Living with Lupus

by Food and Drug Administration (FDA)

It was not the stuff wedding memories are made of. Three days before she was to be married, Angela Richardson was so sick she ended up in the intensive care unit of a Washington, D.C., hospital.

"I was just a basket case," she says. "I had hives. I had swelling. My eyes were hemorrhaging. My blood pressure went out of control. My muscles ached. My joints ached. I was so swollen and in such bad shape, my doctor didn't even recognize me. I looked like somebody had beaten me up."

It wasn't a "somebody" that had beaten her up though. It was systemic lupus erythematosus — a disorder of the body's immune system from which she had suffered for many years.

In lupus, the body produces abnormal antibodies called autoantibodies. Unlike normal antibodies, which are produced in response to and attack foreign substances such as viruses, autoantibodies attack normal body tissue.

Lupus belongs to the family of rheumatic diseases — a group of diseases of the connective tissue. Lupus can affect the body's joints, muscles and skin. It can also damage the kidneys, lungs and heart and cause neurologic symptoms.

Of the approximately 500,000 lupus patients in the United States, most are women in their childbearing years. About 1 in 700 women between the ages of 15 and 64 is afflicted. Although lupus occurs in all racial and ethnic groups, it is seen more frequently among blacks; 1 in 250 black women between the ages of 15 and 64 develops the disease.

Unromantic Honeymoons

Richardson's doctors managed to control her symptoms enough for the wedding to proceed as planned. But the honeymoon was another matter. Richardson said her doctors told her to come right back to the hospital after the wedding. "Believe it or not, by the next week all the symptoms were gone again," she says. "It just hits hard and fast and then goes away."

Roslyn Tavel of Chevy Chase, Md., made it through her wedding without problems like Richardson's. But the lupus she didn't even know she had showed up on her honeymoon. "We had to walk up steps to get to our room and I can remember my knees hurt so much," she says. "I just assumed I had arthritis." She didn't go see a doctor about the pain, she explains, because she thought there wasn't anything that could be done.

Systemic lupus erythematosus can begin with any number of symptoms. The most common include skin rashes, joint pain and inflammation, fatigue, and fever. Lupus patients also frequently suffer from anemia, hair loss, weight loss, and sun sensitivity.

Both Tavel and Richardson escaped one characteristic symptom of the disease — a red facial rash that covers the cheeks and nose, frequently described as a butterfly shape. To the 19th century French physician who named the disease, the rash resembled a wolf bite — hence the name lupus, which is Latin for wolf. However, the rash occurs in fewer than half of all lupus patients.

One of the most serious manifestations of the disease is kidney damage. Nephritis, or kidney inflammation, can result when the kidney's filtration system is clogged by overabundant antibody complexes. Because damage can occur long before symptoms appear, a lupus patient's kidney function must be monitored closely. About half of those with lupus develop nephritis and kidney failure, which is responsible for about 25 percent of the deaths in lupus patients. Other serious complications can include damage to the linings of the heart and lungs, and even the organs themselves.

What makes diagnosis difficult is that any one of these symptoms, or even several of them, can also occur with many other diseases.

"It may take months or years before enough things come together to make somebody think of lupus," says John H. Klippel, M.D., clinical director of the National Institute of Arthritis and Musculoskeletal and Skin Diseases, part of the National Institutes of Health. "A patient may come down with joint pains, and one doesn't immediately think of lupus when one gets joint pains. It's only when things like a rash, which appears 12 months later, comes into the picture — and mouth sores, which come two years later — that suddenly it seems clear that what's been going on for two or three years has been lupus. Yet, from day one, when all the patient was doing was complaining of joint pains, lupus would have been number 50 that anyone would have thought of."

Thinking back, Richardson says her first lupus symptoms — itching all over and hives — appeared in the late 1970s. Other symptoms that began to appear included joint pains and bleeding in her eye. But it wasn't until January 1980, after seeing several doctors who couldn't pinpoint the problem, that doctors at NIH diagnosed lupus, based on a blood test and her symptoms.

For Tavel, "Everything kind of happened when I had the baby." After giving birth to a healthy girl in June 1988, Tavel developed pneumonia, pleurisy (inflammation of the membranes that cover the lungs), and severe joint pain. But she had no idea what was wrong and neither did the doctors. "I guess I knew I shouldn't have felt that bad just after giving birth," she says.

Diagnosis

According to the Lupus Foundation of America there is no single test that can definitively say whether a person has lupus. But the following tests can aid in diagnosis by examining the status of the patient's immune system.

The anti-nuclear antibody test determines if the person has autoantibodies that react with components in cell nuclei. Over 90 percent of lupus patients will have a positive reaction to this test. However, positive results occur with a variety of other illnesses and in up to 10 percent of the normal population.

The anti-DNA antibody test determines if the patient has antibodies to DNA.

The anti-Sm antibody test looks for antibodies to a protein that was first discovered in the blood of a lupus patient (whose initials were S.M.). While many lupus patients do not have anti-Sm antibodies, they are rarely found in people without lupus.

Tests for the presence of immune complexes (the combination of antibodies and the substances with which they react) in the blood are valuable both for diagnosing and monitoring the disease.

An analysis of the serum complement level, which tends to fall when the disease is active, is also useful both for diagnosis and monitoring. The serum complement is a group of proteins involved in the inflammation that can occur in immune reactions.

About the Author

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