Dubois' Lupus Erythematosus and Related Syndromes

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

Daniel J Wallace MD, FACP, MACR is a board-certified internist and rheumatologist. A Clinical Professor of Medicine at the David Geffen School of Medicine at UCLA, he is the Associate Director of the Rheumatology Fellowship Program at Cedars-Sinai Medical Center, where he also serves on the Board of Governors. Dr. Wallace has authored nearly 450 peer reviewed manuscripts, 30 book chapters, eight textbooks (on lupus, osteoarthritis, Sjogren's syndrome and fibromyalgia) and his practice includes caring for 2,000 lupus patients, the largest lupus cohort in the United States. Dr Wallace is co-chair of the Lupus Industry Council of the Lupus Research Alliance, past chair of the Lupus Foundation of America, and has served on the Board of Directors of the United Scleroderma Foundation and the Medical Advisory Board of the Sjogren's Syndrome Foundation. He has been Chief of Rheumatology at the City of Hope and Cedars-Sinai Medical Center, and chairman of the Research and Education Foundation of the American College of Rheumatology. Dr. Hahn received her MD from the Johns Hopkins University School of Medicine, where she later trained in Rheumatology. She trained in internal medicine at Washington University in St. Louis. Dr. Hahn heads research groups that do basic studies in the immune abnormalities that cause systemic lupus erythematosus (SLE) and is developing interventions that help restore the immune response to normal. She also heads a group that studies clinical problems in patients with SLE, including accelerated atherosclerosis and potential new therapies (clinical trials). In addition, Dr. Hahn provides clinical consultations for patients with rheumatic diseases, particularly SLE. She also teaches Rheumatology and heads the administration of the Division of Rheumatology, which has 25 full-time faculty, 8 fellows and several undergraduate students, medical students, PhD students, postdoctoral trainees, and an administrative staff.