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Dubois’ Lupus Erythematosus and Related Syndromes, 10th Edition

ISBN: 9780323932325
ISBN: 9780323932325
Διαστάσεις 28 × 22 cm

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Κύριος Συγγραφέας


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Διαθεσιμότητα: 11-14 ημέρες


For more than 50 years, Dubois’ Lupus Erythematosus and Related Syndromes has been recognized internationally as the go-to clinical reference on lupus and other connective tissue diseases. From basic scientific principles to practical points of clinical management, the 10th edition provides extensive, authoritative coverage of systemic lupus erythematosus (SLE) and its related diseases in a logical, clearly written, user-friendly manner. It’s an ideal resource for rheumatologists and internal medicine practitioners who need a comprehensive clinical reference on all aspects of SLE, connective tissue diseases, and the antiphospholipid syndromes.
Key Features
  • Provides complete clinical coverage of every aspect of cutaneous and systemic lupus erythematosus, including definitions, pathogenesis, autoantibodies, clinical and laboratory features, management, prognosis, and patient education.
  • Includes significant new content throughout: a new chapter on the endocrine system and hormones; newly distinct chapters on the heart and lung; new content on social disparities in lupus, clinical presentation of nephritis, and pulmonary hypertension; a new concluding chapter on future directions in the field; new basic science coverage of the microbiome, autoinflammatory, and monogenic lupus; and new coverage of diversity, inclusion, gender, compliance, and adherence.
  • Offers a fresh perspective from two new associate editors and many new authors with representation from 30 countries.
  • Contains an up-to-date overview of significant advances in cellular, molecular, and genetic technologies, including genetic advancements in identifying at-risk patients.
  • Discusses the clinical management of related disorders such as Sjogren’s syndrome, scleroderma, polymyositis, and antiphospholipid syndrome (APS).
  • Presents the knowledge and expertise of international contributors to provide new global perspectives on manifestations, diagnosis, and treatment.
  • Features a vibrant, full-color format, with graphs, algorithms, differential diagnosis comparisons, new and improved figures, and schematic diagrams throughout.
  • An eBook version is included with purchase. The eBook allows you to access all of the text, figures, and references, with the ability to search, customize your content, make notes and highlights, and have content read aloud. Additional digital ancillary content may publish up to 6 weeks following the publication date.


Cover image
Title page
Table of Contents
    List of Illustrations
    List of Tables
SECTION 1. What Is Lupus?
1.  History of lupus
    Prescientific period
    Differentiation from tuberculosis
    Recognition of systemic lupus erythematosus
    Systemic lupus erythematosus and “collagen disease”
    Serologic aspects
    Pathogenesis: Some breakthroughs
    Classification criteria and disease indices
    Management: Some breakthroughs
    Related diseases
    Prognosis: Just a few comments
2.  Definition and classification of lupus and lupus-related disorders
    Systemic lupus erythematosus
    Chronic cutaneous lupus
    Drug-induced lupus erythematosus
    Mixed connective tissue disease
    Undifferentiated connective tissue disease and overlap syndromes
    Antiphospholipid antibody syndrome
    Neonatal lupus
3.  The epidemiology of lupus
    The fundamentals of epidemiology
    Pediatric systemic lupus erythematosus
    Cutaneous lupus erythematosus
    Other considerations
SECTION 2. Pathogenesis
4.  Overview of lupus pathogenesis
    Phases of SLE: Evolution of disease in susceptible persons
    Overview: The major pathways favoring development of SLE
    Current approved and investigational therapies for SLE4
    Suggested reading
5.  Genetics of SLE
    Genetic studies on SLE
    SLE susceptibility genes in cell death and clearance of self-antigens
    Fc gamma receptors
    SLE genes in toll-like receptor and IFN-I signaling
    NF-κB signaling
    B-cell signaling in SLE genetics
    SLE genes in T cell signaling
    IL12 and JAK/STAT signaling
    Transcription factors and epigenetic modifiers in SLE
    Applications of the genetic findings
    Future directions
6. Single gene defects and autoinflammation: Relation to Lupus
    Type I interfons and their role in autoimmune disease
    Interferonopathies and associated molecular defects
    Informing monogenic lupus
    Therapeutic implications of RNA/DNA sensing in monogenic autoimmune disease and future directions
7.  Epigenetics of lupus
    DNA methylation in SLE
    Histone modification in SLE
    3D genome and SLE
    RNA modification
    miRNA in SLE
    Roles of lncRNAs in SLE
    Roles of circrnas in SLE
    Translational application of epigenetics in lupus
8.  The role of the environment and microbiome in lupus
    Interplay between environmental, genetic, and epigenetic factors
    Dietary influences on SLE
    Gut microbiome and SLE
    Environmental exposures and SLE
    Methodologic considerations
    Future considerations
9.  The innate immune system in SLE
    Cellular components of the innate immune system
    Extracellular components of the innate immune system
    Pattern recognition receptors: Pamps, damps, and pathways of innate immune stimulation in SLE
    Innate immune detection of self versus nonself nucleic acid
    Therapeutic approaches
10.  B cells and generation of antibodies
    Structure of the antibody molecule
    Generation of antibody diversity
    Posttranscriptional modifications of antibodies
    Pathogenic autoantibodies
    Genetic and molecular analysis of anti-DNA antibodies
    B-cell subsets: Implications for SLE
    B-cell activation
    B-cell tolerance
    Autoantibody induction
    Therapeutic interventions
11.  T cells
    Role of T cells in autoimmunity and inflammation
    Intrinsic T cell defects
    Acquired defects
    Concluding remarks
12.  Immunoregulatory cells and networks in lupus
    T-regulatory cells
    B-regulatory cells
    Myeloid-derived suppressor cells
    Dendritic cells
    Natural killer cells
    NKT cells
13.  Cytokines in lupus
    Properties of cytokines and their receptors
    Assessment of cytokine production
    Use of gene expression to study cytokine effects
    Activation of the immune response in systemic lupus erythematosus
    Cytokines of the innate immune response
    Cytokines of the adaptive immune response
14.  Metabolic control of immunopathogenesis in systemic lupus erythematosus
    Metabolic pathways regulate proinflammatory immune cell lineage specification via MTOR activation in SLE
    Mitochondrial oxidative stress underlie MTOR activation in SLE
    Mechanistic checkpoints of metabolic pathways represent targets for treatment in SLE
15.  Animal models of systemic lupus erythematosus (SLE)
    Multigenic spontaneous SLE
    MRL-lpr mice and MRL/Mp (MRL+/+)
    Nonfunctional Fas ligand in gld mice
    (NZW×BXSB) F1 model of antiphospholipid syndrome and coronary artery disease
    BXD2 RI model of spontaneous lupus nephritis and erosive polyarthritis (“rhupus”)
    Monogenic spontaneous SLE
    Induced lupus-like disease in nonautoimmune strains
    Lupus induced by injection of hydrocarbon oil
    Therapeutic interventions in mouse models of lupus
    Lupus in domestic animals
SECTION 3. Mechanisms of Tissue Injury
16.  Abnormalities in clearance of immune complexes and dying cells in lupus
    Defective apoptotic cell clearance mechanisms in autoimmunity
    Phosphatidylserine receptors
    Role of “don’t eat me” receptors in systemic lupus erythematosus
    Role of scavenger receptors in apoptotic cell clearance
    Role of efferosome maturation in apoptotic cell clearance
    Abnormalities in immune complex clearance
    Tissue clearance mechanisms
    Complement receptors
    Role of C1q
    Fcγ receptors
    Genetic abnormalities of Fcγ receptors
    Other cell types involved in immune complex interactions
    Other tissue clearance mechanisms
    Disclosures of interest
17.  Complement and systemic lupus erythematosus
    Historical overview
    Biology of the complement system
    Complement dysregulation and SLE
    Complement defiency and SLE
    Analyses of complement
    Soluble complement components as biomarkers for SLE
    Cell-bound complements as biomarkers for SLE
    Complement-targeted therapeutics for SLE
18.  Apoptosis and inflammatory forms of cell death
    Introduction and definitions
    Biochemistry of apoptosis
    Deficiencies in death receptor signaling lead to systemic autoimmunity
    Removal of dead and dying cells: Tolerance versus autoimmunity
    Cell death abnormalities in human SLE
19. Tissue damage in lupus: Free Radicals and Fibrosis
    Overview of reactive oxygen and nitrogen intermediates
    Regulation of oxidant stress
    Mechanisms through which ROI can modify DNA and change transcription
    Association of ROI and RNI with SLE disease activity
    T-cell regulation and function
    Autophagy regulation and induction
    Neutrophil net formation
    Podocyte dysfunction induced by RNI
    The role of NO• in vascular homeostasis
20.  Lupus nephritis
    Renal anatomy and physiology
    Histologic classification of lupus nephritis
    Genetic risk for lupus nephritis
    Mouse models of lupus nephritis
    Mechanisms for immune complex deposition in the kidneys
    Pauci-immune glomerulonephritis
    Dissociation of immune complex deposition and/or glomerular injury from end-stage renal disease
    Effector mechanisms in the kidney
    Progression to fibrosis and sclerosis
    Systems biology of lupus nephritis
    Future directions in lupus nephritis
21.  Pathogenesis of accelerated atherosclerosis and vascular injury in systemic lupus erythematosus
    Epidemology of cardiovascular disease in systemic lupus erythematosus
    Pathogenesis of premature atherosclerosis in SLE
    The role of inflammatory lipids
    Other factors associated with cardiovascular disease in SLE
    Key points
22.  Pathogenesis of cutaneous lupus
    Clinical considerations
    How understanding pathogenesis is changing treatment
23.  Pathogenesis of neuropsychiatric lupus
    Cell-mediated inflammation
    Complement-mediated immunity
24.  Pathogenesis of Sjögren syndrome
    Disease triggers
    Autoimmune epithelitis
    From a gland disease to a systemic disease
    Research tools
    From pathogenesis to targeted treatment
    Major knowledge gaps
SECTION 4. Autoantibodies
25.  Antinuclear antibodies, antibodies to DNA, histones, and nucleosomes
    Antinuclear antibody overview
    Antibodies to DNA, histones, and nucleosomes
    Antihistone antibodies
26.  Antibodies against extractable nuclear antige ns (RNP, Sm, SS-A/RO, and SS-B/La)
    Structure of the antigens
    Assays for measuring anti-ENA antibodies
    Prevalence and clinical associations in SLE
    Origins of anti-ENA autoimmunity
    Genetic risks and anti-ENA antibodies
    Pathogenic importance of anti-RNP and anti-Sm antibodies
    Pathogenic role of anti–SS-A/Ro and anti–SS-B/La antibodies
    Autoantibodies against other ENA
27. Other autoantibodies: Antibodies to C1q, Lipoprotein, and Endothelial Cells
    Anti-C1q antibodies
    Antilipoprotein antibodies
    Antiendothelial cell antibodies
28.  Pathogenesis of antiphospholipid antibody syndrome
    The clinical importance of antiphospholipid antibodies
    Epidemiology of aPL and APS
    Which aPL tests should be done and how should they be interpreted?
    Antiphospholipid antibody–induced mechanisms of disease
    Coronavirus disease 2019 and antiphospholipid antibodies
29.  Drug-induced lupus
    Role of cytokines—evidence from biologic agents
    Clinical aspects
SECTION 5. Clinical Aspects of Lupus Erythematosus
30.  Overview and clinical presentation
    Clinical manifestations and racial disparities
    Chief complaint, autoantibody development, and immunologic changes
    Variations in clinical presentation
    Categorization of lupus symptoms
31.  Skin disease in cutaneous lupus erythematosus
    Triggers of cutaneous lupus erythematosus
    Clinical features
    Relationship with systemic disease features
    Laboratory findings
    Differential diagnosis
    Lupus-nonspecific skin lesions
32.  The musculoskeletal system and bone metabolism
    Muscle involvement
    Soft tissue disorders and other pain syndromes
    Musculoskeletal infections
    Avascular necrosis
    Joint replacement in SLE
    Musculoskeletal features in outcome measures
33.  Lupus and the cardiovascular system
34.  Pulmonary manifestations of systemic lupus erythematosus
    Pleural disease
    Parenchymal disease
    Vascular disease
    Diseases of the mediastinum and musculature
    Uncommon pulmonary involvement in SLE
    Lung transplantation in SLE
    Novel treatment targets
35. Lupus and the nervous system: Clinical Presentation, Diagnosis, and Treatment
    Classification of NPSLE
    Epidemiology of NPSLE
    Clinical presentation of NPSLE
    Diagnostic evaluation
    Neuroimaging studies in NPSLE
    Treatment of NPSLE
    Prognosis of NPSLE
36.  Gastrointestinal and hepatic manifestations
    Gastrointestinal involvement
    Liver manifestations of systemic lupus erythematosus
    Biliary abnormalities: Cholecystitis, cholangitis, and biliary cirrhosis
    Hepatobiliary and pancreatic malignancies
37.  The gut microbiome and lupus pathogenesis
    Microbiome dysbiosis and the etiopathogenesis of systemic lupus disease
    Limitations of current approaches and future considerations
    Key points and conclusions
38.  Hematologic and lymphoid abnormalities in SLE
    Immune-mediated hemolytic anemias
    Thrombocytopenia and qualitative platelet disorders
    White blood cell disorders
    Lymphadenopathy in SLE
    Spleen in SLE
    Bonus content for this chapter can be found online at
39. Neonatal lupus: Pathogenesis and Clinical Approaches
    Candidate biomarkers and new theories relating to pathogenesis
    Clinical manifestations
40.  Pregnancy and reproductive health issues in systemic lupus erythematosus
    Hormones and reproductive immunology
    Infertility and protection against premature ovarian failure
    Pregnancy in SLE
    Antiphospholipid syndrome and pregnancy
    Medications in SLE pregnancy
    Menopause and SLE
    Bone health and osteoporosis
    Reproductive health care and screening
41.  Systemic lupus erythemato sus in childhood and adolescence
    Diagnosis of SLE in children
    Common medications in cSLE therapy
42.  Clinical aspects of antiphospholipid syndrome
    Risk factors for thrombosis in those with antiphospholipid antibodies
    Diagnosis and laboratory testing
    Laboratory testing
    Clinical features
    Management strategies in antiphospholipid syndrome
    Other therapies in antiphospholipid syndrome
43.  Lupus and infections
    Prevalence of infections in SLE
    Infections as a cause of morbidity and mortality in SLE
    Risk factors for infections and serious infections in SLE
    Protean spectrum of infection in SLE
    Intrinsic immune dysfunction increasing infection susceptibility in SLE
    Therapeutic toxicities
    Select genetic defects and risk of infection
    Can biomarkers be used to differentiate between infection and disease flare?
    Clinical approach to SLE patients with a suspected infection
44.  Ocular, aural, and oral manifestations of lupus
    Systemic lupus erythematosus and the eye
    Lupus and the mouth
    Lupus and the nose
    Lupus and the larynx
    Lupus and the ear
45.  Clinical aspects of Sjögren’s disease
    Introduction and epidemiology
    Clinical presentation
    Classification and diagnosis of Sjögren’s
    Outcome measures
46.  Clinical presentations of lupus in the kidney and urogenital system
    Lupus nephritis epidemiology and risk factors
    Clinical presentation and diagnosis of lupus nephritis
    Differential diagnosis of lupus nephritis
    Histopathologic classification of lupus nephritis
    Lupus nephritis biomarkers
    Lupus nephritis during pregnancy
47.  The endocrine system and hormones in lupus
    Role of the hypothalamic-pituitary-adrenal axis in the pathogenesis of SLE
    Role of stress in SLE
    Does prolactin function as an inflammatory cytokine?
    Sex hormones and their role in SLE
    Specific endocrine disorders in SLE
    Diabetes mellitus and SLE
    Prolactin in SLE
    Parathyroid disease in SLE
    Adrenal disease in SLE
SECTION 6. Assessment of Lupus
48.  Clinical application of serologic tests, serum protein abnormalities, and other laboratory tests in systemic lupus erythematosus
    Diagnosis of SLE
    Monitoring disease activity in SLE
    Clinical significance of anti-dsDNA antibodies
    Anti-Sm antibodies
    Anti–U1 ribonucleoprotein
    Anti–SS-B antibodies
    Antihistone antibodies
    Antinucleosome antibodies in SLE
    Anti-C1Q antibodies
    Anti–ribosomal P antibodies
    Anti-centromere and anti–SCL-70 antibodies
    Serum complements
    Plasma proteins
    Serum immunoglobulins
    Other serologic abnormalities in SLE
    Clustering of autoantibodies
49.  Differential diagnosis and disease associations
    When to suspect SLE
    Laboratory evaluation of suspected systemic lupus erythematosus
    Classification criteria
    Diagnostic challenges
50.  Incomplete lupus, undifferentiated connective tissue disease, and mixed connective tissue disease
    Introduction and historical background
    Undifferentiated connective tissue disease and incomplete lupus syndromes
    Clinical findings in incomplete lupus erythematosus and undifferentiated connective tissue disease
    Autoantibodies and immunologic findings
    Treatment strategies
    Mixed connective tissue disease
51.  Clinical markers, metrics, indices, and clinical trials
    Principles and approaches for assessing patients with lupus
    Disease activity indices
    Damage assessment
    Health-related quality of life
SECTION 7. Management of Lupus
52.  Principles of therapy, local measures, and NSAIDs
    Formulation overview
    Patient education
    General therapeutic considerations
    Compliance and adherence
    Nonsteroidal antiinflammatory drugs for the treatment of SLE
    Mechanisms of action
53.  Antimalarial medications
    Mechanism of action
    Efficacy of antimalarials
    Adverse effects of antimalarials
54.  Systemic glucocorticoid therapy for SLE
    Therapeutic use of glucocorticoids in SLE
    Molecular mechanisms of glucocorticoid action
    Adverse effects of glucocorticoids
    Conclusions and the future of glucocorticoid therapy in SLE
55.  Immunosuppressive drug therapy
    Historical perspective
    Alkylating agents
    Mycophenolate mofetil
    Calcineurin inhibitors
56.  Complementary and integrative health approaches
    Prevalence of use
    Nutritional approaches
    Psychological approaches
    Physical approaches
    Combination approaches
57.  Specialized and niche therapies
    Treatment of patients with SLE and end-stage renal disease
    Laser therapy
    Apheresis and related technologies
    Ultraviolet-1 radiation
    Should radiation therapy be avoided?
    Niche therapies for lupus subsets
58.  Adjunctive and preventive measures
    Infection prevention in lupus
    Antibiotic prophylaxis in lupus
    Drug allergies in patients with lupus
    Vitamin D supplementation in lupus
    Adherence issues in lupus
59.  Management of cutaneous lupus erythematosus
    Topical medications
    Intralesional therapy
    Oral/intravenous corticosteroids
    Mycophenolate mofetil
    Oral retinoids
    Intravenous immunoglobulin
    Biologic therapies
60.  Targeted therapies in SLE
    B cell directed therapies
    T cell directed therapeutic approaches
    Cytokine inhibition
61.  Management of lupus nephritis
    Clinical manifestations of lupus nephritis
    Laboratory assessment of lupus nephritis
    Kidney biopsy in the management of lupus nephritis
    Histopathologic classification of lupus nephritis
    Management of lupus nephritis
    Renoprotective nonimmunosuppressive interventions
    Antimalarial drugs in lupus nephritis
    Expected results of the initial therapy
    Maintenance therapy
    Nonimmune complex-mediated kidney injury in SLE
    Resistant disease
    Long-term monitoring of lupus nephritis patients
    Pregnancy in lupus nephritis
    Progression to end-stage kidney disease
62.  Management of Sjögren disease syndrome
    Nonmedical therapy of dryness
    Treatment of symptoms of mild-to-moderate dry eye
    Treatment of moderate-to-severe dry eye
    Overview of treatment of dry mouth
    Treatment of systemic and extraglandular manifestations of Sjögren disease
    Emerging therapies for the management of Sjögren disease
    Suggested reading
SECTION 8. Outcomes
63.  Socioeconomics, disparities, and disability
    Physical health and quality of life
    Mental health and well-being
    Effect of illness on school and social support systems
    Employment and long-term disability
    Economic costs of systemic lupus erythematosus
    Effects of adverse social determinants of health
    Key take-away points
64.  Prognosis and mortality of systemic lupus erythematosus
    Survival rates in SLE
    Causes of death in SLE
    Mortality in pediatric-onset SLE
    Strategies for improved mortality outcomes in SLE
65. Future directions: Therapeutics
    Innate immune system targets
    Adaptive immune system targets
    Signaling pathways
    Cellular therapies
    Combination therapies
    Repurposed drugs
    Miscellaneous strategies
66. Expanding the opportunities to effectively treat patients: Clinical Considerations
    Classification criteria and treatment guidance documents
    The relevance of lupus subsets
    Identifying relevant biomarkers and clinical determinants for precision medicine
    Preventing lupus
    Role of lupus advocacy groups