The Lupus Book: A Guide for Patients and Their Families, Third Edition (2 page)

of this textbook here in an attempt to ‘‘translate’’ it for my patients. In doing so, I have also kept in mind the many allied health professionals (physical

therapists, nurses, occupational therapists, social workers, psychologists, and others) who may be involved in the detection and care of lupus patients.

I hope all of you find this work informative and enjoyable to read. If you are

[xii]

Preface to the First Edition

reading this book, you may have been diagnosed with lupus or suspect that you

have it. It is my hope that this book will help you work with your physician.

In some instances, to flesh out the details, I have used composite cases based

on real people I treat. Of course—as I learned early in my practice—no two

patients have exactly the same experience with this disease. But some of my

patients’ personal stories may ring true and help you cope with your own symp-

toms.

My book is not intended to be a substitute for advice given by your family

physician or the specialist you have been referred to. These doctors know your

medical history and related problems far better than I ever will and can provide you with a perspective that it is not possible for me to impart.

The Lupus Book
is not meant to be read from front to back. It is intended as a resource for patients and caregivers who are interested in how various aspects of the disease are approached. In particular, Chapters 5 to 9 may be very technical. The reader should not get discouraged; understanding immunology is a

daunting task even for physicians.

I wish to thank Lea & Febiger, my textbook publisher, for allowing me to

use materials from
Dubois’ Lupus Erythematosus
for this publication. Special thanks are owed to Ruth Wreshner, my editor Frances Brock, Allan Metzger,

M.D., Nancy Horn, my medical artist Terri Hoffman, Joan Bossert and the

people at Oxford University Press, as well as my wife (and editor) Janice, and

our three children, Naomi, Phillip, and Sarah.

Additional thanks are owed to my long suffering secretary, Amanda Trujillo,

the Lupus Foundation of America (especially John Huber, Kerryn Coffman,

and Judy Madwin), The American Lupus Society (especially Leslie Epstein),

and Drs. R. H. Phillips and Jim Maguire for their inspiring writings on

lupus and rheumatoid arthritis.

Los Angeles

D. J. W.

April 1995

Part I

INTRODUCTION AND

DEFINITIONS

Where should we start? The most logical place is with a definition of lupus. We look at how it is classified as a disease and place it in its proper historical perspective. This is followed by an overview of how lupus is distributed in the population—in other words, who gets the disease, which parts of the world have

the highest prevalence of lupus, how many people have lupus in the United

States, at what age, and which sex is most affected.

This page intentionally left blank

1

Why Write a Book on Lupus?

The first time someone hears the words ‘‘lupus erythematosus,’’ he or she usu-

ally says
‘‘What?’’
When I first started my practice, patients identified the term with Peter Lupus, one of the characters on
Mission Impossible
, a popular tele-vision series in the late 1960s. Sometimes it looks as though finding a cure for lupus is an impossible mission, but there is much we
do
know, and the aim of this book is to share that knowledge.

Lupus is the common name for the disorder known technically as lupus erythe-

matosus. This formal name includes systemic lupus erythematosus—where
sys-

temic
means affecting the entire body or internal system—or SLE for short. Although underrecognized, lupus is an extremely important disease for many reasons:

▪
In the United States, nearly one million people suffer from lupus
. It is more common than better-known disorders such as leukemia, multiple sclerosis,

cystic fibrosis, and muscular dystrophy
combined
. Those who develop SLE

do so in the prime of life. And 90 percent of these sufferers are women, 90

percent of whom are in their childbearing years. Moreover, the effects of

the disease disrupt family life and account for billions of dollars in lost

productivity.

▪
Understanding the immunology of lupus will help us better understand

AIDS, infections in general, allergies, and cancer
. Medical students are often told, ‘‘Know lupus and you know medicine’’ and lupus is the para-digm of autoimmunity. This is because SLE can affect every part of the

body. The basic pathology of lupus, or the factors that cause the disease,

get to the core of how the human immune system functions. Nearly every

major advance in understanding lupus immunology has had a spillover ef-

fect—it has helped not only SLE patients but also those with immune-

related disorders such as allergies, cancer, HIV, and other infectious pro-

cesses.

▪
Lupus can be a very difficult disease to diagnose
. Many lupus patients look perfectly healthy, but surveys have shown that newly diagnosed patients

[4]

Introduction and Definitions

have had symptoms or signs for an average of 3 years. A young woman

who complains of fatigue, achiness, stiffness, and low-grade fevers or swol-

len glands is often told she is experiencing stress, has picked up a virus that is going around, or—worse—that she is exaggerating her symptoms. By

the time she is diagnosed with SLE, permanent damage to vital organs such

as the lungs or kidneys may have occurred. (Serious lupus is usually easy

to diagnose.) This book attempts to increase public awareness of the disease,

which could lead to earlier diagnosis.

▪
The diseases of females are understudied by organized medicine
. For years, many medical protocols have tended to limit funded studies to males. A

survey done in the late 1980s showed that 70 to 80 percent of all research

participants in treatment protocols being conducted in the United States

were men. (Some of this bias can be explained by the preferential funding

given to Veterans Administration hospitals.) But diseases that primarily af-

fect females are funded to a lesser extent than other less common disorders,

such as leukemia or muscular dystrophy. If the population of patients suf-

fering from lupus were 90 percent male, I daresay that the medical com-

munity would be more responsive. Research on lupus is also relatively un-

derfunded compared to studies of other life-threatening diseases.

▪
It is my opinion that there is a shortage of doctors capable of diagnosing and
treating SLE
, a disease studied and managed by rheumatologists. Rheumatology is one of the recognized subspecialties of internal medicine, along with car-diology, gastroenterology, and pulmonary medicine, but it was certified only in 1972. It is therefore a relative newcomer—a field in which only 4000 of the

600,000 physicians in the United States are certified to practice.

▪
Many patients who are told they have SLE do not
. Some ten million Americans have a positive lupus blood screen (called antinuclear antibody, or

ANA) but only about one million of these actually have SLE. Since normal

patients and healthy relatives of those with autoimmune disease can have

positive tests for lupus, some physicians take the test results at face value

and inform their patients (especially young women) that they do indeed

have the disease or may succumb to it in future. Such patients may suffer

ill effects, especially if unnecessary treatments are prescribed. Also, many

disorders mimic SLE. A positive blood test for lupus may be found during

a viral illness, and unsuspecting physicians may draw the wrong conclu-

sions. Disorders closely related to SLE, such as scleroderma or polymyositis

(see Glossary for definitions of technical terms), may exhibit similar test

results but are treated quite differently. In approaching this difficult diag-

nosis, a complex diagnostic workup is often necessary, and few physicians

are equipped to interpret the necessary battery of tests. In these instances,

most physicians will consult a board-certified rheumatologist or recommend

that their patients visit such a specialist.

Now let’s get started—and we’ll begin by discussing what lupus really is.

2

What Is Lupus?

In simple terms, lupus erythematosus develops when the body becomes allergic

to itself. Immunologically speaking, it is the opposite of what takes place in

cancer or AIDS. In lupus, the body overreacts to an unknown stimulus and

makes too many antibodies, or proteins directed against body tissue. Thus, lupus is called an
autoimmune disease (auto
meaning
self
).

IS THERE AN ‘‘OFFICIAL’’ DEFINITION OF LUPUS?

The American College of Rheumatology (ACR), a professional association to

which nearly all rheumatologists in the United States belong, devised criteria

for defining the disease in 1971. These criteria were revised in 1982 and 1996, and are shown in Table 2.1. The presence of 4 of the 11 criteria confirms the

diagnosis. These criteria apply only to SLE and not to drug-induced or discoid

(cutaneous) lupus. (These various forms of lupus are discussed under the next

heading.)

The first four criteria concern the skin: sun sensitivity, mouth sores, butterfly rashes, and discoid (resembling a disk) lesions.

The second four criteria are associated with specific organ areas: the lining

of the heart or lung, the kidneys, the central nervous system, and the joints.

The remaining three criteria specify relevant laboratory abnormalities: altered blood counts (low red blood cells, white blood cells, or platelets), positive ANA (antinuclear antibody) testing, and other blood antibody abnormalities of the

disease. The ANA test is used as the primary diagnostic tool to determine

whether a person has lupus, but there are limits to its reliability, which we

discuss in Chapter 6. A patient can have SLE without fulfilling ACR criteria.

For example, a patient with a positive kidney biopsy for lupus may meet only

2 criteria if the ANA is also positive. Though over 90 percent sensitive and

specific for the diagnosis of SLE, the ACR criteria are primarily used for re-

search purposes as entry criteria for a study.

Many other manifestations of SLE are not included in the ACR criteria. They

[6]

Introduction and Definitions

are excluded because they are not
statistically
important in differentiating SLE

from other rheumatic diseases. For example, a condition known as Raynaud’s

phenomenon (when one’s fingers turn white and then blue in cold weather) is

present in one-third of lupus patients. But it is not included in the criteria, since 95 percent of those suffering from scleroderma also have Raynaud’s. In other

words, it is not specific to SLE and therefore does not provide enough proof to classify someone as having SLE. These particular manifestations of SLE will

be covered in detail in later chapters.

WHAT TYPES OF LUPUS ARE THERE?

Sometimes the autoimmune reaction of lupus can be limited just to the skin and

may result in a
negative
ANA blood test. This condition is called
cutaneous
or
discoid lupus erythematosus (DLE)
. Though this is not an entirely accurate term (see Chapter 12), it helps distinguish these patients from those suffering with systemic lupus. About 10 percent of lupus patients exhibit this condition. When internal features are also present and fulfill ACR criteria (Table 2.1), we describe the condition as
systemic lupus erythematosus (SLE)
.

SLE patients who have symptoms of achiness, fatigue, pain on taking a deep

breath, fevers, swollen glands, and signs of swollen joints or rashes but whose internal organs are not involved (for example, the heart, lung, kidney, or liver) are said to have
non-organ-threatening disease
. Statistics vary, but on the basis
Table 2.1.
ACR (1996) Revised Criteria for the Classification of Systemic Lupus Erythematosus
A person is said to have SLE if four of the eleven following criteria are present at any time:
Skin criteria

1. Butterfly rash (lupus rash over the cheeks and nose)

2. Discoid rash (a thick, disklike rash that scars, usually on sun-exposed areas) 3. Sun sensitivity (rash after being exposed to ultraviolet A and B light)

4. Oral ulcerations (recurrent sores in the mouth or nose)

Systemic criteria

5. Arthritis (inflammation of two peripheral joints with tenderness, swelling, or fluid) 6. Serositis (inflammation of the lining of the lung—also called the pleura—or the heart—also called the pericardium)

7. Kidney disorder (protein in urine samples or abnormal sediment in urine seen under the microscope)

8. Neurologic disorder (seizures or psychosis with no other explanation)

Laboratory criteria

9. Blood abnormalities (hemolytic anemia, low white blood cell counts, low platelet counts) 10. Immunologic disorder (blood testing indicating either antiphospholipid antibodies, lupus anticoagulant, anti-DNA, false-positive syphilis test, or a positive anti-Sm)

11. Positive ANA blood test

What Is Lupus?

[7]

of my own clinical experience, I estimate that about 35 percent of lupus patients fall into this category. Patients with non-organ-threatening disease have a normal life expectancy, and it is uncommon for them to develop disease in the major