gelatinase-associated lipocalin
Urinary TWEAK
MicroRNAs
Hepcidin
Urinary biomarker panels
Central nervous system biomarkers
Anti-NMDA receptor antibody
Antiribosomal P antibody
Antiphospholipid antibodies
Platelet-bound C4d
Other
Cardiovascular system biomarkers
Respiratory system biomarkers
Lupus arthritis biomarkers
Genetic susceptibility and theranostics
Conclusions
References
Part II - Pathogenesis.
Chapter 9 - Overview of the pathogenesis of systemic lupus erythematosus
Genetics
Epigenetics
Gender
Environment
Immune dysregulation
Tissue damage
Conclusions
References
Chapter 10 - System lupus erythematosus and the environment
Introduction
Infectious agents, dysbiosis the microbiome, and SLE
Cigarette smoking, alcohol and SLE
Cosmetics, chemicals, and risk of SLE
Ultraviolet radiation, vitamin D, and SLE
Drugs, vaccines, and SLE
Geography, socioeconomics, and SLE
Conclusion
References
Chapter 11 - Genes and genetics in human SLE
Introduction
Transcription factors
Clearance of apoptotic cells and immune complexes
Autophagy
Type I IFN pathway
NFkB pathway
Neutrophils and NETosis
T cell signaling
B cell signaling
Genes and phenotypes
SLE risk genes as therapeutic targets
Drug repositioning and predicting clinical outcomes
Conclusions
References
Chapter 12 - Monogenic lupus
Introduction
Complement deficiency
Deficiencies in DNA damage repair and clearance
DNase1L3
DNase II
DNase III/TREX1
Abnormalities of DNA sensing
Stimulator of interferon genes (STING)
IFIH1
Other interferonopathies
Apoptosis
Fas/FasL
Cell signaling
PKCδ
Ras
STAT1
Implications for SLE as a whole
Conclusion
References
Chapter 13 - Hormones
Sex hormones
Estrogen-estrogen receptor signaling
Estrogen and the immune response
Innate immune response
Adaptive immune response
B cells
T cells
Estrogen and SLE
Other hormones
Hormone therapy
Conclusions
References
Chapter 14 - Clinical aspects of the complement system in systemic lupus erythematosus
Introduction
General comments
Historical notes
Complement testing and its interpretation.
Serum levels and hemolytic activities for C4 and C3
Interpretation of C4 and C3 complement tests in SLE
Consumption versus biosynthesis
Chronically low C4 and C3: How to interpret?
When C4 and C3 are discrepant
Cryoglobulins
Cold activation
Copy-number variation (CNV)
Alternative pathway activation
New developments
Assessing a therapeutic response
Interpretation
Renal biopsy
New connections for lupus and complement
Local synthesis
Activation by local proteases
Intracellular complement activation
Exogenous coating with opsonic fragments leads to endogenous consequences
Conclusions
Lupus develops among complement deficient subjects
To treat or not to treat SLE with a complement inhibitor
Cons
Pros
Future considerations
References
Chapter 15 - T cells
Mechanisms through which T Cells promote SLE
B cell help
Proinflammatory activities
Defective regulation
Signaling and gene expression in SLE T cells
Apoptosis defects
References
Chapter 16 - B cells in SLE
Introduction
B lineage cell abnormalities in SLE indicating disturbances of B cell differentiation
Functional abnormalities of SLE B lineage cells
B cell signaling in "post-activated B cells" in SLE
Conclusions
References
Chapter 17 - Neutrophils in systemic lupus erythematosus
Introduction
Neutrophil dysfunction in SLE
NETosis in the pathogenesis of SLE
Low-density granulocytes (LDGs) in SLE
DNA methylation changes in SLE neutrophils and LDGs
Conclusion
References
Chapter 18 - The role of dendritic cells in systemic lupus erythematosus
Dendritic cell origins, subsets, and functions
Origins
Subsets/functions
Conventional/myeloid DCs
Plasmacytoid DCs
Monocyte-derived DCs (Mo-DCs)
DCs and tolerance
DCs in central tolerance.
DCs in peripheral tolerance
DCs and SLE
Myeloid DCs
Number/activation status
Implication for T-cell activation
Implication for B-cells activation
pDCs
Number/activation status
Implication for T-cell activation
Implication for B-cells activation
Amplifying mechanisms promoting IFN-α secretion in SLE and activation of DCs
NK cell help
Platelet help
Neutrophil help
Overall picture of DC implication in SLE pathogenesis
References
Chapter 19 - Cytokines
Cytokines in SLE
IL-2
IL-17
IL-6
TNF
IFN-α
BAFF and APRIL
Conclusions
References
Chapter 20 - RNA/DNA sensing in SLE-Toll-like receptors and beyond
Introduction
Toll-like receptor family (TLRs)
Toll-like receptor 7 (TLR7)
TLR7 expression in SLE
TLR7 in murine lupus
TLR7 polymorphisms in SLE
Toll-like receptor 8 (TLR8)
TLR8 in murine lupus
TLR8 polymorphisms in SLE
Toll-like receptor 9 (TLR9)
TLR9 expression in SLE
TLR9 in murine lupus
TLR9 polymorphisms and copy number in SLE
Cytosolic RNA and DNA sensors
Activation of cytosolic RNA/DNA sensors
cGAS and RLRs in SLE
Conclusions
References
Chapter 21 - The role of interferons in systemic lupus erythematosus
Introduction
The interferon families
Regulation of interferon production
Interferons and the immune system
Interferons in systemic lupus erythematosus
Impact of interferons in pathogenesis and disease heterogeneity
Genetic factors
Measuring IFNs in blood
Targeting the interferon system in lupus
Conclusion
References
Chapter 22 - Fcγ receptors in autoimmunity and end-organ damage
Introduction
FcγRs structure
IgG and FcγR interactions
FcγRs and complement
Activating and inhibitory FcγR signaling
Regulation of FcγR affinity for ligand
Roles of FcγR in SLE.
Maintenance of peripheral tolerance.
Title
Copyright
Dedication
Contents
Contributors
Introduction
Introduction to the second edition
Part I - Epidemiology and diagnosis
Chapter 1 - History of systemic lupus erythematosus with an emphasis on certain recent major issues
History of clinical observations
The age of dermatology
Transition of lupus erythematosus to systemic lupus erythematosus
History of laboratory investigations
Development of clinical tests
History of genetics of SLE
History of therapy in SLE with an emphasis on the use of steroids
History of classification criteria for SLE
References
Chapter 2 - The patient
Improve the interactions with your patients
Do not make your patient wait
Smile as you enter the patient's room
Make a visible show of hand washing
Shake hands
Acknowledge and greet others in the room
Ask open-ended questions
Provide a mechanism for your patient to set the agenda
Speak in nonmedical terms
Be honest
Learn to be empathetic
Always examine your patient
End the visit with, "Do you have any other concerns or questions?"
Consulting in the hospital
Improving adherence
Disability
Patient education
The lupus secrets
References
Chapter 3 - A plea of a young patient to the lupus experts
Reference
Chapter 4 - Epidemiology
Introduction
Incidence and prevalence
Distribution according to gender
Distribution according to age
Distribution according to ethnic/racial group
Distribution around the world
Factors that affect the course of SLE
Impact of race/ethnicity
Impact of gender
Mortality and survival in SLE
References
Chapter 5 - Measuring disease activity
References
Chapter 6 - Disease development and outcome
Historical perspective
Clinical manifestations
Assessment of disease activity.
Disease damage
Patient reported outcomes
Mortality
Causes of death
Treatment guidelines and quality indicators
Conclusions
References
Chapter 7 - Socioeconomic aspects of SLE
Introduction
Sociodemographic determinants of health in SLE
Gender as a construct in risk and morbidity
Race/ethnicity as a multidimensional determinant of outcomes
Age and aging
Income, poverty, and educational attainment
Healthcare access and utilization
Health literacy
Health insurance
The economic burden of SLE
Work and disability
Individual and societal costs
Conclusion
References
Chapter 8 - Biomarkers in systemic lupus erythematosus
Introduction
Biomarker definition and validation
Biomarkers for diagnosis of SLE
Cell-bound complement activation products
Interferon-α and interferon-α-inducible genes
T-cell gene expression assays
DNA methylation
Plasma thermograms
Biomarkers for measuring SLE disease activity
Cell-bound complement activation proteins
Interferon-α and interferon-α-inducible genes
B-lymphocyte stimulating factor/B-cell activating factor
MicroRNAs
Tumor necrosis factor α
CD27high plasma cells
CD44+ T cells
Biomarkers to detect specific organ involvement
Lupus nephritis biomarkers
Antibodies to complement C1q
Monocyte chemoattractant protein-1
Neutrophil gelatinase-associated lipocalin
Urinary TWEAK
MicroRNAs
Hepcidin
Urinary biomarker panels
Central nervous system biomarkers
Anti-NMDA receptor antibody
Antiribosomal P antibody
Antiphospholipid antibodies
Platelet-bound C4d
Other
Cardiovascular system biomarkers
Respiratory system biomarkers
Lupus arthritis biomarkers
Genetic susceptibility and theranostics
Conclusions
References
Part II - Pathogenesis.
Chapter 9 - Overview of the pathogenesis of systemic lupus erythematosus
Genetics
Epigenetics
Gender
Environment
Immune dysregulation
Tissue damage
Conclusions
References
Chapter 10 - System lupus erythematosus and the environment
Introduction
Infectious agents, dysbiosis the microbiome, and SLE
Cigarette smoking, alcohol and SLE
Cosmetics, chemicals, and risk of SLE
Ultraviolet radiation, vitamin D, and SLE
Drugs, vaccines, and SLE
Geography, socioeconomics, and SLE
Conclusion
References
Chapter 11 - Genes and genetics in human SLE
Introduction
Transcription factors
Clearance of apoptotic cells and immune complexes
Autophagy
Type I IFN pathway
NFkB pathway
Neutrophils and NETosis
T cell signaling
B cell signaling
Genes and phenotypes
SLE risk genes as therapeutic targets
Drug repositioning and predicting clinical outcomes
Conclusions
References
Chapter 12 - Monogenic lupus
Introduction
Complement deficiency
Deficiencies in DNA damage repair and clearance
DNase1L3
DNase II
DNase III/TREX1
Abnormalities of DNA sensing
Stimulator of interferon genes (STING)
IFIH1
Other interferonopathies
Apoptosis
Fas/FasL
Cell signaling
PKCδ
Ras
STAT1
Implications for SLE as a whole
Conclusion
References
Chapter 13 - Hormones
Sex hormones
Estrogen-estrogen receptor signaling
Estrogen and the immune response
Innate immune response
Adaptive immune response
B cells
T cells
Estrogen and SLE
Other hormones
Hormone therapy
Conclusions
References
Chapter 14 - Clinical aspects of the complement system in systemic lupus erythematosus
Introduction
General comments
Historical notes
Complement testing and its interpretation.
Serum levels and hemolytic activities for C4 and C3
Interpretation of C4 and C3 complement tests in SLE
Consumption versus biosynthesis
Chronically low C4 and C3: How to interpret?
When C4 and C3 are discrepant
Cryoglobulins
Cold activation
Copy-number variation (CNV)
Alternative pathway activation
New developments
Assessing a therapeutic response
Interpretation
Renal biopsy
New connections for lupus and complement
Local synthesis
Activation by local proteases
Intracellular complement activation
Exogenous coating with opsonic fragments leads to endogenous consequences
Conclusions
Lupus develops among complement deficient subjects
To treat or not to treat SLE with a complement inhibitor
Cons
Pros
Future considerations
References
Chapter 15 - T cells
Mechanisms through which T Cells promote SLE
B cell help
Proinflammatory activities
Defective regulation
Signaling and gene expression in SLE T cells
Apoptosis defects
References
Chapter 16 - B cells in SLE
Introduction
B lineage cell abnormalities in SLE indicating disturbances of B cell differentiation
Functional abnormalities of SLE B lineage cells
B cell signaling in "post-activated B cells" in SLE
Conclusions
References
Chapter 17 - Neutrophils in systemic lupus erythematosus
Introduction
Neutrophil dysfunction in SLE
NETosis in the pathogenesis of SLE
Low-density granulocytes (LDGs) in SLE
DNA methylation changes in SLE neutrophils and LDGs
Conclusion
References
Chapter 18 - The role of dendritic cells in systemic lupus erythematosus
Dendritic cell origins, subsets, and functions
Origins
Subsets/functions
Conventional/myeloid DCs
Plasmacytoid DCs
Monocyte-derived DCs (Mo-DCs)
DCs and tolerance
DCs in central tolerance.
DCs in peripheral tolerance
DCs and SLE
Myeloid DCs
Number/activation status
Implication for T-cell activation
Implication for B-cells activation
pDCs
Number/activation status
Implication for T-cell activation
Implication for B-cells activation
Amplifying mechanisms promoting IFN-α secretion in SLE and activation of DCs
NK cell help
Platelet help
Neutrophil help
Overall picture of DC implication in SLE pathogenesis
References
Chapter 19 - Cytokines
Cytokines in SLE
IL-2
IL-17
IL-6
TNF
IFN-α
BAFF and APRIL
Conclusions
References
Chapter 20 - RNA/DNA sensing in SLE-Toll-like receptors and beyond
Introduction
Toll-like receptor family (TLRs)
Toll-like receptor 7 (TLR7)
TLR7 expression in SLE
TLR7 in murine lupus
TLR7 polymorphisms in SLE
Toll-like receptor 8 (TLR8)
TLR8 in murine lupus
TLR8 polymorphisms in SLE
Toll-like receptor 9 (TLR9)
TLR9 expression in SLE
TLR9 in murine lupus
TLR9 polymorphisms and copy number in SLE
Cytosolic RNA and DNA sensors
Activation of cytosolic RNA/DNA sensors
cGAS and RLRs in SLE
Conclusions
References
Chapter 21 - The role of interferons in systemic lupus erythematosus
Introduction
The interferon families
Regulation of interferon production
Interferons and the immune system
Interferons in systemic lupus erythematosus
Impact of interferons in pathogenesis and disease heterogeneity
Genetic factors
Measuring IFNs in blood
Targeting the interferon system in lupus
Conclusion
References
Chapter 22 - Fcγ receptors in autoimmunity and end-organ damage
Introduction
FcγRs structure
IgG and FcγR interactions
FcγRs and complement
Activating and inhibitory FcγR signaling
Regulation of FcγR affinity for ligand
Roles of FcγR in SLE.
Maintenance of peripheral tolerance.