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by Nancy C. Hanger

Marlowe & Co., September 2003, ISBN 1-56924-509-6
$15.95 (U.S.)
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† † † † ~ CHAPTER ONE ~

Day One


DiagnosisóAt Last

Youíve just gotten a diagnosis from the doctor that you have Systemic Lupus Erythematosus (SLE). Most likely, you have been seeking a diagnosis of some sort for quite a while. Itís not unusual for lupus patients to go undiagnosed for up to ten or fifteen years, while others are diagnosed right away. If youíre in the group of those who have had to wait for a diagnosis, rest assured that youíre not alone. Neither are you alone if you feel you have been undergoing a frustrating and overly long journey towards this diagnosis: lupus, as with many of the autoimmune diseases, is one of the "great imitators." Many other diseases look and act like lupusís symptoms; donít be angry if your doctor has misdiagnosed many other diseases on his way to diagnosing you with lupusóthis is normal.

Itís also normal to be frightened when hearing the diagnosis: after all, lupus has had a reputation for being a scary disease. Hang in there and read further. While lupus can be a serious disease, itís not necessarily fatal, nor is there anything to be scared of once you have educated yourself about this illness.

You Feel Relieved

Itís been a long road getting a diagnosis of lupus. Youíve now made it through what may be the most difficult part of the process: gettting a definitive diagnosis. Feeling relief isnít badóitís normal. We are all conditioned that getting a diagnosis of a serious disease isnít something one should feel relief about; youíre probably having conflicting thoughts on why you feel relief, and you possibly have some guilt associated with these feelings. This is also normalójust about all lupus patients go through this. If you are a woman, these feelings may be compounded. This is primarily a "womanís disease," and many conflicting emotions about why you have lupus, how you got it, and why it was so difficult to diagnose are going to surface from all concerned: from you, your doctor(s), your friends and family.

You may want to talk to your doctor about these feelings, as well as your family and friends. You may want to explain to them that the long process of getting a diagnosis has been as difficult for you as it has for them, and that you feel relieved that a diagnosis has been madeónot that you necessarily feel relief that you have the lupus itself. Open communication is very important with lupus; it will help you in the future as the disease develops and you go through various cycles of flaring and remission, as you seek support from your doctors and family/friends.

Itís Not All In Your Head

Your diagnosis assures you that what youíve been experiencing as symptoms and illnesses is not "all in your head." Itís likely that at one point someone told you that you have exaggerated or even made up your symptoms: this is very common with lupus. Everyone in my lupus support group, Wolfbytes, has had this said to them, often more than once. We compare stories each time one of us runs into yet another physician who utters the words, "Itís all in your head" or "I know you think youíre sick, but ..." or (our personal favorite) "Maybe youíre just a little depressed." If youíve heard these words, we sympathize with you: youíre not alone.

Itís a very difficult disease to diagnose at first, and because it affects primarily women, the symptoms are often dismissed as secondary to depression or even hypocondria. You now know that itís not your fault and itís not something you made up: you have a real disease that has a name, classified symptoms, bonefide support groups and national organizations.

Lupus is a more widespread disease than most people think. The National Lupus Foundation of America estimates that 16,000 Americans develop lupus every year and 500,000 to 1.5 million Americans have been diagnosed with the disease. Eight to ten times more women than men develop lupus, and women of non-Caucasian ethnicity are affected more highly (and develop it later in life and go undiagnosed longer). As yet, no one knows why this is so, but the National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS), as part of its strategic plan for reducing health disparities, is researching this area.

Lupus Isnít Always Fatal

Itís scary to receive a diagnosis of lupus: soap operas and other popular entertainments often portray people with lupus as having a "fatal disease," replete with dramatic deathbed scenes. While that may have had a grain of truth as little as twenty years ago, it simply isnít true anymore.

When it was first suggested to me by my then primary care physician that I had lupus, I told one of my good friends, Bruce, about the news. The first words out of his mouth, after he paused to think, was "So youíre going to die." Another friend, Mark, said, "I donít know if I can handle that." To both of them, I had to explain that lupus wasnít necessarily fatal: that I had a greater chance of being hit by a truck (and I live on a rural, residential street). Both of their reactions is quite typicalóthe general public still views lupus as something (1) people get if they live in a soap opera on TV, and (2) something that kills you fast and nastily. Luckily, neither of those views are correct,and I was able to assure my good friends that I was going probably going to get sick more often than normal, but I wasnít going to die.

While lupus is a serious disease, it is not necessarily fatal. Eighty to ninety percent of lupus patients survive their first ten years after diagnosis and go on to live full lives. Diagnosis of lupus has improved a hundredfold in the last twenty years, particularly with the advent of blood test diagnoses such as the rheumatoid factor (ANA) and others, leading to earlier diagnosis and therefore earlier treatment. Treatment has also improved, adding nonsteroidal antiinflammatories (NSAIDs) and various sophisticated yet gentle chemotherapies to the arsenal in the last twenty years.

Having lupus means living with lupus. This is not the time to give up and decide that the diagnosis means you have something you canít live with and canít fight. Thatís nonsense: lupus is eminently treatable and thousands live with the disease until old age. This is the time for you to educate yourself as much as you can about your disease and decide that living with lupus is your positive answer to your diagnosis.

In a sentence:

Diagnosis of systemic lupus is scary, but living with it is possible and can be a positive experience.


What is Lupus?

Learning what lupus is, and isnít, is key to learning to live with it. In this section we will learn what systemic lupus erythematosus is, what its causes may be, its symptoms, a checklist to diagnose lupus, what other forms of lupus there are, and why lupus is often called a "mimicking" disease.

What is SLE?

Systemic Lupus Erythematosus (SLE), which we will refer to simply as lupus in this book, is a rheumatological and autoimmune disease affecting the entire bodily systems. It primarily affects the pulmonary system (lungs and blood circulation), heart, joints, kidneys, and skin. The immune system is what keeps us healthyóimmune antibodies attack disease. In the case of lupus, the immune system becomes confused and the antibodies attack healthy tissue and organs, creating symtoms that range from skin rashes to inflammation in the heart or kidneys. Most often, lupus patients have joint problems, as lupus is a rheumatological disease affecting the connective tissues (joints, muscles, and organs), and can be treated along with rheumatoid arthritis.

Lupus is also often categorized as a diffuse connective tissue disease. A connective tissue disease is anything that affects the connective tissue in the body: muscles, tendons, organs, and other connecting tissues.

What Causes Lupus?

No one knows definitively what causes lupus. Many researchers today are working on finding a genetic cause of lupus, and in fact recently (1997, National Institute of Health News Release) researchers reported in the Journal of Clinical Investigation that they found a "lupus gene" on chromosome 1. What this means to lupus patients is unclear, however; it could be that lupus can be passed along genetically in family lines, but it also could mean that one of these genes on chromosome 1 can get damaged, causing lupus to develop in the patient. It is also unclear because lupus, like rheumatoid arthritis, is a complex genetic disease, meaning one gene may link to a predisposition to develop lupus, but other genes must interact with that gene plus an external factor (such as environmental exposure, like chemicals or sunlight) to trigger the lupus itself. Research on chromosome 6 was done in the past, also linking lupus to a genetic predisposition.

All this said, according to the Lupus Foundation of America, only ten percent of lupus patients will have a direct family member who also has lupus. This means much more research into the causes of lupus, and all rheumatological diseases, must continue.

As stated before, about ten times more women than men develop lupus, causing researchers to wonder if there is an estrogen (female hormone) component involved in lupus; many women develop lupus in their early twenties, after completing their full growth. Reseach into this area has much more work to go before anything definitive can be stated.

The same goes for ethnic selection: for reasons unknown, it appears from statitics that more African-American, American-Indian, Latino, and Asian women suffer from lupus than Caucasian. Research in this area continues, particularly along the lines of inequalities in health care, living conditions (with the possibilities of environmental contaminants as a factor in damaging these womenís genetic structures), and overall availability of early diagnosis for these ethnic groups.

Lupus can also be caused by exposure to certain drugs (especially the sulfa and penicillin antibiotics) and hormones (in which case it is referred to as drug-induced lupus), and by stress and ultraviolet light. Some cases of lupus appear to be trigered by exposure to certain other diseases and infections.

What does this all mean? No one knows for sure exactly how any one case of lupus has been caused.

What are the Symptoms of Lupus?

Lupus has a large number of symptoms, but the most common, according to the Lupus Foundation of America and other lupus foundation sources are:

Some or all of these symptoms may occur while you have lupus. Not all people develop all symptoms, and some people develop symptoms and complementary diseases and syndromes on their own. When you have other syndromes and diseases along with lupus, it is referred to as overlapping lupus.

There is a standard checklist from the American College of Rheumatology (ACR), issued in 1982, of symptoms and tests to diagnose lupus: the sidebar on this page lists these for you. Note that this checklist is due for updating, and many other physicians, particularly in England, have been coming up with their own alternative checklists (see Suggested Reading in the appendices). From this ACR checklist, you must have four or more of these symptoms in order for a diagnosis of lupus to be positive. Note that the ACR checklist is used primarily for research purposes as versus diagnostic purposes.


Malar Rash Rash over cheeks and nose, in a "butterfly" pattern

Discoid Rash Raised red patches, often on the arms and upper arms

Photosenstivity Reaction to sunlight or ultraviolet light, usually as a skin rash

Oral ulcers Painless (usually) ulcers in mouth and/or nose

Arthritis Nonerosive (joints do not deteriorate) arthritis in two or more peripheral joints

Serositis Pleuritis (inflammation of the pleural lining around the lungs) or pericarditis (inflammation of the lining around the heart)

Renal disorder Excessive protein in the urine and/or abnormal elements derived from red and/or white cells and/or kidney tubule cells

Neurologic disorder Seizures and/or psychosis in the absence of drugs or metabolic disturbances

Hematologic disorder Hemolytic anemia or leukopenia (low white blood count) or lymphopenia or thrombocytopenia (in the absence of drugs or other disturbances). The leukopenia and lymphopenia must be detected twice or more.

Antinuclear antibody Positive test for antinuclear antibody (ANA) in the absence of drugs that can cause it

Immunologic disorder Positive, anti-double stranded anti-DNA test, positive anti-Sm test, positive antiphospholipid antibody such as anticardiolipin, or false positive syphilis test (VDRL)

Note that the false positive syphilis test used to be one of the primary ways of testing for lupus twenty years ago.

(Adapted from Tan, E.M., et al., The 1982 Revised Criteria for the Classification of SLE. Arth Rheum 25:1`271-1277, revised 1992.)

Other Kinds of Lupus

SLE is only one type of lupus, though it covers many of the other types within itself.

There are four basic other types of lupus erythematosus: discoid, drug-induced, neonatal, and overlapping.

Discoid Lupus

Discoid or cutaneous lupus primarily affects the epidermis or skin. This is the most common type, second only to joint pain as a symptom in common LE. Discoid or chronic cutaneous LE, subcutaneous LE, and acute cutaneous LE are the three primary types of skin lupus. There are other types of skin lesions that affect people with lupus, but which also occur in other diseases, such as vasculitis (problems with blood flow), calcinosis (calcium deposits in the skin), hair loss, and Raynaudís syndrome (fingers and toes turn blue or white in the cold). People who have discoid lupus often never develop systemic lupus, while others will go on to eventually develop it. While having discoid LE, its common for patients to not test positive for ANA factors in the blood and other common SLE blood tests.

Drug-induced Lupus

Drug-induced lupus is literally just that: a drug taken for another disease or illness causes lupus to develop. According to Robert L. Rubin, Ph.D., of the Scripps Research Institute, at least 38 different medicines can cause lupus after being taken for prolonged periods, including medicines for heart disease, thyroid disease, hypertension, neuropsychiatric disorders and some anti-inflammatory agents and antibiotics (particularly the sulfa derivatives and penicillin). The three most prevelent drugs related to drug-induced lupus are:†

procainamide (Pronestyl)
hydralazine (Apresoline)
quinidine (Quinaglute)

These are all medicines to treat heart and cardiovascular illnesses. You can see a positive ANA in as early as a few weeks. List also agents for RA: Remicaid and Enbrel. Patients who have lupus do NOT have a proclivity to develop MORE lupus if theyíre on those drugs. No data to suggest that it causes lupus flareups. Often shows the SSA antigen on cell surface, especially if theyíre exposed to sunlight -- the drug exposes cell surface and immune system reacts, causing spontaneous lupus. Again, tie this into SSA antibody and neonatal lupus -- light exposure brings it out in babies with it.

It takes at least several months of continual use for any drug-induced reaction to occur, and those who already have SLE do not show any proclivity to develop more symptoms of lupus from taking those drugs.

No one knows what causes drug-induced lupus or why some people get it and others donít while taking the same drugs. Some research focuses on how drugs metabolize in the body, others focus on multiple processes and how they interact with not only the drug itself but how the immune system interacts with it. Links between how "normal" SLE develops and how drug-induced lupus develops are being researched now.

Neonatal Lupus

Neonatal lupus is the third type of lupus, and is a bit of a misnomer. Neonatal is a very rare condition and is not at all the same thing as SLE. It affects newborns who acquire maternal (from the mother) autoantibodies that affect the heart, skin, and blood. There is also a rash that appears during the first few weeks of the newbornís life, which may persist for several months.

Overlapping Lupus

Lupus does not usually occur in a vaccum. Most people with SLE have overlapping diseases and syndromes, many of which we will discuss later in this book during Day Six and Months Three, Four, and Five. Because of all these overlapping syndromes, lupus is a complicated diseaseóanother reason why it is so difficult to diagnose.

Why is Lupus So Hard to Diagnose?

Lupus is one of the great mimicking diseases, akin to Multiple Schlerosis (MS) in that way (though they are very different diseases!). Lupus can, at times, appear to be anything from chronic migraines to simple gastrointestinal disorders (such as irritible bowel syndrome) to chronic fatigue syndrome to fibromyalgia to dermatitisóanything that can be a symptom or a distinct syndrome can be interpreted as its own disease, and often is. With lupus acting as an overlapping disease in anywhere from five to thirty percent of diagnosed patients, you can only imagine what a nightmare diagnosis can be for some doctors. Far more people have these diseases than have lupus -- theyíre their own distinct disease. Important to tell the difference.

While I was waiting for my own diagnosis, I often got frustrated with all the difficulties inherent in the overlapping syndromes and symptoms. I know from correspondence with many other people with SLE that they have experience just as much or even more frustration than I. I got to the point where I gave up wanting to know what the disease I had was called: I called it "Fred." What the heck, Fred was Fred and at some point he would have a formal nameófor the time being, I was caught up in simply trying to live with all the symptoms and syndromes. And live I didódiagnosing is a long process: it took more than half my lifetime for doctors to come up with a diagnosis for me. And that diagnosis is still in flux: overlapping syndromes pop up all the time in lupus patients.

Be a patient patient. The best way to deal with this great mimic is to relax and not worry about what the doctors want to call it today: they can call if Fred, for all that it matters. Your lupus will shift and change and develop as you live with it. Living with it is what matters.

In a sentence

Diagnosis is difficult, but living with lupus is your goal.



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NANCY C. HANGER is a freelance writer and editor who was diagnosed with lupus-related syndromes in the early 1990s after more than two decades of seeking a diagnosis. She is the coauthor of Essential Business Tactics for the Net and a columnist for BYTE.com and WIRED News. Her freelance business, Windhaven Press, handles book editing and production for most of the major New York publishing houses. She lives in New Hampshire with three cats, one husband, and over ten thousand books in a 150-year-old farmhouse--give or take a few decades. She hopes to have her house/barn rennovations completed before the next century.

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