Chapter 1 What Is Lupus?
At one time, lupus was considered a rare disease, but, thanks to better diagnostic techniques and greater awareness on the part of physicians, we now know that is it far more common than we ever thought. About five hundred thousand Americans have lupus, and more than 90 percent of these are women. About sixteen thousand new cases are diagnosed each year. (Asian, African-American, and Hispanic women appear to be at somewhat higher risk for lupus than Caucasian women.) Notably, lupus is more widespread than many better-known diseases, such as leukemia or multiple sclerosis. Lupus typically strikes women of childbearing age -- usually between fifteen and forty -- however, it can also occur in very young girls and postmenopausal women. In rare cases, infants of mothers with lupus or other connective tissue diseases may be born with neonatal lupus, a transient form of the disease that usually disappears within six months.
I woke up one morning with a strange pain shooting from my neck down to my shoulders. I felt a lot more tired than usual. I went to see my doctor who decided to test me for "the Three L's": Lyme disease, lupus, and leukemia. The tests were negative for all three, so he decided to treat me for Lyme and put me on very high doses of antibiotics. I went away on vacation and did a lot of sunbathing. I got a bright red rash all over my face, even in my scalp, and I was sore and puffy everywhere. When I came back, I went to another doctor, this time a rheumatologist, who took one look at me and said, "You don't have Lyme disease, you have lupus." Sarah, thirty-three
I was feeling ill with all kinds of funny symptoms. I'm normally very active, but I felt exhausted. It was a weird kind of exhaustion. No matter how much I slept, I still felt tired. I felt achy, I had floating arthritis. A joint would hurt one day, and then another joint would hurt the next day. Another strange thing -- I couldn't close my jaw. It felt as if my dentist had put a filling in the wrong way. I went to an orthopedist and he said, "Well, you must have strained yourself lifting something." I went to another doctor, who tested me for Lyme, and I tested positive. My brother, who is a doctor, didn't believe that I had Lyme. He sent me to a rheumatologist, who ran all kinds of tests. She finally told me that I had lupus. Claire, fifty-one
All through high school I was sick. I had seizures, and my parents were told that I had some kind of seizure disorder. In my twenties, I began developing strange neurological symptoms. I thought I was having a nervous breakdown. I had difficulty focusing for very long periods at a time, and I had strange feelings in my head. I went to a doctor who suggested that I see a psychiatrist. I went to a psychiatrist, and after talking with me, he said that I was totally sane but terribly sick. He referred me to a rheumatologist, who told me that I was having a lupus flare. Susan, thirty-seven
These three women experienced vastly different symptoms. Yet, all three have been diagnosed with the same disease: systemic lupus erythematosus (SLE), commonly called lupus, a chronic, inflammatory disease that can affect any part of the body. Like many other women with lupus, these women were initially misdiagnosed and had to go from doctor to doctor before getting the correct diagnosis, usually from a rheumatologist. Their experiences are echoed by hundreds of other patients. In fact, it is the rare lupus patient who gets diagnosed quickly.
Although there is a growing awareness about lupus within the medical community as well as among laypeople, a great deal of ignorance remains. Many cases go undetected or are misdiagnosed. Part of the problem may stem from the lack of emphasis on diseases such as lupus in general medical education. Lupus is very complicated, and because it is such a varied disease, it may be difficult to pigeonhole for medical students. In the past, rheumatology was covered only briefly in medical school, and lupus may have been discussed in only one or two lectures (if at all) and usually only in connection with kidney disease. Although many medical schools now include more on rheumatology in their curricula, the American College of Rheumatology (the professional organization of rheumatologists) has deemed it necessary to develop curricular materials to promote more in-depth coverage of rheumatology in medical school.
To add to the general lack of awareness, lupus is not the kind of disease that captures the constant attention of the media the way that acquired immunodeficiency syndrome (AIDS) and to a lesser extent breast cancer and Lyme disease have. Although it is not known exactly how lupus is contracted, it is believed that it is not contagious. Thus, public health officials do not feel that it is necessary to bombard the media with information on how to prevent lupus as they do with AIDS or Lyme, because as far as we know, lupus cannot be prevented. Second, unlike breast cancer, lupus cannot be detected through a simple test such as a mammogram. In fact, there is no single diagnostic test for lupus. And since women are not routinely screened for lupus, it is not a disease that is on the minds of most patients or even their physicians.
Lupus is also primarily a woman's disease. Historically, diseases that afflict women have been neglected by the medical establishment. In fact, to compensate for this neglect, the National Institutes of Health have recently mandated that women must be included in all research studies. Currently, in comparison to other "women's diseases," however, lupus has received more attention from researchers because of its link to the immune system, which is an area of science that has received much attention in recent years. Even so, there are few controlled clinical trials involving human subjects, and much more research is required in this and other areas pertaining to women's health.
Because most lupus patients are women, there is a risk that physicians may not take their complaints as seriously as they do those of male patients. Many women with lupus say that their complaints, at least initially, were dismissed by their physicians as unimportant. In some cases, vague symptoms such as fatigue or general achiness are quickly labeled psychosomatic, especially if the patient is female. The medical establishment has a long history of dismissing women's symptoms as "hysterical" or "neurotic," and there are many studies that document that this attitude may still prevail.
However, I believe that ignorance and sexism are not the primary reasons why women with lupus frequently have difficulty getting a correct diagnosis. In fact, the real culprit may be the quirky nature of the disease itself. There is no typical case of lupus. In some women, lupus may begin as a rash -- often triggered by sun exposure -- and quickly develop into serious kidney disease. In others, lupus may cause fatigue and arthritis. In others, lupus causes chest pain, hair loss, or cold hands and feet. In still others, seizures and Alzheimer's-type symptoms may be present. In many cases, patients may have difficulty articulating their symptoms because they cover such a broad range. When asked what's wrong, they often reply, "Everything hurts," or "I feel achy all over." They may be accurately describing how they feel, but this kind of vague response can confuse physicians or make them wonder whether the patient is overreacting. (Those of us who routinely treat lupus patients understand that our patients really do hurt all over.)
The diversity of lupus symptoms is mind-boggling, and as I mentioned earlier, to complicate matters even further, there is no one medical test that can positively confirm a diagnosis of lupus. Lupus can easily be mistaken for other diseases, such as Lyme (for which lupus patients often test false positive), chronic fatigue syndrome, and even depression. In fact, to clarify which patients should be considered to have lupus, the American College of Rheumatology developed a list of eleven criteria. A patient fulfilling four or more criteria usually has lupus. (These criteria are discussed in Chapter 2, see p. 27.)
THE COMMON LINK
Although every case of lupus is different, there is one common link: the immune system. Lupus is an auto(self)immune disease. This means that the strange and disparate symptoms characteristic of this disease are caused by a malfunction of the patient's own immune system. In a normal immune system, the body produces substances called antibodies to fight against germs or toxins (antigens) that could cause disease. These antibodies are usually careful to attack only the unwelcome invaders without harming healthy tissue. In lupus, however, the immune system becomes hyperactive: It begins producing excess quantities of antibodies that are directed against the body's own tissues -- autoantibodies. Depending on the severity of the disease, these autoantibodies and cells that are part of the immune system attack different parts of the body. For example, some autoantibodies may be directed against the bone marrow, inhibiting production of red blood cells, which can cause severe anemia. Others can form substances called immune complexes, which can cause inflammation and damage many parts of the body. Connective tissue -- materials between the bone and the muscles, including tendons, collagen, and cartilage -- appears to be especially vulnerable to inflammation. Even major organ systems, including the heart, lungs, kidneys, eyes, and brain, can be damaged by these immune or inflammatory processes.
Although a hyperactive immune system seems to be the common link among lupus patients, that is where the similarity ends. Some patients produce a large quantity of different antibodies, others produce very few antibodies. In fact, to add to the complexity, there is often no clear connection between the amount or type of antibodies that are produced and the severity of the symptoms.
THE DI...
