Table of Contents

Section I: What is Lupus?

1. Definition and classification of Lupus and Lupus-Related Disorders

2. The Epidemiology of Lupus

Section II: The Pathogenesis of Lupus

3. The Pathogenesis of SLE

4. Genetics of Human SLE

5. Epigenetics of Lupus

6. The Innate Immune System in SLE

7. Cytokines and Interferons in Lupus

8. The Structure and Derivation of Antibodies and Autoantibodies

9. T Cells

10. Regulatory Cells in SLE

11. Apoptosis, Necrosis, and Autophagy

12. Abnormalities in Immune Complex Clearance and Fc? Receptor Function

13. Neural-Immune Interactions: Principles and Relevance to SLE

14. Complement and SLE

15. Mechanisms of Acute Inflammation and Vascular Injury in SLE

16. Mechanisms of Tissue Damage - Free Radicals and Fibrosis

17. Animal Models of SLE

18. Pathogenic Mechanisms in Lupus Nephritis

Section III: Autoantibodies

19. Immune Tolerance Defects in Lupus

20. Autoantibodies

21. Autoantigenesis and Entigen-Based Therapy and Vaccination in SLE

Section IV: Clinical Aspects of SLE

22. Overview and Clinical Presentation

23. Pathomechanisms of Cutaneous Lupus Erythematosus

24. Skin Disease in Cutaneous Lupus Erythematosus

25. The Musculoskeletal System and Bone Metabolism

26. Pathogenesis and Treatment of Atherosclerosis in Lupus

27. Cardiopulmonary Disease in SLE

28. Pathogenesis of the Nervous System

29. Clinical Aspects of the Nervous System

30. Psychopathology, Neurodiagnostic Testing, and Imaging

31. Ocular, Aural, and Oral Manifestations

32. Management of Sj�gren Syndrome in Patients with SLE

33. Gastrointestinal and Hepatic Manifestations

34. Hematologic and Lymphoid Abnormalities in SLE

35. Clinical and Epidemiologic Features of Lupus Nephritis

Section V: The Reproductive System & Hormones

36. Pregnancy in Women with SLE

37. Neonatal Lupus Erythematosus

38. Reproductive and Hormonal Issues in Women with Autoimmune Diseases

Section VI: Special Considerations, Subsets of SLE and Lupus-Related Syndromes

39. Drug-Induced Lupus: Etiology, Pathogenesis, and Clinical Aspects

40. SLE in Childhood and Adolescence

41. Mixed Connective Tissue Disease and Undifferentiated Connective Tissue Disease

42. Clinical Aspects of the Antiphospholipid Syndrome

Section VII: Assessment of Lupus

43. Clinical Application of Serologic Tests, Serum Protein Abnormalities, and Other Clinical Laboratory Tests in SLE

44. Differential Diagnosis and Disease Associations

45. SLE and Infections

46. Clinical Measures, Metrics and Indices

Section VIII: Management of SLE

47. Principles of Therapy, Local Measures, Nonsteroidal Medications

48. Systemic Glucocorticoid Therapy in SLE

49. Antimalarial Medications

50. Immunosuppressive Drug Therapy

51. Specialized Treatment Approaches and Niche Therapies for Lupus Subsets

52. Adjunctive and Preventive Measures

53. Novel Therapies for SLE: Biological Agents Available in Practice Today

54. Critical Issues in Drug Development for SLE

55. Socioeconomic and Disability Aspects

Section IX: Outcomes and Future Considerations

56. Investigational Agents and Future Therapy for SLE

57. Mortality in SLE

Appendix: Lupus Resource Materials

Index

About the Author

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.

Reviews

"This is a comprehensive and extensive review of the definition, pathogenesis, serological/clinical manifestations, and current management guidelines of these syndromes. Chapters are organized well, for example, the clinical manifestations section is divided by organ system. Also helpful is the separate chapter on pregnancy in women with SLE, an issue I deal with on a regular basis. This is a thorough reference on lupus erythematosus, covering every aspect of this heterogeneous systemic autoimmune disease. " - Maria Gonzalez-Mayda, M.D, Washington University Medical Center 4 Star - Doody's Rating, March 2013