Dubois’ Lupus Erythematosus and Related Syndromes

 

Description:

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.

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.

 

Table of Contents:

 

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

Epidemiology

Classification criteria and disease indices

Management: Some breakthroughs

Related diseases

Prognosis: Just a few comments

References

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

Summary

References

3.  The epidemiology of lupus

Introduction

The fundamentals of epidemiology

Pediatric systemic lupus erythematosus

Cutaneous lupus erythematosus

Other considerations

Conclusion

References

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

References

6. Single gene defects and autoinflammation: Relation to Lupus

Introduction

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

References

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

References

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

References

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

References

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

Summary

References

11.  T cells

Role of T cells in autoimmunity and inflammation

Intrinsic T cell defects

Acquired defects

Concluding remarks

References

12.  Immunoregulatory cells and networks in lupus

T-regulatory cells

B-regulatory cells

Myeloid-derived suppressor cells

Dendritic cells

Natural killer cells

NKT cells

Conclusions

References

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

Summary

References

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

Conclusions

Acknowledgement

References

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

References

SECTION 3. Mechanisms of Tissue Injury

16.  Abnormalities in clearance of immune complexes and dying cells in lupus

Introduction

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

Acknowledgments

Disclosures of interest

Summary

References

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

Conclusion

References

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

Conclusions

Acknowledgments

References

19. Tissue damage in lupus: Free Radicals and Fibrosis

Introduction

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

Autoantibodies

T-cell regulation and function

Apoptosis

Autophagy regulation and induction

Neutrophil net formation

Podocyte dysfunction induced by RNI

Fibrosis

The role of NO• in vascular homeostasis

Conclusion

References

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

References

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

Conclusions

Acknowledgments

Key points

References

22.  Pathogenesis of cutaneous lupus

Introduction

Clinical considerations

Epidemiology

Etiopathogenesis

How understanding pathogenesis is changing treatment

References

23.  Pathogenesis of neuropsychiatric lupus

Introduction

Cell-mediated inflammation

Complement-mediated immunity

Summary

References

24.  Pathogenesis of Sjögren syndrome

Introduction

Disease triggers

Autoimmune epithelitis

From a gland disease to a systemic disease

Research tools

From pathogenesis to targeted treatment

Major knowledge gaps

Conclusions

References

SECTION 4. Autoantibodies

25.  Antinuclear antibodies, antibodies to DNA, histones, and nucleosomes

Antinuclear antibody overview

Antibodies to DNA, histones, and nucleosomes

Antihistone antibodies

Acknowledgments

Summary

References

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

References

27. Other autoantibodies: Antibodies to C1q, Lipoprotein, and Endothelial Cells

Introduction

Anti-C1q antibodies

Antilipoprotein antibodies

Antiendothelial cell antibodies

References

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

Conclusions

References

29.  Drug-induced lupus

Introduction

Etiology

Pathogenesis

Role of cytokines—evidence from biologic agents

Clinical aspects

Summary

References

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

References

31.  Skin disease in cutaneous lupus erythematosus

Epidemiology

Triggers of cutaneous lupus erythematosus

Clinical features

Relationship with systemic disease features

Pathology

Immunopathology

Laboratory findings

Differential diagnosis

Lupus-nonspecific skin lesions

Conclusion

References

32.  The musculoskeletal system and bone metabolism

Introduction

Arthritis

Muscle involvement

Soft tissue disorders and other pain syndromes

Musculoskeletal infections

Avascular necrosis

Joint replacement in SLE

Musculoskeletal features in outcome measures

Summary

References

33.  Lupus and the cardiovascular system

Introduction

Epidemiology

Pericardium

Myocardium

Endocardium

Vasculature

References

34.  Pulmonary manifestations of systemic lupus erythematosus

Introduction

Pleural disease

Parenchymal disease

Vascular disease

Diseases of the mediastinum and musculature

Uncommon pulmonary involvement in SLE

Lung transplantation in SLE

Novel treatment targets

Summary

References

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

References

36.  Gastrointestinal and hepatic manifestations

Gastrointestinal involvement

Liver manifestations of systemic lupus erythematosus

Biliary abnormalities: Cholecystitis, cholangitis, and biliary cirrhosis

Hepatobiliary and pancreatic malignancies

References

37.  The gut microbiome and lupus pathogenesis

Introduction

Microbiome dysbiosis and the etiopathogenesis of systemic lupus disease

Limitations of current approaches and future considerations

Key points and conclusions

References

38.  Hematologic and lymphoid abnormalities in SLE

Anemia

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

References

39. Neonatal lupus: Pathogenesis and Clinical Approaches

History

Epidemiology

Pathogenesis

Candidate biomarkers and new theories relating to pathogenesis

Clinical manifestations

Management

Prognosis

References

40.  Pregnancy and reproductive health issues in systemic lupus erythematosus

Introduction

Hormones and reproductive immunology

Contraception

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

References

41.  Systemic lupus erythemato sus in childhood and adolescence

Introduction

Epidemiology

Diagnosis of SLE in children

Common medications in cSLE therapy

Summary

References

42.  Clinical aspects of antiphospholipid syndrome

Introduction

Epidemiology

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

Conclusion

References

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

Acknowledgments

Summary

References

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

Acknowledgments

References

45.  Clinical aspects of Sjögren’s disease

Introduction and epidemiology

History

Clinical presentation

Classification and diagnosis of Sjögren’s

Outcome measures

Acknowledgment

References

46.  Clinical presentations of lupus in the kidney and urogenital system

Introduction

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

References

47.  The endocrine system and hormones in lupus

Introduction

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

Acknowledgements

References

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-A

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

References

49.  Differential diagnosis and disease associations

When to suspect SLE

Laboratory evaluation of suspected systemic lupus erythematosus

Classification criteria

Diagnostic challenges

Conclusion

References

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

References

51.  Clinical markers, metrics, indices, and clinical trials

Introduction

Principles and approaches for assessing patients with lupus

Disease activity indices

Damage assessment

Health-related quality of life

References

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

References

53.  Antimalarial medications

Antimalarials

Pharmacokinetics

Mechanism of action

Efficacy of antimalarials

Adverse effects of antimalarials

Summary

References

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

References

55.  Immunosuppressive drug therapy

Historical perspective

Alkylating agents

Chlorambucil

Cyclophosphamide

Azathioprine

Mycophenolate mofetil

Calcineurin inhibitors

Conclusion

References

56.  Complementary and integrative health approaches

Introduction

Prevalence of use

Nutritional approaches

Psychological approaches

Physical approaches

Combination approaches

Conclusions

References

57.  Specialized and niche therapies

Treatment of patients with SLE and end-stage renal disease

Transplantation

Laser therapy

Apheresis and related technologies

Summary

Ultraviolet-1 radiation

Should radiation therapy be avoided?

Niche therapies for lupus subsets

References

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

References

59.  Management of cutaneous lupus erythematosus

Introduction

Photoprotection

Topical medications

Intralesional therapy

Antimalarials

Oral/intravenous corticosteroids

Mycophenolate mofetil

Methotrexate

Azathioprine

Cyclophosphamide

Oral retinoids

Dapsone

Thalidomide

Lenalidomide

Intravenous immunoglobulin

Biologic therapies

References

60.  Targeted therapies in SLE

Introduction

B cell directed therapies

T cell directed therapeutic approaches

Cytokine inhibition

Summary

References

61.  Management of lupus nephritis

Introduction

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

References

62.  Management of Sjögren disease syndrome

Introduction

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

Summary

References

Suggested reading

SECTION 8. Outcomes

63.  Socioeconomics, disparities, and disability

Introduction

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

Interventions

Conclusion

Key take-away points

References

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

Acknowledgments

References

65. Future directions: Therapeutics

Introduction

Innate immune system targets

Adaptive immune system targets

Signaling pathways

Cellular therapies

Combination therapies

Repurposed drugs

Miscellaneous strategies

Summary

References

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

Summary

References

Index

 

An aparitie	30 apr 2024
Autor	Daniel J. Wallace, Bevra Hahn
Editura	Elsevier
Format	Paperback
ISBN	9780323932325
Limba	Engleza
Nr pag	816