Heart and Lung Involvement and Lupus: What You Need to Know

1. Where Do I Begin?
2. What is My Next Step?
3. What are the Symptoms of Heart and Lung Disease?
4. Intervention
5. Percutaneous Intervention
6. TABLE I

Systemic lupus erythematosus (SLE) as its name implies, (redness of the organs from the bite of a wolf) is a disease that affects all body parts. The heart and lungs are no exception. Patients with lupus may develop any combination of heart and lung disease in addition to involvement of other organ systems (Table I).

Systemic hypertension (high blood pressure) and hypertensive heart disease (thickening of the walls of the heart that occurs as a result of chronic high blood pressure) are common in patients with lupus, with or without associated kidney disease.

Another potential problem lupus patients face is pericarditis, an inflammation of the sac surrounding the heart. Patients may experience chest pain, and fluid may accumulate around the heart (pericardial effusion). If the fluid becomes constricting to the heart, symptoms such as shortness of breath and swelling of the legs and abdomen may occur.

Coronary artery disease is a blockage in the arteries of the heart. Coronary artery disease is dramatically increased in patients with SLE. Blockage of coronary arteries can result in chest pain (angina pectoris), heart attacks, damage to heart valves, electrical abnormalities of the heart beat (arrhythmias) and sudden cardiac death. For women 35 to 44 years of age with SLE, the rate ratio of heart attacks is over 50 times that for a comparative population without SLE.

SLE may cause inflammation of the heart valves and subsequent damage to the valvular mechanism with leakage, which can cause shortness of breath (congestive heart failure).

Disease of the small arteries of the lung in SLE may cause pulmonary arterial hypertension, a serious condition which is difficult to treat.

Lupus may affect the lungs in a variety of ways. Lupus can cause inflammation of the support mechanism of the lungs (parenchyma) or the lining of the lungs (pleura) making it difficult to breathe. It may also impair the movement of the diaphragm, leading to shortness of breath.

Because of the systemic nature of SLE, patients are more likely to get lung infections (bronchitis and pneumonia) than the general public, and patients with SLE have more difficulty recovering from these infections.

In addition to lupus causing cardiopulmonary disease, the side-effects of treatment may also be harmful. Specifically, the use of corticosteroids may elevate blood pressure by causing salt and water retention. Steroids are thought to be involved in the development of plaque in arteries by causing the "metabolic syndrome" with elevation of blood glucose, cholesterol and triglycerides.

For a more extensive description of lupus-related disorders of the heart and lungs and their treatment, see Lupus Foundation of America’s summary, Cardiopulmonary Disease and Lupus, By Elliot K. Chartash, M.D.:

http://www.lupus.org/education/brochures/cardio05.html.

Where Do I Begin?

Detection of cardiopulmonary problems begins with a thorough physical examination. During an office visit your health care provider (HCP) can check you for hypertension and signs of cardiovascular and cardiopulmonary disease.

Looking at the eyes with an ophthalmoscope, the provider can look at the blood vessels of the retina (a fundoscopic exam). Patients with SLE often have a specific type of arterial abnormality called "copper wiring." Evidence of increased cholesterol and hypertension can be graded by looking at the arteries of the retina.

Examination of the skin may provide clues about cardiovascular health as well. The presence of certain lesions of the skin, xanthoma and xanthelaema (the yellow accumulation of cholesterol) indicates that a patient’s lipid profile is extremely elevated.

The provider listens to the arteries in the neck and abdomen with a stethoscope. The presence of abnormal sounds, or bruits, can indicate arterial narrowing caused by plaque. The stethoscope can also detect heart murmurs and extra sounds called gallops which indicate stiffness and weakness of the heart muscle.

Examination of the neck veins is important in looking for cardiovascular disease. Swelling of the neck veins can indicate increased pressure in the right side of the heart. Listening to the heart in a patient with distended neck veins may reveal accentuated heart sounds associated with both systemic and pulmonary hypertension (elevation of pressure in the blood vessels of the lungs.)

Examination of the abdomen can reveal an enlarged liver. If the HCP presses on the abdomen and the veins in the neck distend, this is an indication of occult right sided congestive heart failure. If a patient coughs during this maneuver (called the hepatojugular reflux) it indicates the left side of the heart is also diseased.

Laboratory testing should include urinalysis and blood tests. Testing for urine microalbumin may indicate that hypertension has led to kidney damage or that there is a primary disorder of the kidney from lupus. A complete blood count would include checking for an elevated white blood cell count to rule out pulmonary or other infections and to rule out anemia, which can make the heart work harder and precipitate heart failure.

A cholesterol profile should include assessment of the LDL or low-density lipoprotein (the "bad" cholesterol) as well as the HDL (high-density lipoprotein or good cholesterol). In the general population, a patient should aim for LDL cholesterol less than 100 mg/dl. In rural populations who subside on vegetarian and fish diets, LDL cholesterols are as low as 70mg/dl. These populations have little coronary artery disease. Hence, cardiologists are now urging patients to try to reduce their LDL cholesterol to even less than 70 mg/dl.

Specialized cardiovascular blood tests include: the cardiac C - reactive protein: which measures cardiovascular inflammation; homocysteine, a protein which can cause vascular damage; lipoprotein-a, which is an LDL molecule with an attached protein which may also independently cause vascular damage.

If your lipids are abnormal, or if there is a family history of premature coronary heart disease (age at onset less than 55 years old) your provider may send your blood to a laboratory which can sub-fractionate your HDL and LDL. There are five different HDL molecules (Type II b being the most important for vascular health). There are seven different subsets of LDL with small Class III and IV being more damaging to the arteries than the large or "fluffy" Type I and Type II LDL molecules.

Fibrinogen is another important factor, which should be included in the evaluation of a patient with SLE. It is a protein produced by the liver, which is involved in the formation of blood clots. While fibrinogen is helpful in the presence of bleeding, a chronically elevated fibrinogen may lead to a predisposition for heart attacks and strokes by increasing the possibility of clots and plaques in arteries.

A chest x-ray should be performed in patients with SLE to assess for heart size and evidence of lung disease.

A routine electrocardiogram (ECG) may show subtle changes, which may lead your HCP to have an increased index of suspicion about heart disease. A Cornell cardiologist, Dr. Peter Okin, recently published a paper in Diabetes showing that ST-T wave changes seen on electrocardiograms may identify patients at increased risk for heart disease. Hence, an ECG may enable your healthcare provider to increase his or her suspicion that you have an unrecognized heart problem.

If by physical examination, chest x-ray, electrocardiogram or blood tests your HCP suspects that you may have a heart problem, then echocardiography and stress testing should be considered, or referral to a cardiologist.

An echocardiogram is an ultrasound examination of the heart. It evaluates the ability of the heart muscle to contract and of the valves to work. Moreover, the echocardiogram may show calcification of the valves and aorta, which is a sign of potential systemic vascular disease.

Patients with lupus who are thought to be at high risk for having blockages should undergo stress testing. Stress testing evaluates how the heart responds to exercise, usually by walking on a treadmill. The preferred methodology is myocardial perfusion scanning. The nuclear isotopes thallium and/or technetium are injected into a patient’s vein, and images of the heart are taken before and after exercise. If arthritic conditions prohibit exercise, a "pharmacologic" stress test can be performed. Medications administered intravenously can make the heart behave as though the patient were exercising are administered. The images of both kinds of stress tests can be used to assess changes in the coronary circulation indicative of a significant (>70%) blockage.

Another commonly used diagnostic test in assessing for coronary blockages is the electron beam computerized tomogram (ECBT). The EBCT is a specialized type of "CAT" scan. It takes approximately twenty minutes to perform and will identify calcium in the coronary arteries (a sign of plaque and potential blockage). The higher the calcium score, the more likely that the blockages will actually prevent blood flow and cause future heart attacks.

All these diagnostic tests should be coordinated with the cardiologist who is seeing you.

What is My Next Step?

Asymptomatic?
Fifty percent of patients with heart disease have no symptoms. Because of the high prevalence of cardiovascular and cardiopulmonary disease in SLE, visiting a cardiologist for an examination, blood tests, electrocardiogram and chest x-ray every 5 to 10 years is warranted. Even those SLE patients without cardiopulmonary heart symptoms should see a cardiologist and be assessed for possible hidden disease before it becomes symptomatic. In all disorders, there is a latent phase during which disease is present before it becomes symptomatic.

What are the Symptoms of Heart and Lung Disease?

Patients with shortness of breath, chest discomfort associated with activity, or fatigue with activity in the absence of any other explanation such as severe anemia or renal failure should suspect cardiopulmonary disease. For example, if you frequently walk up a hill near your home and one day experience tightness in your chest or shortness of breath, this may be a sign of coronary blockage. It behooves you to discuss this with your HCP immediately, and to be assessed for a potentially serious coronary condition.

Intervention

Medical and surgical intervention in patients with SLE and cardiopulmonary disease is varied. It may be as simple as taking a medication such as a statin (e.g. Pravachol, Lipitor, Zocor or Crestor) to reduce your LDL and raise your HDL cholesterol. In patients with abnormal LDL subtypes and elevated lipoprotein-a, the use of niacin may be advised. In addition, Zetia has been used in combination with statins as a dual mechanism of reducing cholesterol The statins reduce production of cholesterol in the liver and Zetia reduces cholesterol absorption in the intestine.

It is important for patients who are placed on these drugs to realize that both liver and muscle abnormalities may occur, and that your HCP needs to check certain blood tests on a regular basis to rule out occult irritation of the muscle and liver. In addition, you should report muscle aches to your HCP immediately, as this may be the first sign of muscular inflammation from these medications. Combination therapy of hyperlipidemia increases the chance of muscle and liver irritation.

In addition, use of some antibiotics such as erythromycin, Zithromax or Biaxin may exacerbate irritation from the statins. When an antibiotic is prescribed, ask your physician whether you should stop taking one or more of your medications temporarily to avoid a drug-drug interaction.

Percutaneous Intervention

If a serious plaque is found in your heart, you may require an invasive procedure called angioplasty with balloon dilatation and stenting to unblock the arteries. This procedure involves an overnight stay in the hospital.

In cases where blockages in the arteries are diffuse and severe, coronary artery bypass graft surgery (i.e., open-heart bypass) may be necessary. However, the frequency in performing this operation has plummeted in the last decade, with the increased use of balloons and stents.

As with any disease, prevention is the best medicine. Ask your Health Care Professional about whether you are at risk of having cardiopulmonary disease from SLE. Do not ignore symptoms such as unexplained fatigue, shortness of breath or chest pressure. Healthcare begins and ends at home. It is your personal responsibility to make sure that you are getting the best medical attention possible. Get examined on a regular basis, ask questions, eat right, exercise and try to maximize your situation. While you may be despondent that lupus has affected your kidneys, skin or joints, don’t ignore the call from the wolf. Watch your heart and lungs!

Pericardium = sac/lining
Endocardium = valves
Myocardium = muscle
Coronary artery blockages (angina, heart attack, sudden cardiac death)

LUNGS

Pleura = sac/lining
Parenchyma = lung tissue
Arteries (pulmonary hypertension)
Diaphragm weakness

About HSS' SLE Workshop