The Lupus Book: A Guide for Patients and Their Families, Third Edition (17 page)

10 percent of patients with systemic lupus will sustain a pulmonary embolus at

some time in their lives.

A patient with a pulmonary embolus will complain of acute shortness of

breath and chest pains. The diagnosis is confirmed if the patient exhibits a

combination of low levels of oxygen in the blood and a lung scan that shows

a mismatched defect (oxygen not being exchanged for carbon dioxide in the

blood vessels). Characteristic changes can be found on an electrocardiogram, or EKG (which will show right heart strain), and through blood tests (which will

reveal elevation of D-Dimers and LDH). In some cases, a pulmonary angiogram

(dye injected into the pulmonary vessels) may be needed to verify the diagnosis.

The chest x-rays may look normal at first, but after several days most show a

pattern of infarction (dead tissue) with a wedge-shaped defect representing col-lapsed, unoxygenated lung tissue.

For lupus-mediated pulmonary emboli, treatment is threefold: A patient ini-

tially gets an intravenous anticoagulant (heparin) followed by oral medication; warfarin (Coumadin) for at least several months; and then often a daily low

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dose of aspirin. Some patients with very certain types and levels of antiphos-

pholipid antibodies or those who have recurrent emboli on low-dose aspirin are

treated with warfarin indefinitely.

Pulmonary Hemorrhage

Less than 1 percent of patients with systemic lupus sustain bleeding or hemor-

rhage into the air sacs of their lungs
(pulmonary hemorrhage)
, but up to 10

percent of the deaths from all forms of active lupus are associated with such an event. Manifested by coughing up blood, pulmonary hemorrhage usually occurs

early in the course of the disease, and children are unusually susceptible. Pulmonary hemorrhage may look like ALP, but it progresses more rapidly and

targets the air sacs (alveoli) of the lungs. The chest x-ray in a patient with

pulmonary hemorrhage will reveal fluffy alveolar infiltrates; in addition, active, multisystem organ disease is often present. Bronchoscopy (whereby a narrow

tube with a telescope is placed in the airways through the mouth) will reveal

hemosiderin (blood pigment) and stained macrophages, but vasculitis is rare.

Immune complexes, blood complement, and immunoglobulin can be detected

through characteristic stains. How blood gets into the air sacs is unknown, but the process can be triggered by active lupus or a concurrent infection. Pulmonary hemorrhage is usually fatal, but a patient may be saved by aggressive, quick

management that combines high-dose steroids and a chemotherapy known as

cyclophosphamide with or without a blood-filtering treatment know as apheresis, in which antibodies and other chemicals that promote inflammation are removed.

Pulmonary Hypertension

George was mildly short of breath and, after waiting about a year, finally

consulted his family doctor. He had been diagnosed with discoid lupus 20

years earlier and had occasional aches, Raynaud’s and puffy hands but

otherwise he felt well and worked full time. His doctor obtained a normal

chest x-ray but was concerned enough to order pulmonary function tests.

These were only mildly abnormal. A pulmonary consultation was re-

quested, which resulted in ordering a two-dimensional Doppler echocar-

diogram. It showed elevated, twice normal pulmonary pressures. Further

blood testing for antiphospholipid antibodies was negative. By now, George

was winded on minimal exertion and had to stop working. He was brought

to the hospital for a trial of intravenous blood vessel dilators, which failed

to lower his pulmonary pressures. He was started on bosentan (Tracleer).

George is currently on the waiting list for a lung transplant, since he cannot

hope that his own lungs will recover adequate function.

Along with pulmonary hemorrhage, pulmonary hypertension is one of the

most feared complications of systemic lupus. Mildly increased pressure in the

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pulmonary arteries is observed in 10 to 15 percent of patients with lupus and is generally without symptoms, but significant rises (above 50 millimeters of mercury) can be life-threatening. Many lupus patients with pulmonary hypertension

have a positive anti-RNP as well as overlapping features with scleroderma and

polymyositis. Active inflammation can infrequently raise pulmonary pressures.

Like George, most patients feel well at first; later on, they start experiencing mild shortness of breath or a sense of breathlessness. Higher pressures are often associated with a sense of breathlessness, but chest x-rays are surprisingly normal and reveal pulmonary function tests with only mildly restrictive changes.

Under certain circumstances, pressures are measured by a 2-D Doppler echo-

cardiogram (a special heart ultrasound) and more accurately but invasively by

a Swan-Ganz catheter, where a pressure gauge is wedged into the pulmonary

artery. If a patient experiences a rise in pressure because of pulmonary emboli, the condition may reverse itself with the administration of anticoagulant drugs.

However, pulmonary hypertension of greater than 50 millimeters of mercury in

patients without antiphospholipid antibodies (see Chapter 21) is frequently fatal within 5 years unless a lung transplant is performed. Drugs called vasodilators (e.g., hydralazine, prostaglandins, or nifedipine) open up blood vessels and may help temporarily; they are often given in a hospital with the aid of a Swan-Ganz or similar pressure monitor. A drug known as bosentan (Tracleer) given orally

can improve, but not cure pulmonary hypertension. The cause of antiphospho-

lipid antibody-negative pulmonary hypertension in patients with systemic lupus

is not known, but it probably has something to do with the lining of pulmonary

arteries of the lung being sensitive to a variety of chemicals.

The ‘‘Shrinking Lung’’ Syndrome

This uncommon complication of systemic lupus is manifested by a sense of

breathlessness and decreased chest expansion in patients who have elevated di-

aphragms on chest x-rays. The diaphragm is a muscle that weakens as a result

of pleural (lining of the lung) scarring or other proposed mechanisms. The

shrinking lung syndrome may respond to steroid therapy, but is is usually a

benign, non-progressive process.

Adverse Reactions to Medications Used in Lupus

A small but significant group of individuals are sensitive to aspirin or other

salicylates (aspirin products) and develop wheezing (bronchospasm) when they

take salicylates. Similar reactions can also occur, to a lesser extent, with other nonsteroidal anti-inflammatory drugs (e.g., ibuprofen, naproxen). Drugs used to treat autoimmune disease—such as methotrexate and gold—can induce interstitial lung disease and occasionally provoke acute asthma. Steroids and che-

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motherapy drugs can also make a lupus patient more susceptible to pulmonary

infections. These possibilities must be ruled out before any of the lung condi-

tions discussed above are considered.

Summing Up

If you suffer from lupus, are short of breath or it hurts to take a deep breath, have a dry cough, cough up blood, wheeze or have chest pain, your lungs are

probably involved. Inflammation of the pleura (the lining of the lungs) is com-

mon and usually not serious. Interstitial lung disease (which involves the sup-

porting structures of the lungs) and pulmonary emboli are the second and third

most frequently observed lung complications in systemic lupus. They can be

managed with steroids and drugs that thin the blood and prevent clots. Acute

lupus pneumonitis, pulmonary hemorrhage, and pulmonary hypertension are

very serious conditions and are difficult to treat, even in the most experienced hands. Infections, malignancies, allergic reactions, and other processes must be ruled out before a diagnosis of lupus-related lung disease can be arrived at

successfully; a variety of well-understood diagnostic tests are available to do this.

THE HEART

Abnormalities of the heart can also significantly undermine the lupus patient’s quality of life and, like lung diseases associated with lupus, heart disease can be serious. The major cardiac symptom, chest pain, is reviewed in detail here,

along with heart diseases in lupus patients. Unfortunately, blood testing is not particularly useful in looking for active heart disease. Instead, there are many sophisticated tools that allow physicians to assess and manage the cardiac manifestations of lupus.

Why Do Lupus Patients Get Chest Pains?

Esther had systemic lupus with symptoms of occasional pleurisy, a mild

anemia, and swollen wrists. Her disease was adequately controlled with

ibuprofen and Plaquenil. One evening, while reading in bed, she experi-

enced the sudden onset of severe chest pressure. She told her husband, who

thought it might be a heart attack, and he took her to the emergency room.

Esther’s chest x-ray and electrocardiogram were normal. She saw her fam-

ily doctor the next day, who ordered a two-dimensional echocardiogram (a

heart ultrasound) to look for evidence of a pericardial effusion (fluid around

the heart sac). This turned out to be negative. Eventually, an upper gastro-

intestinal endoscopy examination was performed, which showed an erosive

gastritis from ibuprofen with evidence of esophageal reflux (where food is

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pushed back up into the esophagus from the stomach). She was started on

an antireflux and antiulcer regimen consisting of omeprazole (Prilosec) and

told not to lie down right after eating a big meal. Ibuprofen was discontin-

ued, and Esther was much better within a week.

Chest pain is a common but potentially misunderstood feature of systemic

lupus. Fortunately, it only rarely indicates heart disease, but it can be traced to many causes, each requiring its own method of management. I find it important

to exercise patience and have an open mind when I am interviewing patients

with chest pain because of the complexity of its diagnosis and treatment.

The most frequent cause of non-cardiac chest pain in lupus is
esophageal
.

The movement of acid made by the stomach backwards into the esophagus,

termed reflux esophagitis, and a digestive disorder involving the movement of

food have been reported in half of those with the disease (GERD, or gastro-

esophageal reflux disease). Autoimmune diseases and anti-inflammatory medi-

cations used to treat them may also induce esophageal pain. (The diseases, be-

cause of altered motility and the medications, can erode or ulcerate tissue lining.) This type of pain is managed with antacids and medications that improve the

movement of food and block acid release, both of which are reviewed in detail

in Chapter 18.

The second most common type of chest pain is
chest wall pain
. This is easily diagnosed; the patient feels extreme discomfort when pressure is applied to the breastbone or, more technically, the costochondral margins of the sternum. It

can be a symptom of fibromyalgia or a syndrome know as costochondritis (also

called Tietze’s syndrome). Aspirin, local moist heat, and occasional injections into tender points are the treatments of choice.

Fluid that settles around the sac of the heart, or
pericardium
, can also produce a central chest pain that occurs when the patient is at rest, which is relieved by leaning forward.
Angina pectoris
, or decreased blood flow to the coronary arteries with pain resulting from lack of oxygen, is just as likely if not more likely to occur in lupus patients as in anybody else. However, it often has an earlier onset in patients taking long-term steroids that can be a sign of coronary vasculitis or myocarditis as well as atherosclerosis of the coronary arteries (discussed later in this chapter). Chest pain of
pulmonary origin
is common with pneumonia or a pulmonary clot (embolus) and may have nothing to do with

lupus.

Other Cardiac Symptoms

A persistently rapid heartbeat, called
tachycardia
, is a feature of active lupus resulting from inflammation or fevers. Other causes of tachycardia, such as infections or an overactive thyroid gland, must be considered. Rapid heartbeats

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are treated with anti-inflammatory medications and occasionally beta-blockers

(e.g., Inderal, Tenormin).
Irregular heartbeats
are noted when the myocardium (heart muscle) or pacing system of the heart is inflamed or scarred.
Shortness
of breath
can be due to lung disease (such as asthma) or a failing heart muscle that cannot provide adequate cardiac output.

CARDIAC DISORDERS

What Is the Pericardium?

Heather was recently diagnosed with lupus on the basis of skin rashes,

fatigue, and a positive ANA (antinuclear antibody) test. She began expe-

riencing chest pressure, a low-grade fever, and a rapid pulse associated

with shortness of breath. The chest pain diminished in intensity when she

leaned forward. Her doctor obtained a chest x-ray, which showed an en-

larged heart shadow as well as small pleural effusions. An electrocardio-