The Lupus Book: A Guide for Patients and Their Families, Third Edition (31 page)

trying to achieve an international normalization ratio (INR), or blood-thinning level, of 3.0 to 4.0 as opposed to the usual 2.0 to 2.5. If the clots are arterial, the addition of a platelet antagonist such as dipyridamole (Persantine) or clo-pidogrel (Plavix) may be helpful. Even though corticosteroids decrease levels

of antiphospholipid antibodies or eliminate them from the blood, they promote

clotting and do not necessarily decrease the risk of thromboemboli.

A few patients have what has been termed the
catastrophic primary anti-

phospholipid syndrome
. In other words, they experience repeated thromboembolic insults despite platelet antagonist therapy with aspirin, antimalarials or other agents, or therapeutic warfarin. This rare subset of patients can be difficult to treat, but giving chronic heparin intravenously or subcutaneously, with or

without immunosuppression, is helpful. Using low molecular weight heparin

(e.g., Lovenox) is more expensive but user friendly since it has fewer side effects and dosing is always the same.

Summing Up

Approximately one-third of lupus patients have an antibody to phospholipids

which, in the presence of certain predisposing factors unique to lupus, causes

abnormal clotting. Individuals at risk are identified by performing antibody tests of phospholipids (especially to anticardiolipin antibodies), by using studies that check for prolonged clotting times, or by testing for syphilis and obtaining a

false-positive result. Between 10 and 15 percent of those with SLE have clinical evidence of abnormal clotting, which can result in strokes, recurrent miscarriages, pulmonary emboli, and low red cell or platelet counts. There are few or no symptoms or warning signs. Few dietary or activity restrictions can aid prevention. Clotting complications can be prevented or minimized with platelet

antagonist therapy (e.g., aspirin, antimalarials). Patients with thromboembolic events on platelet antagonists should be given warfarin, and a small subset of

patients need chronic heparin.

22

Lupus Through the Ages: Lupus in

Children and the Elderly

Lupus comes in many sizes, shapes, and varieties unique to the age of onset,

the presence of a specific autoantibody, and the appearance of certain clinical features. Most SLE—some 85 percent—occurs in individuals between the ages

of 20 and 60. Does the disease manifest itself differently in youth and differently in those of more advanced age? The answer is yes. Four distinct types of SLE

have been described that represent the remaining 15 percent of patients with the disease: neonatal lupus, lupus in childhood, lupus in adolescence, and older-age-onset SLE.

NEONATAL LUPUS

At first, the concept that one can be born with lupus seems frightening. When

a report appeared in 1954 that an infant was diagnosed with discoid lupus and

its mother developed lupus shortly thereafter, the rheumatology community was

concerned about the presence of such a chronic disease in infancy. However,

between 1954 and the present, only a few hundred such cases have been de-

scribed in the world’s literature. Neonatal lupus is certainly quite rare.

If the neonatal period is defined as within 30 days of birth, how can one de-

velop lupus during this period? In the 1950s, LE cells were shown to cross the

placenta and produce positive LE cell results in healthy infants of lupus moth-

ers for several weeks. It turns out the IgG but not IgM or IgA autoantibodies

cross the placenta. Three IgG-containing autoantibodies that cross the placenta can damage fetal tissue: anti-Ro (SSA), anti-La (SSB), and anti-RNP (Chapter

11). Fortunately, all of these autoantibodies disappear by the eighth month after birth, since the baby is not able to make them. A review of the literature suggests that the prevalence of these autoantibodies in children with neonatal lupus is anti-Ro, 90 percent; anti-La, 53 percent; and anti-RNP, 2 percent.

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Where and How Can the Body Be Affected by Lupus?

How Does a Physician Detect Neonatal Lupus?

The autoantibodies responsible for neonatal lupus settle in different tissues, especially the skin and heart. A review of published cases suggests that 54 percent of infants with neonatal lupus have varying degrees of myocardial dysfunction

or congenital heart block, 37 percent have cutaneous lupus lesions, and 7 percent have both. Additionally, less than 8 percent have liver, gastrointestinal, blood, neurologic, or lung manifestations of lupus. Two-thirds of all reported patients are female.

Congenital heart block
is the most serious complication of neonatal lupus.

Anti-Ro and anti-La are attracted to fetal heart pacing tissue and can interfere with its development. Congenital heart block is defined by a slow fetal heart

rate, evidence of heart block on an electrocardiogram (ECG) at birth, or abnor-

malities on a fetal heart echocardiogram (ultrasound). The death rate is 20 percent, but many babies can be saved by implanting a pacemaker. The incidence

of congenital heart block in the general population is 1 in 20,000 births. Overall, the risk for mothers with SLE of having a child with congenital heart block is

estimated at 1 to 2 percent. If they carry anti-SSA, anti-SSB, or anti-RNP an-

tibodies, the risk is about 5 percent; if they do not, it is zero. Minor pacing abnormalities with myocardial dysfunction may respond to corticosteroids.

Cutaneous neonatal lupus
is manifested by the development of discoid or subacute cutaneous-type lesions during the neonatal period. The rashes, which

disappear spontaneously after several months, respond to sun avoidance and

steroid creams. Approximately 1 case of cutaneous neonatal lupus is found for

every 30 mothers with SLE who carry the anti-Ro or anti-La antibody.

What Kind of Disease Is Found in the Mothers?

Surprisingly, many women who have children with neonatal lupus feel perfectly

healthy and do not have lupus. In a summary of the world’s literature where

maternal health has been ascertained, 40 percent had SLE, 38 percent had no

disease, 13 percent carried a diagnosis of Sjo¨gren’s syndrome, and 9 percent

had other autoimmune diagnoses. It should be remembered that 70 percent of

all Sjo¨gren’s patients have anti-Ro. Why do healthy women have babies with

neonatal lupus? Simply because one healthy women per thousand carries the

anti-Ro or anti-La antibody. Many women are family members of patients with

autoimmune diseases. Moreover, some healthy women develop clinically evident

lupus or Sjo¨gren’s within several years of giving birth to a child with neonatal lupus, but most remain in good health.

What Is the Outcome of Children with Neonatal Lupus?

With or without treatment, cutaneous lupus disappears within a few months. As

mentioned above, the mortality rate of congenital heart block is 20 percent.

Lupus Through the Ages: Lupus in Children and the Elderly

[167]

Those who survive generally do quite well. Unfortunately, occasional reports

have shown that some of the children born with neonatal manifestations of lupus develop SLE 10 to 15 years later. It is therefore strongly advised that all children with neonatal lupus be screened for SLE during their adolescent years.

What Should Mothers with SLE Know About Neonatal Lupus?

Mothers with SLE who lack the anti-Ro, anti-La, or anti-RNP antibody are not

at risk for delivering children with neonatal lupus. The 30 to 40 percent with

lupus who carry these autoantibodies should be told that the risk of congenital heart disease or block in their child is about 5 percent and of neonatal cutaneous lupus, 2 percent. Since cutaneous lupus is benign, the only precaution I take is to perform a fetal echocardiogram sometime between the 16th and 24th week

of gestation, and making sure that the fetal heart rate is not too slow. Fetal

pacemakers can be implanted into the womb if necessary. I have never rec-

ommended that women with these potentially risky autoantibodies terminate a

pregnancy, since a neonatal lupus risk of 1 in 14 is small and these children

usually have normal lives.

LUPUS IN CHILDREN

Somewhere between 5000 and 10,000 children in the United States have lupus.

Even though it looks like adult lupus under the microscope and is treated with

the same medications, there are important conceptual and treatment considera-

tions that enter into the overall equation.

First of all, lupus proportionately occurs more often in boys than men. Adult

males make up 5 to 15 percent of the lupus population, but boys represent 20

to 40 percent of those with childhood lupus. Lupus is more severe at early ages and milder in older age groups. Whereas half of those with adult SLE develop

organ-threatening disease (e.g., heart, lung, kidney, or liver involvement), 80

percent of those with childhood-onset SLE develop organ-threatening condi-

tions. Nearly 70 percent of children with lupus have kidney disease, as opposed to 30 to 40 percent of adults. As a result, children with SLE require more

aggressive monitoring and management. This becomes especially apparent since

they are less likely to complain or to understand the seriousness of the inflammatory process. Fortunately, lupus is usually fairly easy to diagnose in children.

The average lupus patient in the third decade has symptoms for 1 to 2 years

before being diagnosed; in children, a diagnosis is usually made within 3

months.

The outcome of adult diseases is often discussed in medical journals and

textbooks in terms of 5- or 10-year survival; in children, we are trying to achieve a 50-year survival. Since many of those with childhood SLE live out a normal

life course, anything a doctor does has long-term implications. For example,

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Where and How Can the Body Be Affected by Lupus?

corticosteroids may stunt growth and influence a child’s stature for a lifetime.

Chemotherapies may render a patient sterile and rarely cause cancer many years

later, which naturally has an impact on dating, career, and life-style choices.

Certain medications used to treat lupus—such as the nonsteroidal anti-

inflammatory indomethacin—are specifically ill-advised in young children.

Other medications are not available as liquids or in strengths that can easily and safely be used by children. There are only a few hundred pediatric rheumatologists in the United States. All parents of children with SLE should have their pediatrician or healthcare-provider network seek out one of these practitioners for counsel and advice.

LUPUS IN ADOLESCENTS

Pubertal teenagers with SLE have the same clinical manifestations as young

adults with the disease. However, several psychosocial considerations are unique to adolescents.

Adolescents are notorious for not complying with prescriptions and medica-

tion. They find out very quickly what steroids do to their appearance and mood.

Many would rather not take a medication that promotes fluid retention, acne,

facial hair, easy bruising, and a puffy face. A fair percentage pretend to take their steroids but don’t. Fragile social relationships with friends can be altered by sun avoidance, a skin rash, hair loss, swollen joints, and fatigue. They often have special concerns when confronted with issues of dating, marriage, or childbearing. All too often, physicians treat adolescents as adults and fail to address these issues. This mistake can be deadly. The importance of compliance with

medication, regular blood monitoring, and being honest with the physician must

be specifically spelled out and frequently reinforced. See Chapter 25 for a more detailed discussion.

LUPUS AMONG THE ELDERLY

Two groups of individuals with SLE are found among patients over the age of

60: those who have had lupus for years and those who develop it for the first

time. For those with a history of long-term SLE, the golden years are usually

just that. Lupus tends to burn itself out after many years and rarely progresses after menopause. Patients still need to be careful in the sun and may have joint inflammation, but newly evolved organ-threatening disease is extremely rare.

The onset of lupus past the age of 60 is often very subtle. Our work has

suggested that it takes an average of 3 years of symptoms before non-drug-

induced SLE is diagnosed. Drug-induced lupus makes up most of the new lupus

cases in older age groups, so a careful drug history should be taken. This topic is discussed in detail in Chapter 9. Several other diseases mimic SLE and dif-Lupus Through the Ages: Lupus in Children and the Elderly

[169]

ferentiation can be difficult. In individuals over the age of 60, rheumatoid arthritis, polymyalgia rheumatica, and primary Sjo¨gren’s syndrome can all produce positive ANA tests, inflammatory arthritis, stiffness, aching, and elevated sedimentation rates. A rheumatology consultation is often helpful in arriving at the correct diagnosis. Late-onset SLE includes more males among its victims and

is clinically manifested by aching, stiffness, dry eyes, dry mouth, and pleuritic pain. Organ-threatening disease is quite rare and found in less than 20 percent of patients. Rashes, fevers, swollen glands, Raynaud’s phenomenon, and neu-ropsychatric lupus are also much less common than in young adults. Low-dose

steroids, methotrexate, and antimalarials along with NSAIDs are frequently em-

ployed as treatment.

Less than 2 percent of all SLE occurs after the age of 70. Lupus in the elderly has an excellent outcome and deaths from the disease are extremely unusual.