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Ajohns Hopkins Press Health Book
Do n a l d E. Th o m a s� Jr.
MD1 FACP1 FACR

Lupus
E N C YCL O P E DIA

A Comprehensive
Guide for Patients
and Families

JOHNS HOPKINS UNIVERSITY PRESS BALTIMORE

Note to the reader. This book is not meant to substitute for medical care of people who have lupus, and
treatment should not be based solely on its contents. Instead, treatment must be developed in a dialogue
between the individual and his or her physician. This book has been written to help with that dialogue.
The author and publisher are not responsible for any adverse consequences resulting from the use of in·
formation in this book.

Drug dosage. The author and publisher have made reasonable efforts to determine that selection and dos·
age of drugs discussed in this text conform to the practices of the general medical community. The medi·
cations described do not necessarily have specific approval by the U.S. Food and Drug Administration for
use in the diseases and dosages for which they are recommended. In view of ongoing research, changes
in governmental regulations, and the constant flow of information relating to drug therapy and drug re·
actions, the reader is urged to check the package insert of each drug for any change in indications and
dosage and for warnings in precautions. This is particularly important when the recommended agent is a
new and/or infrequently used drug.

© 2014 Johns Hopkins University Press

All rights reserved. Published 2014

Printed in the United States of America on acid·free paper
987 654321

Johns Hopkins University Press

27 15 North Charles Street
Baltimore, Maryland 21218-4363
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Library of Congress Cataloging-in-Publication Data

Thomas, Donald E., Jr., 1961-

The lupus encyclopedia: a comprehensive guide for patients and families I Donald E.
Thomas,Jr., M.D., FACP, FACR.
pages em. - (A Johns Hopkins Press health book)
Includes bibliographical references and index.
ISBN 978-1-4214-0983-2 (hardcover: alk. paper)- ISBN 1-4214-0983-6 (hardcover:
alk. paper)- ISBN 978-1-4214-0984-9 (pbk.: alk. paper)- ISBN 1-4214-0984-4
(pbk.: alk. paper)- ISBN 978-1-4214-0985-6 (electronic)- ISBN 1-4214-0985-2
(electronic) 1. Systemic lupus erythematosus-Encyclopedias. I. Title.
RC924.5.L85T46 2013
616.7'72003-dc23
2012042648

A catalog record for this book is available from the British Library.

Figures 1.1, 10.1, 11.1, 12.1, 12.2, 13.1, 14.1, 16.1, 17.1, 21.1, 21.2, 24.1, 24.2, 26.1, 27.1,
28.1-28.3, 30.1, 30.2, 33.1, 37.1-37.3, and 38.1 and those on pages 255, 568, 570, and 572
are by Jacqueline Schaffer.

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DEDICATED . . .

To my parents, Donald and Nancy Thomas, who worked very hard all
their lives in very difficult, demanding jobs in factories. The many hours
that they spent working double shifts so their family of six children could
benefit in the end are priceless; I will never be able to repay them for their
sacrifices. It is because of these sacrifices that I am able to live the Ameri­
can Dream. I am lucky to have them as my parents.
To my grandparents, L. Ross and Laura Thomas. Ever since I was a
small child, they told me I could do whatever I put my mind to. When
I thought I was stupid, they told me I was smart. A big turning point in
my life was in the fifth grade; I was in the remedial reading class due to
problems during my fourth-grade year. They urged me to strive beyond
my preconceived abilities. Taking on their challenge, I memorized a long
poem about a pirate. I got up in front of the class and recited it by heart
without missing a line. My teacher, Mrs. Wine, took me by the hand, led
me to the "smart" reading classroom, and told me that that was where I
belonged. How empowering an event that was. I never looked back. In fact,
I have made it a point throughout my life to encourage others who are not
performing up to their abilities to do the same in their lives. My grandma
almost died of asthma when I was young. I told her that I was going to be­
come a doctor to help people like her, and that is what I have done. I wish
my grandparents were alive today to see what I am doing. I know that they
are looking down on me from heaven with a smile. Thanks, Grandma and
Grandpa, your memory lives on.
To one of my best friends in high school, Gordon Blue, his parents
(Ruth and Carlos), and all the Blue sisters. When I was in college, I ran out
of money and had to drop out of school at a critical point in my educa­
tion. When I had nowhere else to turn, the Blue family took me into their
home. They knew I wanted to become a doctor and they wanted to help
me. Living with them in the Houston area gave me the chance to work, get
back on my feet, and finish college. By fortune, it facilitated my ability to
be accepted to and attend one of the best medical schools in the United
States, Baylor College of Medicine. The Blues are my second family and I
am indebted to them for extending to me their hand and love.
To my Bengal cat, Adam; my African grey parrot, Timmy; my Coton
de Tulear dog, Citeaux; and my newest puppy, Musette. They can be my
biggest fans and supporters. I would be so exhausted and worn out from
writing on many a weekday night and long weekend, yet their uncondi-
tional love and snuggling would always revive me, renew me with energy,
and replace my tired frown with a smile!
Most importantly, I dedicate this to those stricken with lupus who
have succumbed to the disease and its complications. I will never forget
the teenager who died of severe lupus in our county hospital many years
ago because she and her family could not afford proper healthcare. By the
time I saw her, it was too late. I will never forget how she told me she was
not going to live and how I grieved alongside her loving mother. I hope our
healthcare system will continue to improve so that people do not have to
die from this disease simply because they come from families with little
money. To my neighbor Tim's mother: I never knew her, but he talks about
how she died of lupus and its complications during a time when therapies
were not nearly as good as they are today. I dedicate this book to all lupus
patients who have not survived the fight. I hope that the current research
and new therapies for lupus will prevent the deaths of lupus patients in
the not too distant future. I know this book can give to you, the reader,
the knowledge and wisdom that can protect you from a similar fate. Keep
reading on.
 CONTENTS

List of Illustrations ix
List of Tables xi
Foreword, by GEORGE C . TSOKOS, M D XVii
Preface xxi
Acknowledgments xxix

PART 1 Defi n itions, Ca uses, and Diagn osis of Auto i m m u n e Disease

1 . What I s Lupus and How I s It Diagnosed? 3

2. The Other Systemic Autoimmune Diseases 24

3. What Causes Lupus? 37

4. The Meaning of All Those Test Results 51

PART 11 H ow Lu p u s Affects the Body

5. How SLE Directly Affects the B o dy 89

6. Constitutional Symptoms 95

7. The Musculoskeletal System 103

8. Skin and Mucous Membranes 129

9. Blood and Lymph Systems 147

10. The Respiratory System 166

11. The Heart and Blood Vessels 181

12. The Urinary System 196

13. The Nervous System 213

14. The Exocrine Gland System 240

15. The Digestive System 254

16. Eyes 265

17. The Endocrine System 269

18. The Reproductive System and Pregnancy 27 2

19. Special Populations and Surgery 283

20. Prognosis 296

PART 111 Other C o m p l ications of Lu p u s and Their Treatments

21. Heart Attacks and Strokes 309

22. Infe ctions 331

23. Cancer and SLE 344

24. O steoporosis 352

25. Avascular Necrosis of B one 390

26. Adrenal Insufficiency 397

27. Fibromyalgia 405

28. Gastroesophageal Reflux and Stomach Ulcers 443

PART IV Treating Lu p u s

29. General Treatment of Lupus 463

30. Anti-Malarials 494

31. Steroids 522

32. Immunosuppres sants 542

33. Biologic Agents 567

34. Benlysta: The First FDA-Approved Drug for Lupus i n More

Than Fifty Years 586
35. Other Therapie s for SLE 605

36. Prescription Pain Medicines 618

37. Future Treatments for SLE 639

38. Nonmedical Therapies for Lupus 662

39. C omplementary and Alternative Medicine 683

PART v Practical Matte rs

40. Talking to Your Doctor and D eciphering Symptoms 7 05

41. Becoming Pregnant, Breast-Feeding, and Using

C ontraception 732
42. Health Insurance and Afford i ng Healthcare 747

43. Working and Dealing with Disability 766

44. The Lupus S ecrets Checklist 788

Patient Resourc e s 7 91

Bibliography 807

Index 843
 ILLUSTRATIONS

FIGURES

Saint Martin and Saint Brice curing the bishop of Liege of lupus 4

1.1. How the immune system causes inflammation 7

3.1. The causes of lupus 39

10.1. The respiratory system 167

11.1. The heart 182

11.2. Normal artery compared to SLE artery with cold exposure caused
by Raynaud's 193
12.1. The urinary system 197

12.2. Parts of the nephron 197

13.1. The central nervous system and peripheral nervous system 214

14.1. Major salivary glands 247

The digestive system 255

16.1. The eye 266

17.1. The thyroid gland 27 0

20.1. Effects of therapies on survival in people who have SLE 298

21.1. Normal artery and artery with plaque 311

21.2. Measuring waist and hip circumferences 319

24.1. Normal bone compared to bone with osteoporosis 354

24.2. Effects of vertebral fractures due to osteoporosis 355

26.1. Locations of the hypothalamus, the pituitary gland, and the adrenal
gland s 399
27.1. Tender points of fibromyalgia used in the 1990 ACR classification
criteria 415
28.1. How the lower esophageal sphincter works to prevent GERD 445

28.2. Hiatal hernia 447

28.3. Possible locations of peptic ulcers 452

29.1. Weighing the risks versus the benefits of a medicine 474

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I L L U S T R AT I O N S

29.2 . The benefits of Fosamax versus the potential risk of osteonecrosis of

the jaw 483
30.1. Anti-malarial Plaquenil interferes with skin antigen presentation to
T-cells 500
30.2. Amsler grid 511
Cytokines causing normal immune response with inflammation 568
Normal inflammation versus inflammation from overproduction
of inflammatory cytokines 570
Overproduction of inflammatory cytokines versus the effect ofTNF
inhibitor treatment 572
33.1. How Rituximab works on the immune system in SLE 581
37.1. How lupus occurs 641
37.2. Current therapies to treat lupus 642
37. 3 . Where future therapies may help lupus 643
38.1. Electromagnetic wave spectrum 663
38.2. MyPlate recommendations of a proper diet by the USDA 675
39.1. ACR-20 response in people who have rheumatoid arthritis 685
39.2. ACR-20 response in people who have rheumatoid arthritis: typical
placebo effect 686

PHOTOGRAPHS

8.1. Malar erythema 131

8.2. Subacute cutaneous lupus erythematosus, annular form 132
8.3. Discoid lupus erythematosus with scarring alopecia 134
8.4. Lupus profundus (lupus panniculitis) 135
8.5. Non-scarring alopecia before (A) and after (B) treatment 138
8.6. Livedo reticularis 140
8 .7. Telangiectasias 141
8.8. Erythromelalgia 142
8 .9. Vitiligo 144
8.10. Dark patches of skin due to Plaquenil 145
11.1. Raynaud's phenomenon 192
14.1. Schirmer test to diagnose dry eyes in Sjogren's syndrome 243
World War II Atabrine (quinacrine) bottles 497
30.1. Hull's eye maculopathy 507

X
 TABLES

1.1. Types of Lupus 12

2.1. Systemic Autoimmune Diseases 27
2.2. Similar Problems Caused by Systemic Autoimmune Diseases 28
3.1. How the Children of People Who Have SLE Can Try to Prevent Lupus 42
4.1. Lab Tests Most Helpful in Making a Diagnosis of SLE 54
4.2. Lab Tests Most Helpful in Following Disease Activity in SLE 55
5.1. Some Pred ictors of Possible Problems in SLE 91
6.1. How to Help Decrease Your Fatigue and Increase Your Energy Level 97
6.2. Sleep Hygiene Techniques 98
6 .3. STOP Questionnaire for Ob structive Sleep Apnea 99
7.1. How to Decrease Aches and Pains from Lupus 1 10
7.2. General Joint Protection Techniques 111
7. 3 . Joint Protection Techniques for Specific Parts o f the Body 112
7.4 . Dosing o f Acetam inophen (Tylenol) 1 2 1
7. 5. Potential Side Effects of Acetaminophen (Tylenol) 1 2 2
7.6 . Over-the-Counter NSAIDs 123
7.7. Potential Side Effects of NSAIDs 125
7. 8. Potential Side Effects of Capsaicin 127
8.1. Types of Cutaneous Lupus (Lupus-Specific Rashes) 130
8.2. Nonspecific Rashes Seen in People Who Have SLE 137
11.1. Non-Medicine Ways to Treat Raynaud's Phenomenon 194
12.1. Lupus Nephritis Biopsy Classifications 204
12.2. ACE Inhibitors and ARBs Used in Lupus Nephritis 210
13.1. ACR Neuropsychiatric Syndromes Observed in SLE 215
13.2. GAD-7 Screening Test for Anxiety 227
1 3.3. Ways to Cope with Cognitive Dysfunction 230
13.4. PHQ-9 Depression Questionnaire 233
14.1. Exocrine Gland Involvement in Sjogren's Syndrome 241
14.2. Symptoms of Dry Eyes in Sjogren's Syndrome 242

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14. 3 . General Measures to Alleviate Dryness in Sjogren's Syndrome 244

14.4. Keeping the Eyes Moist in Sjogren's Syndrome 245
14. 5 . Symptoms of Dry Mouth in Sjogren's Syndrome 246
14.6. Keeping the Mouth Moist in Sjogren's Syndrome 249
14.7. Keeping the Inner Nose Moist in Sjogren's Syndrome 250
14. 8 . Treating Itchy, Dry Ear Canals in Sjogren's Syndrome 251
14.9. Treating Dry, Itchy Skin in Sjogren's Syndrome 252
14.10. Treating Vaginal Dryness in Sjogren's Syndrome 252
15.1. Measures to Help with Esophageal Dysmotility and GERD 257
18.1. Factors That Increase the Risk for Complications during Pregnancy
in SLE 274
19.1. Surgical Preparation Checklist Specifically for People Who Have SLE 289
19.2. Checklist of What to Bring to Your Surgeon and Anesthesiologist 290
19.3. Medication Stop and Restart Dates for Surgery 291
20.1. Factors Associated with Increased Mortality in SLE 301
21.1. How Diabetes Is Diagnosed 316
21.2. BMI 318
21.3. Definitions of Obesity 320
21.4. Statins Used to Control Cholesterol Levels 329
22.1. How to Prevent Infections in SLE 335
22.2. Vaccines Containing Live, Attenuated Organisms 339
23.1. Cancers in SLE 345
23.2. Recommended Cancer Screenings 350
24.1. Risk Factors for Having Osteoporosis 356
24.2. Drugs That Can Cause Decreased Bone Strength 358
24.3. Medical Diseases That Can Cause Osteoporosis 359
24.4. Reasons Most People Who Have Lupus Are Deficient in Vitamin D 360
24.5. Using the T-score from a DXA Scan to Diagnose Osteoporosis 362
24.6. Non-Medicine Ways to Treat and Prevent Osteoporosis 366
24 .7. Calcium-Rich Foods 367
24.8. Vitamin D Content of Food 369
24.9. Preventing Falls 370
24.10. Bisphosphonates Used to Treat Osteoporosis 372
24.11. Potential Side Effects of Bisphosphonates 375
24.12. Potential Side Effects of Teriparatide (Forteo) 380
24.13. Potential Side Effects o f Denosumab (Prolia) 382
24.14. Potential Side Effects of Raloxifene (Evista) 384
24.15. Potential Side Effects of Calcitonin Nasal Spray (Miacalcin, Fortical) 386
25.1. Causes of AVN Other Than High-Dose Steroids 392

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 TA B L E S

26.1. Symptoms Suggesting Adrenal Insufficiency Due to Steroid Withdrawal 401

26.2. People Who Probably Have Adrenal Insufficiency Due to Steroids 402
27.1. Common Symptoms ofFibromyalgia 408
27.2. Other Common Pain Problems in People Who Have Fibromyalgia 409
27. 3 . Abnormal Find ings in Research Stud ies of People Who Have Fibromyalgia 413
27.4. Treatments Used for Fibromyalgia 417
27. 5 . Possible Benefits of Some Fibromyalgia Medicines over Others 421
27.6. Tricyclic Antidepressants (TCAs) 423
27.7. Potential Side Effects of TCAs 425
27.8. Selective Serotonin Reuptake Inhibitors (SSRis) Stud ied in Fibromyalgia 427
27.9. Potential Side Effects of SSRis 429
27.10. Potential Side Effects of SNRis 433
27.11. Potential Side Effects of Pregabalin and Gabapentin 436
27.12. Potential Side Effects of Cyclobenzaprine 438
27.13. Potential Side Effects of Tramadol 441
28.1. Causes of Gastroesophageal Reflux Disease (GERD) 446
28.2. Potential Complications of Gastroesophageal Reflux Disease 449
28.3. Proton Pump Inhibitors 451
28.4. Risk Level for Getting Ulcers When Taking NSAIDs 456
28.5. Strategies to Prevent Ulcers While Taking NSAIDs 457
29.1. Immunomodulating Medicines Used to Treat SLE 469
29.2 . Med ications Other Than Immunomodulatory Medicines Used to Treat
Problems from SLE 469
29. 3 . Medicines Used to Prevent or Treat Side Effects of Medicines Used in SLE 471
29.4. Multiple Medications Used in a Patient Who Has SLE 472
29. 5. Frequency of Side Effects Seen in a Research Study on a Common Arthritis
Medication 475
29.6. Frequency of Side Effects Seen in a Research Study on C elebrex 478
29.7. Evaluation of Potential Side Effects of Drugs Compared to Placebo 481
29.8. Strategies to Ensure That You Take Your Med ications Regularly 4 85
29.9. Medication List Form Example 489
29.10. Disposing of Medications 492
History of the Use of Anti-Malarial Medicines in Lupus 495
30.1. Benefits of Anti-Malarial Medicines in People Who Have Lupus 503
30.2. Recommended Dosing of Hydroxychloroquine Based on Height-Determined
Ideal Body Weight 508
30.3. How to Ensure That You Do Not Get Hydroxychloroquine Retinopathy 510
30.4. Potential Side Effects of Hydroxychloroquine 514
30.5. Potential Side Effects of Chloroquine 517
30.6. Potential Side Effects of Quinacrine 520

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TA B L E S

31.1. Reasons Steroids Are Used in Lupus 524

31.2. Systemic Steroids Most Commonly Used to Treat Lupus 526
31.3. Potential Side Effects of Systemic Steroids (Pill or IV Form) 528
31.4. How to Prevent Side Effects from Steroids 537
Chronology of Immunosuppressant Medicines in SLE 543
32.1. Potential Side Effects of Cyclophosphamide 547
32.2. Potential Side Effects of Azathioprine 550
32.3. Potential Side Effects of Methotrexate 554
32.4. Potential Side Effects of Cyclosporine 557
32.5. Potential Side Effects of Mycophenolic Acid 560
32.6. Potential Side Effects of Leflunomide 563
32 .7. Potential Side Effects of Tacrolimus 565
Biologic Agents versus Chemical Agents 573
33.1. TNF Inhibitors 575
33.2. Potential Side Effects of TNF Inhibitors 578
3 3 .3 . Potential Side Effects of Rituximab 584
34.1. History of U.S. Drug Regulations 588
34.2. Drugs FDA-Approved to Treat Lupus 591
34.3. Why It Is Difficult to Develop New Drugs for SLE and Get FDA
Approval 592
34.4. Effects of Placebo Group versus Benlysta in the BLISS-76 Study 598
34.5. Side Effects Reported in the Major Benlysta Studies Used for
FDA Approval 601
34.6. Potential Side Effects o f Benlysta 601
35.1. Potential Side Effects of Dapsone 607
35.2. Potential Side Effects of Thalidomide 609
35.3. Potential Side Effects of Danazol 610
35.4. Potential Side Effects of DHEA 612
35.5. Potential Side Effects of iVIG 614
35.6. Potential Side Effects of Plasmapheresis 616
36.1. Prescription NSAIDs 620
36.2. Potential Side Effects of NSAIDs 623
36.3. Potential Side Effects of Tramadol 627
36.4. Scheduled Opioid Drugs 630
36.5. Potential Side Effects ofOpioids 632
36.6. Risk Factors for Developing Psychological Addiction to Opioid
Med ications 635
36.7. Signs of Psychological Addiction to Opioid Med ications 636
37.1. Potential Side Effects of Abatacept 645
37.2. Potential Side Effects of Cholecalciferol 647

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 TA B L E S

37. 3 . Potential Side Effects of Sirolimus 649

37.4. Potential Side Effects of Stem Cell Transplantation 651
37. 5 . Potential Side Effects of Tocilizumab 652
37.6. Potential Side Effects ofUVA-1 Phototherapy 656
38.1. Occupations at Potentially Increased Risk of UV Light Exposure 666
38.2. Devices Emitting Higher Amounts of UV Radiation 666
3 8 . 3 . Ultraviolet Light Protection Measures 667
38.4. Why C igarette Smoking Is Bad for Lupus 669
3 8 . 5 . Recommended Diet in Lupus 671
38.6. Recommended Daily Diet by the U.S. Department of Agriculture 675
38 .7. Benefits of Regular Exercise in Lupus 677
38 .8. Stress Reduction Techniques 680
39.1. Complementary Therapies Sponsored by the NCCAM 689
39.2. How to Choose a Naturopathic Doctor 692
39.3. Dietary Supplements Possibly Helpful in Some People Who Have SLE 694
40.1. How to Make the Most of Your Doctor Visits 707
40.2. How to Describe a Medical Problem to Your Doctor 713
40.3. If You Have Lupus and You Develop One of These Symptoms, When Do You
Need to Seek Immediate Medical Attention? 718
41.1. Calculating Risk for Pregnancy Complications during Pregnancy 733
41.2. Medications Used to Treat Lupus and How They Are Managed during
Pregnancy 737
41 .3. Decreasing the Risk of Lupus Flares and Pregnancy Complications during
Assisted Reproduction, Ovarian Stimulation, and IVF 742
41.4. Potential Benefits of Breast-Feeding for Babies and Their Mothers 743
41.5. Medications Used to Treat Lupus during Breast-Feeding 744
41.6. Women Who Have Lupus Who Should Not Take Oral Contraceptive Pills 745
42.1. Prescription Drug Assistance Organizations 757
43.1. Potential Requests of Employers to Ensure a Better Work Environment 768
43.2. Difficulties Facing People Who Must Go on Disability 771
43.3. Companies That Offer Health Insurance to Part-Time Employees 773
43.4. Documents Required When Applying for Social Security Disability 774
43.5. What Items Need to Be Added to Your Disability Note by Your Doctor 781

XV
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 FOREWORD

How do you understand, much less solve, a problem like lupus? Even a quick scan
through the pages of this book will tell you that you are about to undergo a crash
course in medicine, including nearly every medical specialty. How do you grasp such a
complex disease, one that can involve every system in the body? Lupus was first recog­
nized as a disease of the skin in the m id-nineteenth century, but the systemic nature of
the disease was not appreciated until the 1940s. It took practically the entire twentieth
century to complete the list of primary clinical and laboratory manifestations of the
disease and the conditions that occur along with lupus.
Where does such a clinically variable disease start? Definitely not from just a single
point. This book guides you through many factors that contribute to the expression of
the disease. The first is genes. As researchers advance in their understanding of the
nature of the human genome, they believe that dozens of genes may be involved.
Another factor is environmental influences, including drugs, toxins (people who
have lupus should never smoke, as this book stresses several times), and radiation.
Science is now shedding light on how the environment influences gene expression. As
there is nothing that we can do about the nature of the genes that our parents "chose"
to give us, there is hope that, not too far in the future, we can control how genes are
expressed.
Then comes the factor of hormones. Hormones clearly play a role because most of
the people who have lupus are women. Still, we do not know how exactly hormones
instruct the immune system to go awry against itself, and most of the studies so far are
circular, without any useful insights into this mechanism.
Next come the factors that interfere with the function of the immune system. Sim­
ply put, the immune system loses its supervisory skills and fails to control self-de­
structive elements. But this failure comes along with an inherent limitation to fend off
infections. This book teaches how infections remain among the main causes of death
and how proper vigilance and prompt treatment of infections prevent days spent in
the hospital and prolong a good life for patients.
From my perspective it seems that each person who has lupus starts from a differ­
ent point and follows a unique set of pathways in developing lupus . Many of the path­
ways overlap, and as Dr. Thomas reports in this book, people who have lupus all have
in common auto-antibodies, immune complexes, and cells with reactivity against
their own elements. Something that I would like to add is that each organ that be­
comes involved offers its own special mooring facilities.

XVI I
F O R E W O R D

My experience was similar to that shared by Dr. Thomas in his preface. I was as­
signed to participate in the care of a 24-year-old woman when I was a senior in medical
school. She had fevers, chest pain, and swollen joints and legs. Systemic lupus erythe­
matosus was on the top of the list of possibilities , and the subsequently discovered
presence of antinuclear antibodie s , anemia, and low platelet and lymphocyte counts
helped seal the diagnosis. The treatments in those days were not nearly as good as
they are today, and we had to depend on giving her large doses of prednisone, which
unfortunately gave her a whole set of other problems in addition to her lupus. Her face
is still vivid in my memory. At the time, she was engaged to be married and wanted to
live a normal life. Her anxious and inquisitive eyes have continued to ask me, "Will you
find a cure for me? " Now, decades later, doctors can diagnose patients with the disease
faster, treat them more effectively, m inimize drug-related side effects, and take better
and faster care of infections and other conditions linked to lupus. Doctors can prom­
ise a normal and lengthy life to most people who have systemic lupus but cannot be
complacent without positively answering the proverbial "Are we there yet? "
During the last half-century, intense study of the immune system granted us a bet­
ter understanding of how the immune system develops , how it does its job, and how
it malfunctions in people who have autoimmune diseases and in particular systemic
lupus erythematosus. Clinical investigators and the pharmaceutical industry alike
have taken advantage of this knowledge and have tried to develop novel drugs to better
control lupus. As you will learn, the last time the FDA approved a drug for lupus , prior
to the recent approval of Benlysta, was in the late 1950s. Fair to say that the approved
use of steroids and anti-malarials, both with clear ability to control the immune sys­
tem, should not subtract from the value of cyclophosphamide and mycophenolic acid
(CellCept) in treating people who have lupus nephritis. The approval of Benlysta was
welcomed with great fanfare despite the fact that its effect was slow to come and the
trials somehow did not include enough of those who suffer more from the disease.
There has been much discussion among lupus experts addressing the question of
why doctors do not have more new drugs for lupus patients. Many new biologic drugs
have seen the light of approval at the FDA courts for rheumatoid arthritis, which is a
closely related cousin to lupus. There is no one correct answer. Common arguments
used include the clinical diversity of the disease, the lack of tools to measure disease
activity with sufficient fidelity, and the inherent difficulty in designing proper clini­
cal trials. Notwithstanding the reasons for the failure to enlist more approved drugs
for lupus, doctors become dependent on using therapies that are not FDA-approved to
treat systemic lupus.
Dr. Thomas and I met for the first time when he was a fellow in rheumatology and
I was a young attending physician at the Walter Reed Hospital in Washington, DC. He
and I share a strong interest in understanding the nature of the disease and the top­
notch care of patients who have lupus. Walter Reed allowed us to take care of the inter­
ests of the patients without ever needing to be concerned about the cost and the ability
of the patients to afford care. Lupus is a demanding partner in the life of the patient,
and you will soon learn that prompt diagnosis, close care by specialists, tight control
of medications and their side effects, and tenacious efforts to control comorbidity (in­
fections , cardiovascular disease, and cancer) make all the difference in the world .

XVI I I
 F O R E W O R D

Doctors are continuously pressed to shorten their encounter with their patients
while the documentation component of patient care looms continuously larger. And
this could be fine in the wisdom of the regulators should the doctors deal with sim­
ple, self-limited, one-dimensional diseases. Obviously, the Procrustean (one fits all)
approach is damning to patients who have lupus and their doctors. The doctors have
little time to go over the many dimensions of the disease that each patient brings to
each office visit and no time to educate them properly. Doctors who care deeply about
their patients know very well that it is the patients who take charge of their diseases
who fare the best. They come to the office prepared, and it is their doctors' duty to
educate them further. They bring knowledge acquired from the internet (usually un­
filtered and noncritical), and their doctors are pressed to transfer scientific knowledge
to properly fill in the gaps. It is incumbent upon the healers to translate the medical
information to the patients so patients can maximize the earned benefit. In these en­
counters much is lost in the translation that compromises the welfare of the patient.
Dr. Thomas m ight well have majored in classics, as he does a beautiful job in trans­
lating all Greek- and Latin-infested medical jargon into clear English (his writing is
the Orwellian clean windowpane). Patients want to be educated and The Lupus Ency­
clopedia will provide them with tools to fight the Wolf of the American writer Flan­
nery O'Connor (chapter 20). People who have lupus and their primary care doctors will
find in this book all the information they need-and the information is not lost in
translation!

GEORGE C. TSOKOS, MD
Professor of Medicine, Harvard Medical School
Chief, Division of Rheumatology
Beth Israel Deaconess Medical Center
Boston, Massachusetts

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 PRE FACE

Keep reading this preface! I suspect that most modern readers in our fast-paced,
social networking world do not read the preface to a book. They would rather just dive
right in. But if you or someone you care about has lupus, the information and advice in
this preface could be lifesaving.
Did you know that . . .

cigarette smoking decreases the effectiveness of the most important therapy used
to treat lupus?

eating alfalfa sprouts and mung bean sprouts can make lupus worse?

eating foods such as walnuts, olive oil, and flax seed may potentially be helpful in
lupus?

taking Echinacea (an herb used to "treat" colds) can make lupus worse?

any source of ultraviolet light, including indoor lighting, makes lupus worse?

the newer, environmentally friendly home light bulbs (compact fluorescent bulbs)
give off more ultraviolet light than the older incandescent light bulbs and can
make lupus more active?

you are exposed to less ultraviolet light from the sun if you drive with your car
windows up as opposed to driving with them down?

memory problems and fatigue are very common in people who have lupus, and
there are ways to look for their causes and therefore potentially help decrease their
severity?

most people who have systemic lupus erythematosus will live a long, normal life,
especially if they know the right things to do to keep their lupus under control?

if someone who has systemic lupus die s , it more commonly is due to a heart
attack, stroke, or infection instead of the lupus itself?

broken bones from osteoporosis are potentially deadly (a silent killer), are
common in lupus, and are preventable?

annoying dry mouth, dry eyes, or dry skin may be important clues for an under­
recognized complication of lupus called Sjogren's syndrome?

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P R E FAC E

20% of people who have lupus will have severe pain, profound fatigue, and trouble
with memory due to a condition called fibromyalgia?

if you take medicines to calm down the acidity of your stomach, then you should
be taking a specific type of calcium supplement?

I have had a special interest in lupus for more than twenty years after I saw a young
woman ravaged by severe kidney disease from lupus, and I witnessed the troubling
complications that she had experienced from high doses of steroids. I felt a sense of
sadness and helplessness in caring for this young person who should have been liv­
ing her dreams and aspirations instead of spending days at a time in a hospital bed. I
asked, "Why does the immune system of a person who has lupus decide to attack the
very body to which it belongs? What can we do to keep lupus from damaging the body
and give people their lives back? " Those questions drove me to become a rheumatolo­
gist (the specialist who most commonly treats lupus).
Over the years, I realized that there were many things that a person who has lupus
needs to do in order to do well. Lupus is an immensely complex disease, so there were
too many things to teach a person who has lupus during the brief time allotted at doc­
tor appointments. Therefore, I came up with a list of "do's and do not's" that I called
the Lupus Secrets. I named them "secrets" not because doctors and nurses are trying
to hide them from patients but because, even though they were very important mea­
sures that all lupus patients needed to do to stay healthy, most patients did not know
about them. Doctors and nurses as health care providers need to focus so much on the
physical examination, lab test results, and medicines that they often do not have the
time to discuss these other important aspects of lupus care. Yet knowing and practic­
ing these lesser-known facts can make the difference between living a good quality of
life with lupus or letting the lupus control one's life. Therefore, over the years I have
given the Lupus Secrets to my patients as an easy-to-read, concise, important handout.
I have amended the handout over the years based on the latest medical information. I
have reproduced it in chapter 44 of this book. People who have lupus and want to be in
more control of it should practice these " secrets."
It is much easier to practice these measures if you know why they are important in
the first place. Therefore, this book goes into detail about why they are important, as
well as giving out practical advice on how to incorporate these strategies into your dai­
ly life. For example, most people who have lupus know it is important not to be out in
the sun. Yet it is important to learn how to decrease ultraviolet exposure from sources
other than just the sun; chapter 3 8 explains these important concepts thoroughly.
During my career, I have been saddened when I have seen people who have lupus
not do well or, even worse, die. Most of these cases were completely preventable. I do
not mean to m inimize how severe and devastating this disease can be because it can
be deadly. Yet most people who I have witnessed die from their systemic lupus did not
receive adequate treatment. One was a young woman who had no access to adequate
health care, which reflects many of the inadequacies of the current health care system.
Her situation was truly a sad event for me to witness. Most of the others were due to the
patients them selves deciding not to take the advice of their physicians. Instead, they
decided to rely on herbs and natural therapies. Others were afraid of the side effects

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of the medicines their doctors were recommending. If these people could have been
more educated in what lupus is, what causes it, what happens when it is treated versus
when it is not, and how to put the potential side effects of medicines into perspective,
then maybe they would have accepted treatment, lived much longer, and had a much
better quality of life.
I can understand why some people are afraid to take medicines. When pharmacists
hand them a piece of paper along with their prescriptions that says "this medicine may
cause hepatitis, kidney failure, cancer, and even death," it has to make them think
twice before taking these medications . As a physician, I know how infrequent these
side effects occur in reality compared to how much more often the medicines help
people who have lupus. Therefore, I have put this information into easier to under­
stand terms so that patients can learn what the potential side effects of the medicines
are, how often they actually occur, how to look out for side effects, and even how to
prevent them in the first place.
Other problems from lupus are not directly due to the overactive immune system.
These additional problems include such things as heart attacks, infections, cancer,
broken bones from osteoporosis, fatigue, memory problems, and many more. Instead
of simply listing these problems in this book, I explain them in detail. More impor­
tantly, I give practical advice on what to do about them . For example, if you have lupus ,
have trouble getting out o f b e d , a n d have n o energy, what is going o n a n d what can
you do? The sections of this book on fatigue, sleep problems, fibromyalgia, and de­
pression give you very practical advice on things you can try out yourself to try to feel
better. Often, at a doctor's visit, the physician does not have the time to go through
all of these very important possibilities and solutions. In this book, they are right at
your fingertips . You can let your doctor work on looking for things such as low blood
counts, lupus activity, kidney problems, thyroid problems, and the like while you work
on looking at these other potential causes and treatments.

H ow to Use This B o o k

O n e thing that I have been very impressed about in m y patients who have lupus is
the research many of them have done to learn about lupus. While many people only
want to know the basics and straightforward instructions, others want to know all the
medical facts about their problems, including the meaning of all their abnormal lab
results. Therefore, I wrote this book with both groups in mind. Much of the informa­
tion is written in a basic, easy-to-understand format in nonmedical terms so that most
people can understand the medical aspects of the disorder and the proper things to
do well. In addition, I give complex medical terms with explanations as well. I want
people who have lupus to be able to look up the actual medical terminology that their
doctors use, so that they can understand in more depth about what is going on. There­
fore, I do not apologize for some of the highly technical aspects of the book. You can
skip over these parts of the book and stick to the more practical and easy-to-read sec­
tions if you wish.
The book is divided into five main parts. Part I explains in detail what lupus is.
It discusses what causes it, what other immune disorders are associated with it, and

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