The Autoimmune Connection: Essential Information for Women on Diagnosis, Treatment, and Getting On With Your Life (14 page)

A slow fetal heart rate can be identified by routine examination using an amplified stethoscope, an obstetrical sonogram, or a special fetal echocardiogram. In almost all cases, heart block occurs as an isolated problem (there are no structural deformities of the heart itself). Curiously, the presence of these autoantibodies is not associated with heart problems in the mother, only in her offspring. The risk of having a child with heart block is 2 percent if you have antibodies to SSA/Ro and SSB/La. If you have had one baby with heart block, there is about an 18 percent risk of having a second affected child.

The skin rash of neonatal lupus is most commonly noted at about six weeks after delivery but can be detected at birth. The rash often involves the eyelids, face, and scalp. It is generally very red and can have a circular appearance. The skin rash can be triggered by sun exposure, so babies born to women with lupus should not be exposed to the sun without the use of sunscreen during the first months of life.

Liver abnormalities and low white blood cell and platelet counts are extremely rare problems but need to be checked. Most affected children have either heart block or skin rashes, but some do have both. Studies to date suggest that girls may be more prone to the rash than boys, but both sexes are equally susceptible to congenital heart block.

Actually, the term neonatal lupus is misleading. The name came about because the skin rash seen in infants resembled that seen in adults with SLE. While many mothers do have lupus, some mothers of affected children are totally asymptomatic themselves and have only the anti-Ro and/or anti-La antibodies in their blood. It’s important to note that just because you may have anti-Ro or anti-La antibodies and your baby has neonatal lupus does not mean you have lupus or Sjögren’s syndrome. Even more important to know: a child with heart block or skin rash does not have SLE. The rash, liver, and blood abnormalities are transient and usually disappear by six months of age as the maternal antibodies disappear from the child’s circulation. Unfortunately the heart block is permanent.

If you’re found to be at risk for having a child with neonatal lupus, it’s best to take precautionary steps, whether it’s a first pregnancy or you’ve previously had a baby with heart block or other signs of neonatal lupus.

It’s recommended that you have a fetal echocardiogram (a sonogram of the heart that gives a far better picture of the heart structure and function than routine obstetrical ultrasound) between the sixteenth and eighteenth weeks of pregnancy. This should be done every week, if possible, until about the twenty-sixth week. After that, it may not be needed as further research suggests that heart block first occurring after 26 weeks is extremely rare. Just listening with a stethoscope may be sufficient.

Should a problem in the fetal heart be detected, therapies may be needed to treat the inflammation in the heart. However, once established, complete heart block is not likely to be reversed (sometimes the block is not complete, which is better). Most children will eventually need a pacemaker.

In the situation of a mother with known antibodies who has already had a child with heart block, the risk of having a second affected child may be 10 times higher. In studies from the National Research Registry for Neonatal Lupus, the recurrence rate is approximately 18 percent.
²⁴
It is also possible to have one child with heart block and a second child with a rash and vice versa. At this time, no data suggest a benefit to prophylactic therapies, such as plasmapheresis (to filter antibodies from the blood) and/or steroids prior to the detection of a problem, but there are some new considerations, as follows.

If the fetus is diagnosed with heart block, and the block has been present for more than three weeks, the baby is watched and weekly echocardiograms are performed to determine if there are signs that heart function is deteriorating (for example, if fluid builds up around the heart or lungs).

If the heartbeat is second-degree block (incomplete), it is theoretically possible (but not yet proven) that treating the mother with a steroid such as dexamethasone, which can cross the placenta and be available in the fetal circulation, will reverse the block before it progresses to third-degree heart block. Unfortunately, the block is usually complete when it is first detected. If the fetus shows signs of inflammation, such as fluid around the heart or lungs or abdomen, this may be a more serious sign and dexamethasone may be initiated.

A study is underway to see if it’s possible to prevent a recurrence of fetal heart block by giving hydroxychloroquine early in pregnancy (before 10 weeks) to women with anti-Ro antibodies. Since hydroxychloroquine can cross the placenta, it’s hoped the drug may dampen some of the
inflammation triggered by maternal anti-Ro antibodies in the fetal circulation. If the study—Preventive Approach to Congenital Heart Block with Hydroxychloroquine, PATCH for short—shows good results, a nationwide screening and prevention trial may be possible.

What’s the outlook for your baby?
The prognosis for the child with heart block is generally good. However, heart block is permanent, and in 20 percent of cases the condition is fatal (most often before three months of age). Most children will require pacemakers, probably for life. Pacemakers are commonly implanted within the first three months after birth. In a child whose only manifestation of neonatal lupus is a skin rash, the situation is generally excellent since the rash usually disappears by about six months. In most cases no medications are needed and no scars or marks are left.

Although any child born to a mother with SLE does have a higher risk of developing SLE later in life (approximately one in 10 if it’s a girl), no data persuasively indicate that the risk is increased if the child also had neonatal lupus.

For both healthy women and women with SLE, there are times when estrogen may be needed. A woman may want birth control, and the best method may be oral contraceptives (OCs). After menopause, women may need the symptom relief that estrogen therapy can bring.

It’s been suggested that OCs may even be useful in controlling cyclical disease activity in some patients. The estrogen in oral contraceptives may be useful in preventing steroid-induced osteoporosis and may preserve fertility in women taking cyclophosphamide. For years, the conventional wisdom was that estrogens might provoke lupus flares. However, based on the Safety of Estrogens in Lupus Erythematosus, National Assessment (SELENA) trials, a duo of randomized double-blind placebo-controlled trials, one for hormone therapy and one for oral contraceptives, the OC trial found that birth control pills do not increase the risk of flares in women with stable SLE.
²⁵

In the HT-SELENA trial, researchers found that one year of HT (0.625 mg of conjugated estrogen daily and 5 mg medroxyprogesterone 12 days each month) increased mild and moderate, but not severe flares in women with inactive or stable-active SLE. In both trials, antiphospholipid/anticardiolipin antibodies and/or a history of blood clots in the leg (deep vein thrombosis) were exclusion criteria.
²⁶
However, even in these women, in the HT trial (351 women) there was one death, one stroke, two cases of deep vein thrombosis, and one case of thrombosis in an arteriovenous graft; in the placebo group, only one patient developed deep vein thrombosis.

The Women’s Health Initiative (WHI), a major clinical trial of one form of combined hormone therapy (
Prempro
) among healthy women, was halted in 2002 because of slight increases in heart attacks, strokes, blood clots, and invasive breast cancers among women taking the drug (although there was a reduced risk of colon cancer and benefit to the bones).

Given the consideration that HT may further increase the already elevated risk of cardiovascular disease (CVD) in women with lupus, the HT arm of the SELENA trial was also stopped early due to DVTs and cardiovascular complications.
²⁶

Women with a history of DVTs and elevated levels of antiphospholipid antibodies (aPLs),
anticardiolipin antibodies (aCLs)
, the
lupus anticoagulant (LAC)
, and
anti-beta2 glycoprotein I antibodies
should not take postmenopausal hormones. A false positive VDRL may also be a reason for seeking alternatives to estrogen therapy.
²⁷
While there are a number of nonhormonal remedies for menopausal symptoms, among them vitamins E and B, soy, and herbs like black cohosh (see
page 54
), you should avoid some menopausal remedies. Evening primrose oil promotes clotting and cannot be used by women who have antiphospholipid antibodies, and red clover can interfere with blood thinners used to treat antiphospholipid syndrome and promote bleeding.

Experts also advise women with lupus to be extremely cautious about plant estrogens like soy or black cohosh. Both act like estrogens in the body, binding to the same receptors. Herbal products are not regulated, and their
contents can vary quite widely. So don’t try herbs or soy as menopausal remedies without first talking it over with your rheumatologist.

Women with SLE have an increased risk of cardiovascular disease, and a review of almost two dozen studies found that the risk of CVD among people with lupus has doubled in recent years compared to the general population.
²⁸
The risk may be greater before age 45. One study found that women aged 35 to 44 were actually 50 times more likely to have a heart attack than women of similar age in the general population, but women with SLE aged 45 to 64 had only two to four times the risk.

The natural estrogen present in your body thought to protect women before menopause may actually promote blood clotting in younger women with SLE who have aPLs, high blood pressure, or kidney disease. Immune complexes in the blood may irritate the lining of blood vessels and cause inflammation, promoting atherosclerotic lesions. These fatty plaques narrow the carotid arteries—the major blood vessels supplying the brain—more often in premenopausal women with SLE, says Susan Manzi, MD, MPH, co-director of the Lupus Center of Excellence and chair of the Department of Medicine at West Penn Allegheny Health System in Pittsburgh.

A study by Dr. Manzi and colleagues also found that stiffness in the aorta might be an early marker of atherosclerotic disease. As women become perimenopausal and develop more heart disease risk factors (such as high cholesterol, high blood pressure, and obesity), this process may speed up, adds Dr. Manzi. Having aPLs increases the risk of stroke.

Taking corticosteroids causes abnormalities in blood fats, including cholesterol, and may promote insulin resistance. Cholesterol-lowering drugs may be one way to lower the risk of heart attacks.

Other steps you need to take to protect your heart include keeping your weight under control, getting regular exercise, and following a heart-healthy diet (see
page 56
). Blood pressure should be monitored carefully, and levels of LDL and HDL cholesterol, triglycerides, and homocysteine should be measured once a year. Your annual physical exam should include an electrocardiogram (ECG).

Lower than average bone mineral density (BMD), or
osteopenia
, may affect as many as 39 percent of women with SLE, and bone loss (osteoporosis) is seen in upwards of 5 percent of people with SLE.
²⁹

The use of corticosteroid therapy is a major risk factor for osteoporosis in SLE, but the disease itself may lead to bone loss through abnormalities in bone metabolism, pro-inflammatory cytokines that promote bone resorption, and premature menopause.

Avoidance of sun exposure and low vitamin D also play a role.
³⁰
Without exposure to ultraviolet light, the body cannot produce enough natural vitamin D through the skin.

Pain and fatigue may keep many women from exercise and other activities that help keep bones strong. Age is a major risk factor for bone loss in women, and vertebral compression fractures of the spine (resulting in loss of height and
kyphosis
or “widow’s hump”) are common in SLE. They may be painless and only show up on x-rays.

Any woman taking corticosteroid therapy for autoimmune disease is at risk for bone loss. The ACR Task Force on Osteoporosis Guidelines recommends oral contraceptives to prevent steroid-induced bone loss in premenopausal women, and antiresorptive bisphosphonate drugs like
alendronate (Fosamax)
for older women and those on long-term glucocorticoid therapy (see
pages 57
to
68
).
³¹
The monoclonal antibody drug
denosumab (Prolia, Xgeva)
interferes with a signaling protein to reduce the action of osteoclasts.
³²
It can be prescribed for people with impaired kidney function and could be an option for women with lupus nephritis.
²⁷
The antimalarial drug hydroxychloroquine may also help protect against bone loss due to corticosteroids.

Deanna’s story continues:

The one important thing is not to let it get you. Make sure you get the proper care, take care of yourself, eat right, and exercise, and you can beat it. I firmly believe that. I’ve met women in the support groups who let lupus consume them, and their disease just gets worse and worse. I’ve had kidney problems, intestinal problems; I had bone death because of the steroids I was on, and I have had both hips replaced. But I focused on my work, on living my life no matter what, and that kept me going. I got married last June,
and I look at the wedding pictures and outwardly, you’d never know anything was wrong. I look healthy. Sometimes when I don’t feel well, my husband will still say, “But you don’t look sick.” Despite all the problems I’ve had, I try not to think of myself as being sick. Maybe you can’t control the disease, but you can control your attitude. I really believe the power of the mind can be stronger than the disease.

D
EANNA