Lupus

Lupus

When the Immune System
Goes Awry

You wouldn't know that Mary Pat Whiteley has a chronic disease. In her early thirties, she works full-time photographing crime scenes and collecting evidence for the police department in Baltimore.

But Whiteley has lupus, a disease in which the immune system doesn't function the way it should. It makes her tired a lot, and it makes pregnancy a high risk. She had four miscarriages before her doctors were able to get the lupus under control. She adopted a child, then delivered a baby under the careful guidance of her doctor, and now she's expecting another. To help decrease the risk, she took early maternity leave.

She has the most common form of lupus, called systemic lupus erythematosus; 90 percent of those who have it are women of childbearing age. While the total number who have lupus is unknown, researchers estimate that the disease strikes 1 in 400 Caucasian women and 1 in 250 African American women.

An Attack from Within

With lupus, there's a glitch in the immune system. Instead of performing its normal duty of fighting off infections, the immune system demolishes normal tissue, says Michelle Petri, M.D., associate professor of medicine and co-director of the Lupus Pregnancy Center at Johns Hopkins University in Baltimore. It does this by producing more antibodies than normal and by producing antibodies that are excessively destructive. The skin, joints, kidneys, lungs and nervous system can be affected. Some patients have a limited form of lupus called discoid lupus, which involves only the skin.

A butterfly rash on the face, extending from one cheek over the bridge of the nose to the other cheek, which many consider to be a classic sign of lupus, actually appears in only about 50 percent of patients.

Common symptoms include fatigue, arthritis pain, fever, hair loss, muscle weakness and tenderness. Women with lupus are also prone to Raynaud's disease, a circulatory ailment that affects the hands and feet, and are often sensitive to light. Some can develop life-threatening complications such as kidney disease and problems with their lungs and heart.

How Do You Know It's Lupus?

Diagnosis is based on having 4 of 11 symptoms established by the American College of Rheumatology. One of these is a positive blood test for a group of antibodies called antinuclear antibodies (ANA). Other signs may include arthritis, the butterfly rash, mouth sores, seizures or psychosis, pleuritis (inflammation of the lining of the lungs), pericarditis (inflammation of the lining of the heart) or skin lesions.

Women who test negative for ANA may have a condition in which subtle signs of lupus are present. Usually this condition doesn't develop into lupus, but women should still seek medical advice.

A woman may test positive for ANA but have no symptoms, says Philip Mease, M.D., clinical associate professor of rheumatology at the University of Washington School of Medicine in Seattle. These women often ask whether they will eventually develop full-blown lupus, but that's hard for doctors to predict, says Dr. Mease.

In a few cases the disease can be fatal, says Dr. Petri, but most women with lupus can have a normal life expectancy. And the chances of living a full life are greater now than ever, she says.

Dr. Petri says good medical care and following the doctor's advice should help anyone with lupus "do quite well." Women with lupus can lead full lives, says Dr. Petri. Marriage, children and career are more than possible, she says.

A Complex Puzzle

Researchers don't know what causes lupus, but they have identified several factors that may predispose women to it or trigger its onset.

Genetics is first on the list. There appear to be at least four genes involved in lupus, says Peter H. Schur, M.D., professor of medicine at Harvard Medical School. The probability of inheriting the disease is fairly low. There's about a 5 percent chance that if a parent or sibling has lupus, you'll develop it, too, says Dr. Schur. (Some researchers believe the chances of inheriting lupus may be as low as 2 percent.) But women can get lupus even when it doesn't run in the family.

If a woman does carry lupus, a wide variety of factors may spur a flare-up of symptoms. Among the possible culprits are certain medications like sulfa drugs and penicillin, viral infections, exposure to the sun and possibly even stressful events, says Dr. Schur.

Because lupus occurs more often in women than in men during the years that women menstruate, researchers suspect that female hormones play a role--though they don't know what that role might be. Ten women for every one man get lupus during their childbearing years. Before and after that time, the rate of diagnosis is equal for both genders.

Further support for the role of hormones comes from laboratory studies. The introduction of male hormones in mice has been shown to suppress development of the disease, whereas female hormones accelerate it. Studies have also determined that lupus symptoms get worse during the hormonal fluctuations of pregnancy and the postpartum period.

And, through questionnaires answered by 104 women who had lupus, Dr. Petri found that two symptoms--joint pain and the facial rash--were worse in the two weeks prior to menstruation, when hormone levels change.

A Strategy for Pregnancy

Pregnancy can be tricky for women with lupus. Besides the possibility of a worsening of symptoms, they're at risk for complications of pregnancy. About 15 percent of these pregnancies end in miscarriage, and women with lupus are three times more likely to deliver prematurely than women who do not have it.

Pregnancy risk varies from woman to woman, however. Those who have certain types of antibodies that interfere with the circulation of blood to the placenta and fetus are said by experts to be at greater risk for miscarriage in midpregnancy. It's best to get pregnant when the disease has been inactive for at least three to six months, researchers say.

Living with Lupus

Lupus has no cure and can't be prevented. But it can be managed with medication, and there are ways to keep your symptoms under control. Here are some suggestions.

See a specialist. Lupus is a complex disease that's best managed by a specialist. A rheumatologist or internist with a specialization in lupus should coordinate your care, says Dr. Schur.

Once you're in the care of a specialist, you're likely to be put on a program that includes medication and careful observation. Different women require different medications, says Dr. Schur. Most are treated with nonsteroidal anti-inflammatory drugs, antimalarial drugs, corticosteroids or drugs that suppress the immune system. Lupus can be monitored through kidney function tests, x-rays, MRIs, ultrasound and EKGs. Specialists can also observe lupus through exercise tolerance tests.

Get a second opinion. If you have any doubts about your diagnosis or treatment, get a second opinion, says Dr. Schur. And if the two opinions conflict, get a third, he says.

Join a support group. Some women find such groups helpful, says Dr. Petri. The Lupus Foundation of America and the American Lupus Society have groups all over the country, she says. If a group is not your style, the Lupus Foundation can also put you in contact with another woman with lupus whom you can call to talk with about your disease. Sometimes doctors can put you in touch with other lupus patients, too, says Dr. Mease, so don't be afraid to ask.

Don't feel guilty. Some people feel they did something wrong that led to the disease, says Dr. Petri. "Because we don't know what causes it, you don't have to feel guilty about it," she says.

Consider a course. A series of self-help courses has been designed and tested by Carrie Jo Braden, Ph.D., professor of nursing at the University of Arizona School of Nursing in Tucson. They help women gain coping skills, improve communication, boost self-esteem and deal with depression that can accompany chronic disease. Contact your local chapter of the Arthritis Foundation for information.

Take it easier. Lupus causes fatigue, so it's important for women to get the rest they need, experts say. If you work full-time, explore options for flex-time. Ask family members or friends to run errands, help clean the house, take care of the yard and help with the shopping and other chores-- or hire outside help if you can afford it.

Go walking. "We are very big believers in exercise, even though lupus is a disease that causes fatigue," says Dr. Petri. If you can keep up with a walking program, she says, you may be less fatigued. Or pick another activity that you like that has some aerobic benefit, adds Dr. Schur. Try to do some activity every day, for as long as you can.

Protect yourself. Exposure to sunlight can trigger a lupus flare-up, so be careful not to get too much. Women don't have to avoid outdoor activity altogether, says Dr. Petri, but they do need to wear sunscreen with a sun protection factor (SPF) of 15 and a brimmed hat. Be especially careful around sand and water, because they reflect sun.

Develop an emergency plan. Having a plan for what should happen if you go into the hospital with a flare-up can help make things a little less stressful, says Dr. Braden. Write out a list of who has keys to the house, who can take care of the kids and who will feed the pets, she says. Update the plan every six months.

Think of alternatives. Unexpected lupus flare-ups can require you to change plans--from simple things like maintaining your work schedule to something more unusual like going camping--at the last minute. It helps to practice coming up with different options so when a flare-up occurs, making plans is easier, says Dr. Braden. Practice brainstorming, either alone or with others, she says.

Review your contraceptive choice. Talk to your doctor before you choose a contraceptive. Women with lupus used to be advised against using oral contraceptives for fear that the Pill's high estrogen content would stimulate the disease. Some newer products, however, contain less estrogen and may be considered if you choose not to use barrier contraception. However, women with lupus and at risk for blood clots should not take estrogen-containing oral contraceptives, says Dr. Petri. Other hormonal options for women with positive ANA tests are Norplant and Depo-Provera, two contraceptives that contain progesterone but no estrogen, she adds.

Consider HRT after menopause. Some medications for lupus, particularly prednisone, increase a woman's risk for osteoporosis and heart disease, experts say. Talk to your doctor about hormone replacement therapy (HRT), which can lower your risk for both of these diseases, says Dr. Petri.