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.
1. Cover image - p. i 2. Title page - p. ii 3. Table of Contents - p. iii 4. Copyright - p. iv 5. Dedication - p. v 6. Preface - p. vi 7. Acknowledgments - p. vii 8. Contributors - p. viii 9. List of Illustrations - p. xv 10. List of Tables - p. xvi 11. SECTION 1. What Is Lupus? - p. xvi 12. 1. History of lupus - p. 1 13. 2. Definition and classification of lupus and lupus-related disorders - p. 12 14. 3. The epidemiology of lupus - p. 22 15. SECTION 2. Pathogenesis - p. 38 16. 4. Overview of lupus pathogenesis - p. 39 17. 5. Genetics of SLE - p. 49 18. 6. Single gene defects and autoinflammation: Relation to Lupus - p. 62 19. 7. Epigenetics of lupus - p. 74 20. DNA methylation in SLE - p. 74 21. Histone modification in SLE - p. 77 22. 3D genome and SLE - p. 80 23. RNA modification - p. 81 24. miRNA in SLE - p. 85 25. Roles of lncRNAs in SLE - p. 92 26. Roles of circrnas in SLE - p. 95 27. Translational application of epigenetics in lupus - p. 96 28. References - p. 100 29. 8. The role of the environment and microbiome in lupus - p. 108 30. 9. The innate immune system in SLE - p. 116 31. 10. B cells and generation of antibodies - p. 124 32. 11. T cells - p. 139 33. 12. Immunoregulatory cells and networks in lupus - p. 152 34. 13. Cytokines in lupus - p. 163 35. Properties of cytokines and their receptors - p. 163 36. Assessment of cytokine production - p. 164 37. Use of gene expression to study cytokine effects - p. 165 38. Activation of the immune response in systemic lupus erythematosus - p. 165 39. Cytokines of the innate immune response - p. 165 40. Cytokines of the adaptive immune response - p. 171 41. Summary - p. 174 42. References - p. 174 43. 14. Metabolic control of immunopathogenesis in systemic lupus erythematosus - p. 181 44. 15. Animal models of systemic lupus erythematosus (SLE) - p. 189 45. Multigenic spontaneous SLE - p. 189 46. MRL-lpr mice and MRL/Mp (MRL+/+) - p. 200 47. Nonfunctional Fas ligand in gld mice - p. 202 48. (NZW×BXSB) F1 model of antiphospholipid syndrome and coronary artery disease - p. 204 49. BXD2 RI model of spontaneous lupus nephritis and erosive polyarthritis (“rhupus”) - p. 204 50. Monogenic spontaneous SLE - p. 205 51. Induced lupus-like disease in nonautoimmune strains - p. 206 52. Lupus induced by injection of hydrocarbon oil - p. 207 53. Therapeutic interventions in mouse models of lupus - p. 208 54. Lupus in domestic animals - p. 216 55. References - p. 217 56. SECTION 3. Mechanisms of Tissue Injury - p. 234 57. 16. Abnormalities in clearance of immune complexes and dying cells in lupus - p. 235 58. 17. Complement and systemic lupus erythematosus - p. 245 59. Historical overview - p. 246 60. References - p. 259 61. 18. Apoptosis and inflammatory forms of cell death - p. 265 62. 19. Tissue damage in lupus: Free Radicals and Fibrosis - p. 277 63. 20. Lupus nephritis - p. 292 64. Renal anatomy and physiology - p. 292 65. Histologic classification of lupus nephritis - p. 296 66. Genetic risk for lupus nephritis - p. 297 67. Mouse models of lupus nephritis - p. 298 68. Mechanisms for immune complex deposition in the kidneys - p. 299 69. Pauci-immune glomerulonephritis - p. 300 70. Dissociation of immune complex deposition and/or glomerular injury from end-stage renal disease - p. 300 71. Effector mechanisms in the kidney - p. 300 72. Progression to fibrosis and sclerosis - p. 306 73. Systems biology of lupus nephritis - p. 308 74. Future directions in lupus nephritis - p. 310 75. References - p. 310 76. 21. Pathogenesis of accelerated atherosclerosis and vascular injury in systemic lupus erythematosus - p. 316 77. 22. Pathogenesis of cutaneous lupus - p. 329 78. 23. Pathogenesis of neuropsychiatric lupus - p. 338 79. 24. Pathogenesis of Sjögren syndrome - p. 349 80. SECTION 4. Autoantibodies - p. 365 81. 25. Antinuclear antibodies, antibodies to DNA, histones, and nucleosomes - p. 366 82. 26. Antibodies against extractable nuclear antige ns (RNP, Sm, SS-A/RO, and SS-B/La) - p. 378 83. 27. Other autoantibodies: Antibodies to C1q, Lipoprotein, and Endothelial Cells - p. 384 84. 28. Pathogenesis of antiphospholipid antibody syndrome - p. 388 85. 29. Drug-induced lupus - p. 402 86. SECTION 5. Clinical Aspects of Lupus Erythematosus - p. 412 87. 30. Overview and clinical presentation - p. 413 88. 31. Skin disease in cutaneous lupus erythematosus - p. 421 89. 32. The musculoskeletal system and bone metabolism - p. 433 90. 33. Lupus and the cardiovascular system - p. 449 91. 34. Pulmonary manifestations of systemic lupus erythematosus - p. 463 92. 35. Lupus and the nervous system: Clinical Presentation, Diagnosis, and Treatment - p. 476 93. Classification of NPSLE - p. 476 94. Epidemiology of NPSLE - p. 479 95. Clinical presentation of NPSLE - p. 479 96. Diagnostic evaluation - p. 489 97. Neuroimaging studies in NPSLE - p. 492 98. Treatment of NPSLE - p. 496 99. Prognosis of NPSLE - p. 498 100. References - p. 498 101. 36. Gastrointestinal and hepatic manifestations - p. 505 102. 37. The gut microbiome and lupus pathogenesis - p. 521 103. 38. Hematologic and lymphoid abnormalities in SLE - p. 528 104. 39. Neonatal lupus: Pathogenesis and Clinical Approaches - p. 542 105. 40. Pregnancy and reproductive health issues in systemic lupus erythematosus - p. 557 106. 41. Systemic lupus erythemato sus in childhood and adolescence - p. 580 107. 42. Clinical aspects of antiphospholipid syndrome - p. 596 108. 43. Lupus and infections - p. 608 109. 44. Ocular, aural, and oral manifestations of lupus - p. 625 110. 45. Clinical aspects of Sjögren’s disease - p. 637 111. 46. Clinical presentations of lupus in the kidney and urogenital system - p. 655 112. 47. The endocrine system and hormones in lupus - p. 665 113. SECTION 6. Assessment of Lupus - p. 672 114. 48. Clinical application of serologic tests, serum protein abnormalities, and other laboratory tests in systemic lupus erythematosus - p. 673 115. 49. Differential diagnosis and disease associations - p. 693 116. 50. Incomplete lupus, undifferentiated connective tissue disease, and mixed connective tissue disease - p. 703 117. 51. Clinical markers, metrics, indices, and clinical trials - p. 710 118. SECTION 7. Management of Lupus - p. 726 119. 52. Principles of therapy, local measures, and NSAIDs - p. 727 120. 53. Antimalarial medications - p. 738 121. 54. Systemic glucocorticoid therapy for SLE - p. 750 122. 55. Immunosuppressive drug therapy - p. 763 123. 56. Complementary and integrative health approaches - p. 777 124. 57. Specialized and niche therapies - p. 784 125. 58. Adjunctive and preventive measures - p. 792 126. 59. Management of cutaneous lupus erythematosus - p. 802 127. 60. Targeted therapies in SLE - p. 810 128. 61. Management of lupus nephritis - p. 817 129. 62. Management of Sjögren disease syndrome - p. 838 130. SECTION 8. Outcomes - p. 852 131. 63. Socioeconomics, disparities, and disability - p. 853 132. 64. Prognosis and mortality of systemic lupus erythematosus - p. 864 133. 65. Future directions: Therapeutics - p. 880 134. 66. Expanding the opportunities to effectively treat patients: Clinical Considerations - p. 884 135. Index - p. 891
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.
1. Cover image - p. i 2. Title page - p. ii 3. Table of Contents - p. iii 4. Copyright - p. iv 5. Dedication - p. v 6. Preface - p. vi 7. Acknowledgments - p. vii 8. Contributors - p. viii 9. List of Illustrations - p. xv 10. List of Tables - p. xvi 11. SECTION 1. What Is Lupus? - p. xvi 12. 1. History of lupus - p. 1 13. 2. Definition and classification of lupus and lupus-related disorders - p. 12 14. 3. The epidemiology of lupus - p. 22 15. SECTION 2. Pathogenesis - p. 38 16. 4. Overview of lupus pathogenesis - p. 39 17. 5. Genetics of SLE - p. 49 18. 6. Single gene defects and autoinflammation: Relation to Lupus - p. 62 19. 7. Epigenetics of lupus - p. 74 20. DNA methylation in SLE - p. 74 21. Histone modification in SLE - p. 77 22. 3D genome and SLE - p. 80 23. RNA modification - p. 81 24. miRNA in SLE - p. 85 25. Roles of lncRNAs in SLE - p. 92 26. Roles of circrnas in SLE - p. 95 27. Translational application of epigenetics in lupus - p. 96 28. References - p. 100 29. 8. The role of the environment and microbiome in lupus - p. 108 30. 9. The innate immune system in SLE - p. 116 31. 10. B cells and generation of antibodies - p. 124 32. 11. T cells - p. 139 33. 12. Immunoregulatory cells and networks in lupus - p. 152 34. 13. Cytokines in lupus - p. 163 35. Properties of cytokines and their receptors - p. 163 36. Assessment of cytokine production - p. 164 37. Use of gene expression to study cytokine effects - p. 165 38. Activation of the immune response in systemic lupus erythematosus - p. 165 39. Cytokines of the innate immune response - p. 165 40. Cytokines of the adaptive immune response - p. 171 41. Summary - p. 174 42. References - p. 174 43. 14. Metabolic control of immunopathogenesis in systemic lupus erythematosus - p. 181 44. 15. Animal models of systemic lupus erythematosus (SLE) - p. 189 45. Multigenic spontaneous SLE - p. 189 46. MRL-lpr mice and MRL/Mp (MRL+/+) - p. 200 47. Nonfunctional Fas ligand in gld mice - p. 202 48. (NZW×BXSB) F1 model of antiphospholipid syndrome and coronary artery disease - p. 204 49. BXD2 RI model of spontaneous lupus nephritis and erosive polyarthritis (“rhupus”) - p. 204 50. Monogenic spontaneous SLE - p. 205 51. Induced lupus-like disease in nonautoimmune strains - p. 206 52. Lupus induced by injection of hydrocarbon oil - p. 207 53. Therapeutic interventions in mouse models of lupus - p. 208 54. Lupus in domestic animals - p. 216 55. References - p. 217 56. SECTION 3. Mechanisms of Tissue Injury - p. 234 57. 16. Abnormalities in clearance of immune complexes and dying cells in lupus - p. 235 58. 17. Complement and systemic lupus erythematosus - p. 245 59. Historical overview - p. 246 60. References - p. 259 61. 18. Apoptosis and inflammatory forms of cell death - p. 265 62. 19. Tissue damage in lupus: Free Radicals and Fibrosis - p. 277 63. 20. Lupus nephritis - p. 292 64. Renal anatomy and physiology - p. 292 65. Histologic classification of lupus nephritis - p. 296 66. Genetic risk for lupus nephritis - p. 297 67. Mouse models of lupus nephritis - p. 298 68. Mechanisms for immune complex deposition in the kidneys - p. 299 69. Pauci-immune glomerulonephritis - p. 300 70. Dissociation of immune complex deposition and/or glomerular injury from end-stage renal disease - p. 300 71. Effector mechanisms in the kidney - p. 300 72. Progression to fibrosis and sclerosis - p. 306 73. Systems biology of lupus nephritis - p. 308 74. Future directions in lupus nephritis - p. 310 75. References - p. 310 76. 21. Pathogenesis of accelerated atherosclerosis and vascular injury in systemic lupus erythematosus - p. 316 77. 22. Pathogenesis of cutaneous lupus - p. 329 78. 23. Pathogenesis of neuropsychiatric lupus - p. 338 79. 24. Pathogenesis of Sjögren syndrome - p. 349 80. SECTION 4. Autoantibodies - p. 365 81. 25. Antinuclear antibodies, antibodies to DNA, histones, and nucleosomes - p. 366 82. 26. Antibodies against extractable nuclear antige ns (RNP, Sm, SS-A/RO, and SS-B/La) - p. 378 83. 27. Other autoantibodies: Antibodies to C1q, Lipoprotein, and Endothelial Cells - p. 384 84. 28. Pathogenesis of antiphospholipid antibody syndrome - p. 388 85. 29. Drug-induced lupus - p. 402 86. SECTION 5. Clinical Aspects of Lupus Erythematosus - p. 412 87. 30. Overview and clinical presentation - p. 413 88. 31. Skin disease in cutaneous lupus erythematosus - p. 421 89. 32. The musculoskeletal system and bone metabolism - p. 433 90. 33. Lupus and the cardiovascular system - p. 449 91. 34. Pulmonary manifestations of systemic lupus erythematosus - p. 463 92. 35. Lupus and the nervous system: Clinical Presentation, Diagnosis, and Treatment - p. 476 93. Classification of NPSLE - p. 476 94. Epidemiology of NPSLE - p. 479 95. Clinical presentation of NPSLE - p. 479 96. Diagnostic evaluation - p. 489 97. Neuroimaging studies in NPSLE - p. 492 98. Treatment of NPSLE - p. 496 99. Prognosis of NPSLE - p. 498 100. References - p. 498 101. 36. Gastrointestinal and hepatic manifestations - p. 505 102. 37. The gut microbiome and lupus pathogenesis - p. 521 103. 38. Hematologic and lymphoid abnormalities in SLE - p. 528 104. 39. Neonatal lupus: Pathogenesis and Clinical Approaches - p. 542 105. 40. Pregnancy and reproductive health issues in systemic lupus erythematosus - p. 557 106. 41. Systemic lupus erythemato sus in childhood and adolescence - p. 580 107. 42. Clinical aspects of antiphospholipid syndrome - p. 596 108. 43. Lupus and infections - p. 608 109. 44. Ocular, aural, and oral manifestations of lupus - p. 625 110. 45. Clinical aspects of Sjögren’s disease - p. 637 111. 46. Clinical presentations of lupus in the kidney and urogenital system - p. 655 112. 47. The endocrine system and hormones in lupus - p. 665 113. SECTION 6. Assessment of Lupus - p. 672 114. 48. Clinical application of serologic tests, serum protein abnormalities, and other laboratory tests in systemic lupus erythematosus - p. 673 115. 49. Differential diagnosis and disease associations - p. 693 116. 50. Incomplete lupus, undifferentiated connective tissue disease, and mixed connective tissue disease - p. 703 117. 51. Clinical markers, metrics, indices, and clinical trials - p. 710 118. SECTION 7. Management of Lupus - p. 726 119. 52. Principles of therapy, local measures, and NSAIDs - p. 727 120. 53. Antimalarial medications - p. 738 121. 54. Systemic glucocorticoid therapy for SLE - p. 750 122. 55. Immunosuppressive drug therapy - p. 763 123. 56. Complementary and integrative health approaches - p. 777 124. 57. Specialized and niche therapies - p. 784 125. 58. Adjunctive and preventive measures - p. 792 126. 59. Management of cutaneous lupus erythematosus - p. 802 127. 60. Targeted therapies in SLE - p. 810 128. 61. Management of lupus nephritis - p. 817 129. 62. Management of Sjögren disease syndrome - p. 838 130. SECTION 8. Outcomes - p. 852 131. 63. Socioeconomics, disparities, and disability - p. 853 132. 64. Prognosis and mortality of systemic lupus erythematosus - p. 864 133. 65. Future directions: Therapeutics - p. 880 134. 66. Expanding the opportunities to effectively treat patients: Clinical Considerations - p. 884 135. Index - p. 891
