Preface, vii List of Contributors, viii SECTION 1 What Is Lupus? 1 History of Lupus, 1 2 Definition and Classification of Lupus and Lupus-Related Disorders, 15 3 The Epidemiology of Lupus, 23 SECTION 2 Pathogenesis 4 Overview of Lupus Pathogenesis, 44 5 Genetics of Human SLE, 54 6 Epigenetics of Lupus, 69 7 The Environment and the Host, 86 8 The Innate Immune System in SLE, 93 9 B Cells and Generation of Antibodies, 101 10 T Cells, 116 11 Adaptive and Innate Immunoregulatory Cells, 125 12 Cytokines in Lupus, 137 13 Immunometabolism, 153 14 Animal Models in Lupus, 164 SECTION 3 Mechanisms of Tissue Injury 15 Abnormalities in Immune Complex Clearance and Apoptotic Cell Clearance, 216 16 The Role of Complement in SLE, 224 17 Apoptosis and Inflammatory Forms of Cell Death, 237 18 Tissue Damage in Lupus: Free Radicals and Fibrosis, 248 19 Pathogenesis of Fibrosis-The Lung as a Model, 261 20 Pathogenesis of Lupus Nephritis, 269 21 Pathogenesis of Accelerated Atherosclerosis and Vascular Injury in Systemic Lupus Erythematosus, 294 22 Pathogenesis of Cutaneous Lupus, 305 23 Pathogenesis of Neuropsychiatric Lupus, 317 24 Pathogenesis of Antiphospholipid Antibody Syndrome, 324 25 Pathogenesis of Sjögren''s, 338 SECTION 4 Autoantibodies 26 Structure and Derivation of Autoantibodies, 340 27 Antinuclear Antibodies, Antibodies to DNA, Histones, and Nucleosomes, 355 28 Antibodies Against ENA (Sm, RNP, SSA, SSB), 366 29 Antibodies Against C1q, 372 30 Antilipoprotein and Antiendothelial Cell Antibodies, 375 31 Drug-Induced Lupus Erythematosus, 377 SECTION 5 Clinical Aspects of Lupus Erythematosus 32 Overview and Clinical Presentation, 389 33 Skin Disease in Cutaneous Lupus Erythematosus, 395 34 The Musculoskeletal System and Bone Metabolism, 407 35 Lupus and the Cardiopulmonary System, 418 36 Lupus and the Nervous System: Clinical Aspects, Psychopathology, and Imaging, 434 37 Gastrointestinal and Hepatic Systems, 457 38 Hematologic and Lymphoid Abnormalities in SLE, 473 39 Neonatal Lupus: Pathogenesis and Clinical Approaches, 486 40 Pregnancy and Autoimmune Disease, Reproductive and Hormonal Issues, 499 41 Systemic Lupus Erythematosus in Childhood and Adolescence, 520 42 Clinical Aspects of Antiphospholipid Syndrome, 532 43 Lupus and Infections, 543 44 Ocular, Aural, and Oral Manifestations of Lupus, 556 45 Clinical Aspects of Sjögren''s, 566 SECTION 6 Assessment of Lupus 46 Clinical Application of Serologic Tests, Serum Protein Abnormalities, and Other Clinical Laboratory Tests in Systemic Lupus Erythematosus, 579 47 Differential Diagnosis and Disease Associations, 598 48 Incomplete Lupus, Undifferentiated Connective Tissue Disease, and Mixed Connective Tissue Disease, 606 49 Clinical Markers, Metrics, Indices, and Clinical Trials, 614 50 Lupus Biomarkers, 631 SECTION 7 Management of Lupus 51 Principles of Therapy, Local Measures, and NSAIDs, 640 52 Antimalarial Medications, 650 53 Systemic Glucocorticoid Therapy for SLE, 661 54 Immunosuppressive Drug Therapy, 673 55 Complementary and Alternative Medications, Specialized and Niche Therapies, 689 56 Adjunctive Treatments and Preventive Measures, 702 57 Targeted Therapies in SLE, 710 58 Management of Cutaneous Lupus Erythematosus, 719 59 Epidemiology and Management of Lupus Nephritis, 727 60 Management of Sjögren''s, 745 SECTION 8 Outcomes 61 Socioeconomic and Disability Aspects, 759 62 Prognosis and Mortality of SLE, 770 Index, 782.