The Lupus Book: A Guide for Patients and Their Families, Third Edition (18 page)

gram revealed inflammation of the pericardium, and the diagnosis of per-

icarditis was made. When a 2-D echocardiogram (heart ultrasound) showed

a moderate amount of fluid in the pericardium, a diagnosis of lupus serositis

was also made (fluid in both the pleura and pericardium) and Heather was

started on 40 milligrams of prednisone daily. Shortly thereafter, 3 Aleve

twice a day was added to her treatment. Within 3 weeks, she was able to

discontinue the steroids, but she had to take Aleve for another month.

Plaquenil (an antimalarial) was also begun, and a year later she has yet to

have a recurrence.

The term
pericardial effusion
is used to describe fluid around the sac of the heart. Present in 50 percent of patients with lupus who have undergone an

ultrasound (the technical name is two-dimensional echocardiography), the con-

dition is usually without symptoms and may not require any specific measures.

During the course of their disease, 25 percent of my patients, like Heather,

complain of chest pains below the breastbone, which are frequently relieved by

bending forward. The pain correlates with abnormalities in what are called ‘‘ST

segments’’ on an electrocardiogram (ECG). These individuals have
pericarditis
, or inflammation of the pericardium, the sac surrounding the heart. Evidence of

prior pericarditis is found microscopically in 60 percent of autopsied lupus patients. In pericarditis, the pericardial fluid contains several thousand white blood cells, and areas of the sac show that lymphocytes and plasma cells are present

in pericardial tissue. By listening through a stethoscope, a doctor can hear a

harsh rubbing sound, or
pericardial rub
, in up to 20 percent of those with acute pericarditis. Pericarditis does not imply an organ-threatening disease, since the heart tissue is not involved. If the pericardial fluid does not show evidence of infection, acute pericarditis is managed with high-dose nonsteroidal anti-Pants and Pulses: The Lungs and Heart

[97]

inflammatory drugs (e.g., indomethacin) along with a short course of moderate-

dose corticosteroids.

On rare occasions, pericarditis is complicated by
pericardial tamponade
or
constrictive pericarditis
. Tamponade occurs when so much fluid is formed that the heart muscle is prevented from pumping blood adequately. This life-threatening complication necessitates immediate removal of the fluid, or ‘‘win-

dowing,’’ to allow the fluid an outlet. Chronic inflammation can promote ad-

hesions and scars in the pericardial lining, which can result in signs similar to those of tamponade. Constrictive pericarditis, as this process is called, is cured surgically by stripping or removing the affected pericardial tissue, a procedure known as a
pericardiectomy
.

What Is Myocarditis?

Shanna was 20 years old and had already had lupus for 8 years. Her course

had been very rocky and was complicated by kidney involvement and one

attack of seizures from central nervous system vasculitis. At this point,

Shanna became concerned about her appearance and was reluctant to take

the steroid dose her doctors recommended; she ‘‘forgot’’ to take it two to

three times a week. One morning she began experiencing low-grade fevers,

a rapid pulse, and a dull chest pressure. Her electrocardiogram showed

nonspecific abnormalities with an elevated blood creatine phosphokinase

(CPK—a muscle enzyme released during a heart attack or with muscle

inflammation). A myocardial infarction (heart attack) was ruled out, but

her continued symptoms warranted admission to the coronary care unit. A

cardiologist was called to see her in consultation, and since her clinical

status was unclear, he recommended a cardiac catheterization. When it was

performed the next day, a myocardial biopsy was also obtained. Shanna

was diagnosed with lupus myocarditis. High doses of intravenous steroids

were administered immediately, and a rapid improvement in Shanna’s con-

dition was noted.

Underneath the pericardium lies the
myocardium
, which is the body’s heart muscle. Each time this pump contracts, we experience a heartbeat. The blood

supply to this muscle is provided by the
coronary arteries
. Ten percent of lupus patients eventually experience myocarditis, or inflammation of the heart muscle.

In fact, at autopsy, 40 percent of patients with systemic lupus show evidence of prior myocardial involvement. The symptoms of myocarditis usually include a

rapid pulse and chest pains and frequently coexist with active, systemic lupus.

Chest x-rays frequently show an enlarged heart, and signs of congestive heart

failure may be evident. Infections, particularly viruses, can also induce myo-

carditis. It is important to remember that lupus patients are very susceptible to

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Where and How Can the Body Be Affected by Lupus?

a variety of infections. Creatine phosphokinase (also called CPK)—a muscle

enzyme that is released with trauma, inflammation, and heart attacks—is often

elevated when the blood is tested. Since it may not be possible to differentiate a heart attack from lupus myocarditis or viral myocarditis, a
heart catheterization
might be undertaken. Inflamed heart muscles can decrease blood flow through coronary arteries. Catheterization consists of injecting dye into the coronary arteries to determine how open they are and whether the heart is receiving enough oxygen. The procedure may also include an
endomyocardial biopsy
, in which a tissue sample of the myocardium is taken. Under the microscope, lupus

myocarditis reveals the presence of plasma cells and lymphocytes.

Because myocarditis is a serious complication of systemic lupus, the condition

is treated with high doses of corticosteroids for at least several weeks. Other measures include medications that alleviate heart failure and coronary artery

insufficiency.

Congestive Heart Failure and Myocardial Dysfunction

Raphael was 50 years old and his lupus had been in remission for over 10

years. Nevertheless, when he was in his twenties and thirties, his disease

had been quite active and he had taken moderate doses of steroids for quite

a while. During this time, he developed high blood pressure and elevated

cholesterol levels. His blood sugars were borderline high. As long as he

watched his diet, limited his salt intake, and walked for a half hour twice

daily, Raphael felt well. He was on a mild blood pressure medication. When

his daughter graduated from college in Boston, he flew from the West Coast

with his wife to attend the ceremony. Unfortunately, he forgot to take his

medicine with him, and when he called his clinic on a weekend to have

them call it in to a Boston pharmacy, he was unable to get an immediate

response. That evening, he went to a seaside restaurant and splurged. After

consuming a 3-pound lobster in drawn butter with french fries, Raphael

began wheezing and complaining of shortness of breath. Within 3 hours,

he couldn’t breathe and had to be taken to an emergency room. His blood

pressure was 200/130 and his chest x-ray showed pulmonary edema (water

in the air sacs). Diuretics were administered intravenously. Within an hour,

he had urinated over 800 milliliters and felt much better.

A failing heart muscle cannot pump enough blood into the arteries and tissues

to maintain normal body functioning.
Congestive heart failure
results when either the left or right side of the heart fails to pump enough blood. In right-sided heart failure, fluid accumulates in the ankles, the liver enlarges, and the neck veins become fuller. A failing left heart, as in Raphael’s case, pushes fluid back into the lungs, which may lead to
pulmonary edema
. In the past, 5 to 10 percent of lupus patients developed congestive heart failure, although recent surveys

Pants and Pulses: The Lungs and Heart

[99]

suggest that—as a result of general medical advances and improvements in life-

style and diet—its incidence is decreasing.

Failure can be brought on or aggravated by the long-term administration of

corticosteroids, anemia, hypertension, serositis, fevers, or disorders of the heart valves (see below). To minimize heart failure, patients are advised to restrict salt in their diets; or they might be prescribed diuretics, drugs that reduce pressure on the heart muscle; digitalis, a drug that makes the heart pump more

efficiently; beta blockers, or other agents that dilate blood vessels.

Two-dimensional Doppler echocardiograms (heart ultrasounds) and studies of

wall motion have shown that many patients with active lupus have subtle ab-

normalities in the left side of the heart that affect filling and pumping, but they show no evidence of congestive heart failure. Termed
myocardial dysfunction
, these abnormalities reflect a low-grade myocarditis or inflammation-induced

stress on the heart muscle. These abnormalities usually disappear when lupus is in remission.

Libman-Sacks Endocarditis and Other Valve Disorders

Eliza had three miscarriages before her physician found the presence of

antiphospholipid antibodies upon blood testing. Her doctor diagnosed her

as having a very mild case of lupus that did not require treatment. Several

weeks later, after she had had several teeth repaired and root canal work

done, Eliza complained of feeling lethargic. Her doctor detected a low-grade

fever of which Eliza was not aware, but he also obtained a blood count that

revealed anemia, and this needed attention. When the fevers persisted and be-

came more pronounced, blood cultures were obtained that were positive for

a bacterium called
streptococcus
. A diagnosis of subacute bacterial endocarditis was made when her 2-D echocardiogram showed a bacterial growth,

known as a vegetation, on her aortic valve. Eliza was hospitalized and treated

with intravenous antibiotics for 3 weeks, but she became increasingly short

of breath. A cardiac catheterization was performed which suggested severe

impairment of an aortic valve. She was taken to surgery, where her aortic

valve was replaced. At surgery, evidence for Libman-Sacks endocarditis was

found, with the complication of an infected vegetation.

The inner surfaces of the heart, particularly those that line the four heart

valves, are known as the
endocardium
. Materials such as cellular debris, proliferating cells, and immune complexes that may average 1 to 4 millimeters in

diameter, which may be found on the endocardium in lupus patients, are called

vegetations
. Originally described by doctors Emanuel Libman and Benjamin Sacks in 1923,
Libman-Sacks endocarditis
is almost exclusively found in patients with antiphospholipid antibodies (see Chapter 21). Clinically manifested as a heart murmur, the vegetations are usually so small that they are detectable

[100]

Where and How Can the Body Be Affected by Lupus?

by a 2-D echocardiogram only 30 percent of the time. Transesophageal echo-

cardiograms (an ultrasound performed after a tiny tubelike camera is swallowed

to rest in the esophagus) increase detectability to about 60 percent. Although

they alter the dynamics of the heart only 1 to 2 percent of the time, Libman-

Sacks vegetations have two potentially serious complications. First, they are

prone to become infected, which leads to what is called
subacute bacterial

endocarditis
, where the vegetation is a growth site for bacteria. This condition has a high mortality rate and may necessitate a cardiac valve replacement. As

in Eliza’s case, a vegetation can become infected after a visit to the dentist.

Hence, I advise all patients with antiphospholipid antibodies to take prophylactic antibiotics before and after any dental procedure to prevent the valve from
receiving infected materials swallowed during surgery to the mouth
. Second, portions of the vegetations that flake off and travel into the brain through the carotid artery may cause a cerebral clot or stroke.
Therefore, I advise all patients
with established vegetations that they should be treated prophylactically with
low-dose aspirin or other drugs that reduce the ability of platelets to promote
clotting or initiate anticoagulation therapy
.

Damage to the mitral, aortic, pulmonic, or tricuspid heart valves is found only slightly more often in patients with lupus than in the general population. Pulmonary hypertension in these patients is associated with damage to the tricuspid

valve, which results in a condition know as tricuspid regurgitation (see Figure 14.2).

For unclear reasons, a floppy mitral valve, or
mitral valve prolapse
, is probably more prevalent among lupus patients. Palpitations, chest pains, and fibro-

myalgia are also associated with this syndrome, which is managed with beta

blockers (e.g., Tenormin, Inderal), antibiotics for dental or surgical procedures, and a decrease in caffeine intake.

Coronary Artery Disease and Myocardial Infarction (Heart Attack)

Bonnie was 38 years old and had been treated for lupus since she was 20.

For the last 3 years, she had been maintained on dialysis. Prior to that, she

had taken high doses of steroids along with chemotherapy for active sys-

temic lupus. While on vacation, she developed sudden pressure in her chest

and visited the local emergency room. The emergency room doctor told