The Lupus Book: A Guide for Patients and Their Families, Third Edition (16 page)

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rating oxygen when a patient breathes. By asking the patient to breathe in and

out of a balloon (i.e., performing a
pulmonary function test
), the doctor can help to determine whether an asthmatic or bronchospastic component is present, assess lung breathing capacities, and evaluate interstitial lung function. The interstitium is tissue that provides general support to the lung and facilitates the exchange of oxygen and carbon dioxide when we breathe. Interstitial lung function is tested by the diffusing capacity, which, if low, suggests a defect in the exchange of oxygen for carbon dioxide in the lung tissue rather than in the air sac.

Lung scans
are also used. They are painless, noninvasive nuclear medicine studies that can diagnose pulmonary artery blood clots called emboli, infection, or inflammation of the interstitium. A ventilation/perfusion scan is used to detect pulmonary emboli; an indium or gallium scan is used to detect inflammation of

the interstitium. If an infection is suspected or a diagnosis of active lupus needs confirmation, doctors sometimes perform a
thoracentesis
, which is the removal of the pleural fluid for analysis.
Bronchoscopy
permits the removal of lung tissue through a flexible, thin tube for diagnostic purposes. A washing of cells obtained through this procedure is called
bronchoalveolar lavage
; it enables physicians to analyze the cell types present in the lung tissue.
Pulmonary angiograms

(whereby dye is injected into the pulmonary arteries) are the ‘‘gold standard’’

for diagnosing pulmonary emboli but are not without risks. A
two-dimensional
(2-D)
Doppler echocardiogram
is a simple, noninvasive ultrasound procedure that enables doctors to estimate pulmonary pressures in most patients with significant pulmonary hypertension. As a last resort for diagnosing pulmonary dis-

ease, doctors can perform a surgical procedure known as an
open-lung biopsy
, but this is rarely necessary.

Pleurisy

John was a healthy young man until one day, upon taking a deep breath,

he felt an odd sensation in his chest. Thinking it would pass, he ignored

this symptom for several months. Finally, he measured his pulse and found

it to be fast. John consulted his family doctor, who obtained a temperature

of 99.8ЊF and a pulse of 120. Although he did not hear anything unusual

upon listening to John’s chest, a chest x-ray showed that there was fluid

lining the lungs on both sides. At this point, an ultrasound estimated that

John had a liter of fluid on his right side (around his lung). Since John felt

well otherwise, Dr. Smith called in a pulmonary consultant, who removed

the fluid. Under the microscope, the fluid showed LE (lupus) cells and an

elevated white blood cell count. A blood ANA test was ordered, which

came back positive, and a diagnosis of lupus was made. John was started

on 20 milligrams of prednisone (a steroid) a day along with indomethacin

(an anti-inflammatory drug) at 50 milligrams every 8 hours. Within 10 days,

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Where and How Can the Body Be Affected by Lupus?

John was off steroids and holding his own with indomethacin. Plaquenil

(an antimalarial drug) was then initiated, and John was able to stop indo-

methacin 3 months later.

What Is Pleurisy?

The principal symptom of pleurisy is pain on taking a deep breath. The thin

membrane sac enveloping the lung is known as the
pleura
. When it is inflamed, the term
pleuritis
is used. If fluid forms and seeps out of this membrane, there is a
pleural effusion
. The pleura does not contain lung tissue, so there should be no fear of lung disease.

Pleuritic pain (with or without effusions) is evident in 40 to 60 percent of

patients with systemic lupus. Effusions are found in about 20 to 30 percent of

patients, and for 2 to 3 percent of patients pleurisy is the initial manifestation of lupus. The pain can be on either side or both sides of the rib cage or it can be felt as front or back chest pain. At autopsy, more than 90 percent of lupus

patients show pleural abnormalities resulting from the disease. The lining of the lungs looks similar to the lining of the heart (
pericardium
) and the lining of the abdominal cavity (
peritoneum
). Under a microscope, the pleural membranes normally consist of a few layers of loose connective tissue, but in lupus the

tissue drastically thickens and shows signs of inflammation. Chemicals that the body makes as part of the inflammatory process irritate the lung and pleura and can eventually form scars and adhesions, resulting in further pain. Effusions are usually visible on a chest x-ray. Occasionally, their presence is confirmed when the patient is lying down and the fluid can be observed spreading into other

body cavities. Pleural fluid moves much like water in a glass which has been

turned on its side. An ultrasound or CT scan can also confirm fluid.

What Can Be Learned from Looking at Pleural Fluid?

The fluid made by an irritated pleura can be clear, a
transudate
, or cloudy, an
exudate
. Transudates, associated with simultaneous irritation of the pericardium and pleura, are observed clinically when the abdomen swells (a condition known

as
ascites
); when massive amounts of protein leak from the kidney (a condition called
nephrosis
); or when the kidney fails.

Exudates suggest three possibilities: active lupus in the organs, an infectious process, or a malignancy. By performing a
thoracentesis
, a procedure by which pleural fluid is withdrawn from the lung at the bedside and analyzed under the

microscope, a doctor can diagnose exudative effusions. Since lupus patients are susceptible to infections, pleural fluid is usually cultured.

How Is Pleurisy Treated?

In addition to infection, pleural-like pain is also observed in lupus patients who have rib fractures because of osteoporosis, trauma, or steroid use. Since 20

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percent of patients with systemic lupus have fibromyalgia (see Chapter 23), the physician will have to distinguish fibromyalgia from pleurisy, which share certain symptoms (chest wall tenderness, for example).

If pleuritic pain is present but a pleural effusion is not, the doctor may try a higher dose of a nonsteroidal anti-inflammatory drug (e.g., indomethacin). Prednisone given in doses up to 40 milligrams daily is also effective but is not always necessary. Radiographically evident effusions usually necessitate corticosteroid therapy. A group of anti-inflammatory drugs known as antimalarials (e.g., Plaquenil) decrease pleural inflammation over a several-month period. Rare in-

stances of recurrent pleural effusions may call for removal of the pleura, a

procedure known as
pleurectomy
, or for the introduction of irritating materials (such as talc, tetracycline, or quinacrine) into the pleural lining to prevent fluid from forming.

Acute Lupus Pneumonitis

Over a 2-day period, Anastasia noticed a temperature of 101ЊF, a dry,

hacking cough, and shortness of breath. She was taking 30 milligrams of

prednisone and 100 milligrams of azathioprine (Imuran) a day for systemic

lupus that involved autoimmune hemolytic anemia and low platelet counts.

She called her family doctor, who was unable to see her and prescribed

erythromycin over the telephone. Three days later, when she was still not

better, Dr. Matthews obtained a chest x-ray that showed an infiltrate in the

interstitial tissues. As an internist, he knew that these infiltrates are most

frequently associated with mycoplasmal pneumonia; therefore he hospital-

ized her for intravenous antibiotics. Three days later, Anastasia was still so

short of breath that she had to be transferred to the intensive care unit. A

pulmonary specialist and her rheumatologist were called to see her in con-

sultation. A bronchoscopy failed to show any evidence of infection. The

rheumatologist started her on high-dose intravenous steroids, and after a

few days her breathing improved.

Occasionally, lupus patients develop shortness of breath, a dry cough, pleuritic pain, and a blood-tinged sputum. More often than not, this signifies a bronchial infection or pneumonia. However, as in Anastasia’s case, lupus itself can inflame the lungs and produce a condition known as
acute lupus pneumonitis (ALP)
.

Seen in 1 to 9 percent of lupus patients during the course of their disease, ALP

affects the lung’s interstitium (its supporting tissue), which becomes inflamed.

ALP is easily observed on a chest x-ray. Most physicians are cued to look for

ALP when their patients’ symptoms do not clear up with antibiotics and the

chest x-ray remains abnormal. When a bronchoscope is used to take a tissue

biopsy of the lung, the physician will observe the interstitial areas of the lung filled with lymphocytes. Clots or vasculitis are rarely noted. Under an immu-

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Where and How Can the Body Be Affected by Lupus?

nofluorescent microscope, lung tissue is stained to determine the presence of

immune complexes, complement, and immunoglobulin.

If it is promptly managed with high doses of steroids, ALP can be completely

reversible. Drugs that decrease steroid requirements, such as azathioprine or

cyclophosphamide, are sometimes added. Despite this, it is unfortunate that up

to 50 percent of patients with ALP die within months, often due to a delay in

diagnosis.

Diffuse Interstitial Lung Disease

Florence was diagnosed with lupus 10 years ago and had only mild aching

along with sun-sensitivity. She also complained of dry, gritty eyes, and her

ophthalmologist recommended that she use artificial tears. Over the next

several months, Florence began having a dry, hacking cough with only

occasional shortness of breath. She consulted a pulmonary specialist, who

obtained a chest x-ray showing increased interstitial (within the tissue)

markings. Pulmonary function tests demonstrated mild restrictive abnor-

malities. Dr. Hughes ordered a gallium lung scan and interstitial markings

lit up, revealing where the gallium had collected. This indicated that the

interstitial changes were reversible. She was started on a moderate dose of

steroids and now feels a lot better.

When the symptoms described for acute lupus pneumonitis (ALP) evolve over

a several-year period, it is called diffuse
interstitial lung disease (ILD)
. This is a common complication of scleroderma, Sjo¨gren’s syndrome, mixed connective

tissue disease, and rheumatoid arthritis. It is seen in 10 to 20 percent of patients with systemic lupus. Florence’s case is not atypical. Indeed, the symptoms can

be so subtle that patients may not even tell their doctors about it at first. Most cases are finally identified after about 10 years of disease.

Chest x-rays along with a restrictive defect on pulmonary function testing

help diagnose ILD. A specialized imaging study known as a high-resolution CT

scan frequently sheds light on the degree and nature of pulmonary involvement.

Lung biopsies of ILD are occasionally necessary and appear similar to those of

ALP, though they are more chronic and less aggressive.

If identified early, ILD is responsive to treatment with steroids and immune

suppressive therapies. After years of the disease, the inflammation diminishes

and heals with scars. At this point, treatment is not helpful, since scars do not respond to anti-inflammatory medication. Although ILD rarely leads to respiratory failure, it leads to rapid, shallow breathing with decreased stamina in its chronic phase. In order for patients to be treated properly, ILD must be differentiated from chronic aspiration (swallowed material going down the wrong

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pipe), adverse reactions to medication, interstitial lung infections such as pneumocystis pneumonia, and environmental irritants.

Pulmonary Embolism

Gertrude noticed that her right leg was swollen and tender. This had never

happened before, and her lupus was in remission. Her friend, a nurse’s

aide, told Gertrude that it might be phlebitis and that she should see her

doctor. Her life was hectic and Gertrude couldn’t spare the time to have it

checked out. Three days later, Gertrude suddenly complained of severe

chest pain on the right side and couldn’t breathe. It was so severe that her

sister had to drive her to the nearest emergency room. Her arterial blood

gases showed low oxygen levels, so she was sent upstairs to radiology for

a lung scan, which confirmed the presence of a pulmonary embolus. It

turned out that a clot from the phlebitis in her leg had broken off and

traveled to the lung. Her serum anticardiolipin antibodies were positive,

suggesting a predisposition to form blood clots. Gertrude was admitted to

the hospital, given intravenous heparin followed by oral blood thinners

(Coumadin), and discharged a week later, very lucky to be alive.

One-third of lupus patients have antiphospholipid antibodies such as anticar-

diolipin (see Chapter 21), and one-third of these patients have a clot or thromboembolic episode during the course of their disease. One-third of these episodes consist of blood clots that travel through the blood and settle in the vessels of the lung; once there, they are called
pulmonary emboli
. Even though blood clots can be caused by something other than lupus, surveys have reported that 5 to