Expert Information About Lupus Treatment A Drug That Helps Ease Lupus Symptoms in Women

Nine out of 10 people with lupus are women, and it strikes most often when they're 15 to 44 years old. To mark Lupus Awareness Month, rheumatologist Diane Kamen, M.D., describes symptoms, explains how to get an accurate diagnosis and tells us about good treatments, including a breakthrough drug now on the market… All of us produce beneficial antibodies that fight against harmful viruses and bacteria. But that's not the case when a woman has lupus. With lupus, her immune system goes haywire, producing antibodies that attack not invading germs but the body’s own healthy cells. In the process, it systematically damages many different tissues and organs, such as the skin, kidneys, lungs and heart. “It’s a lifelong disease, but most women live fairly normal lives even when they have it,” says lupus expert Diane Kamen, M.D., a rheumatologist and associate professor at Medical University of South Carolina. Although there is no cure for lupus, treatments have improved dramatically in the last decade. In this exclusive Lifescript interview, Kamen explains lupus symptoms in women, treatments and more. What causes lupus? It’s partly genetic. If a twin has lupus, her identical twin, with the same genes, has a 20% to 30% chance of developing it too — far above average. Family history increases your risk, but 80% of those who get it have no family members with lupus. So the environment matters too. [For example,] If a woman is exposed to silica dust, [working with] pottery or farming sandy soil, that seems to increase her risk for lupus. Smoking and getting either too little vitamin D or too much sun exposure also can trigger lupus. And one form of lupus is induced by taking certain medications. But we don’t really know what causes this switch in the immune system to turn on a woman’s healthy tissue. What medications can cause lupus? There’s a blood pressure drug, Hydralazine, and another for heart arrhythmia called Procainamide. They’re not used a lot by women. There’s one for rheumatoid arthritis called Infliximab, and that’s used more often by women. But drug-induced lupus is rare and reversible. Women are just switched to other medicines. Why is lupus more prevalent in women? Female hormones may help explain the sex disparity. Boys are about as likely as girls to get lupus before puberty. So the shift happens when hormones kick in. On the other hand, women who take birth control pills or hormone replacement after menopause aren’t at higher risk for lupus. But we advise women to avoid high doses of estrogen as a precaution, because animal research suggests this might contribute to developing lupus. Is there anything women can do to prevent the disease? There’s no proven way of preventing lupus. Based on what raises the risk, we advise women not to smoke, and to wear goggles or a mask if you’re exposed to silica dust, to make sure your vitamin D levels are normal and not to get too much sun. What are the most common lupus symptoms in women? Joint pain is what sends most women to the doctor. Fatigue is another huge problem — lots of women have had it for months, even years, and it feels like having the flu all the time. Loss of appetite, weight loss, low-grade fever and swelling in the legs are other typical symptoms of lupus. And there may be hair loss. I don’t mean just the thinning that can come with age but losing chunks of hair. More than half of people with lupus have rashes that worsen with sun exposure. We call it the butterfly rash because it goes across one cheek, over the bridge of the nose and then across the other cheek. Is it hard for doctors to get to a lupus diagnosis? It can be difficult for primary-care doctors to diagnose because you have to be able to see the pattern. If you’re not thinking about lupus, you could easily conclude the woman just has the flu or arthritis. Sometimes it takes women years to get an accurate lupus diagnosis. And they may be prescribed medications, like antibiotics, which make lupus worse. If women suspect they have lupus, what are tests doctors should give to diagnose it or rule it out? A good screening test is the antinuclear antibody blood test (ANA), and 99% of people with lupus will screen positive on it. Although it’s a good test for ruling out lupus, it’s not great for confirming it because the older you are, the more likely [a false positive]. In fact, 20% of women older than 60 screen positive on the ANA and don’t have lupus. We tell women, don’t freak out if you’re positive. You also should get a complete blood count test because we’re usually going to see low white blood cells with lupus. Doctors also should test to see if you have protein in the urine, since that’s an early tipoff that you might have lupus and that it’s starting to harm your kidneys. If you screen positive on the ANA, what’s the next step to an accurate diagnosis? At this point, a woman seeing a primary-care doctor would be wise to ask for a referral to a rheumatologist. You’ll need a battery of more sophisticated tests to confirm lupus, and a specialist will know how to do them. Also, there are potential insurance problems from here on. Many insurers won’t cover these tests if they’re done through a primary-care doctor — they have to be ordered by a specialist. One blood test typically after the ANA is the double-stranded DNA antibody test. It’s more specific for lupus, so if you’re positive on this one, you’re much more likely to have lupus. Another good test at this point measures antibodies to phospholipids [lipids that are a major component of all cells]. It too makes a lupus diagnosis more likely. And there are other specific antibody tests a specialist does to get to a definite lupus diagnosis. What are the treatments if you do have lupus? For many years the gold standard has been Cytoxan, which suppresses the immune system and helps keep symptoms of lupus manageable. But it can put you into early menopause and lead to dangerously low white blood counts or bleeding from the bladder. Some women take arthritis drugs, aspirin or steroids, depending on their symptoms of lupus. Finally, in the spring of 2011, the FDA approved the first drug designed specifically for lupus, and it’s a big improvement on what we’ve had so far. What is the drug, and how is it different from the others? It’s called Benlysta. One of the harmful effects of lupus is that B cells in the blood make antibodies that can damage healthy organs. Benlysta makes the B cells die off more quickly, so they can’t create these antibodies. It works well for 60% to 70% of patients, and that’s a big response rate. Lupus symptoms in women really ease off. For example, there’s less joint pain and swelling, if that’s the problem, and rashes mostly go away. It has fewer side effects than other medicine, and they’re very minor. What other treatments might women with lupus need, and what kinds of doctors do they need to see? It all depends on how the lupus has affected them. Lupus creates a higher risk for cardiovascular problems, so some are treated by cardiologists and benefit from preventive medicines or standard treatments for heart problems. Others might have kidney problems and see kidney specialists. They might need tissue biopsies or other kidney tests. If there’s lung involvement, sometimes women need specialized biopsies. And it’s not uncommon to get skin biopsies or treatment by a dermatologist for rashes. Typically, there are flare-ups of symptoms of lupus from time to time. How long do these flare-ups last, and how serious are they? That varies depending on the patient and how quickly steps are taken to get the flare under control. With treatment, minor flares can resolve quickly, within days. But major flares involving organs, like the heart, lungs or kidneys, can take weeks to months to resolve, even with treatment. Sometimes, women come to recognize what triggers the flare-ups and can avoid it. For example, sun exposure, viruses or stress are often triggers. What is the prognosis for women with lupus? Although there isn’t a cure, the chances of living a pretty normal life are good -- certainly better than ever. Less than 10% of people with lupus die from it, and almost all of them are African-American. That’s because [they're] are not just at higher risk to get the disease, they often have more severe cases. Any woman with lupus needs to take good care of her heart and kidneys, going for regular medical checkups, since lupus can have harmful effects on these organs if a woman isn’t carefully monitored. Are there lifestyle habits to help ease lupus symptoms in women? We recommend a balanced diet and avoiding highly processed foods. Women should exercise regularly to help keep their weight normal because being overweight can make joint problems worse. Anything natural that eases stress can be helpful to women with lupus, and they should try to avoid what seems to trigger the flare-ups. Are there alternative beneficial therapies? Something like meditation that relieves stress could be helpful. But be careful with herbs and supplements. Women need to check out any natural product they’re taking with their doctors ahead of time. Echinacea, for example, is popular, but can worsen lupus. It stimulates the immune system and counteracts what we’re trying to do with lupus treatments. Is it safe to get pregnant with lupus, and will you have a healthy baby? Lupus symptoms in women usually worsen during pregnancy. Also, women with lupus are more prone to miscarriages and stillbirths. But if they plan ahead, most women can have healthy pregnancies and babies. They shouldn’t try to get pregnant during a flare-up, though, and it’s important to have the kidneys working okay before pregnancy. There are medications that can help them through [a pregnancy] safely. An anti-malarial drug called hydroxychloroquine is good for pregnant women if they’re not already taking it. Some women with lupus have been taking CellCept, a drug that suppresses the immune system, and they have to switch to another drug during the pregnancy because CellCept can cause birth defects. We often have to use steroids during pregnancy to keep the lupus quiet, and generally they are safe, though women have to be monitored closely. For the best care, women should have a high-risk OB managing the pregnancy in close partnership with their doctor who treats the lupus. How Much Do You Know About Lupus? Roughly 1.5 million Americans are afflicted with lupus. A diagnosis can be difficult, because the symptoms are sometimes intermittent and changing. But recent improvements in lupus treatment have made the condition somewhat easier to control.