Home Cardiopulmonary Disease and Lupus
Elliot K. Chartash, M.D.
North Shore University Hospital - New York University School of Medicine

The heart and lungs are frequently affected in people with systemic lupus erythematosus (SLE) and can cause a variety of problems, ranging from mild to serious or even life-threatening. It is very important to know the differences between cardiopulmonary (heart and lung) complications and non-lupus related problems. When investigating a person with symptoms of a cardiopulmonary problem, a number of possible causes must be considered. Some of these, such as pericarditis, myocarditis, coronary vasculitis, pleuritis, pneumonitis, or pulmonary emboli can occur in SLE. Others, such as infectious pneumonia, esophageal spasm, reflux esophagitis, and costochondritis are not necessarily lupus-related. The medical examination and laboratory and other tests will help to determine the cause of the problem so appropriate therapy can be used.

Cardiac (Heart) Involvement

Lupus can involve all parts of the heart: the pericardium (sac surrounding the heart); the myocardium (muscle layer); the endocardium (lining of the inside of the heart); and the coronary arteries.

Pericarditis

Pericarditis, or inflammation of the sac around the heart, is the most common heart structure involved in people with lupus. This condition occurs when antigen-antibodies, or immune complexes, made during active SLE, cause inflammation within the pericardium. The usual symptoms include sharp chest pain and occasionally, shortness of breath. The pain can change with changes in position. Frequently, it is relieved by leaning forward slightly. This chest pain may feel like a heart attack. In some cases of pericarditis some individuals may not experience physical symptoms.

Blood tests, chest x-rays, an EKG, and an echocardiogram may be ordered to help diagnose pericarditis. The echocardiogram is an ultrasound of the heart that will tell the physician if there is fluid around the heart. Pericarditis can occur in conditions other than lupus; therefore the cause must be determined before treatment begins. If pericarditis is due to infection or kidney failure, the treatment is different than if it is due to lupus. Lupus pericarditis can be treated with anti-inflammatory agents. If this form of therapy is unsuccessful, a brief course of corticosteroid treatment is usually needed.

Myocarditis

When lupus causes inflammation of the myocardium, myocarditis occurs. However, serious heart muscle disease is not common in SLE. The symptoms of myocarditis include: unexplained rapid heart beat; abnormal electrocardiogram; irregular heart beat; and heart failure. Myocarditis is often seen with inflammation of other muscles in the body. Treatment of lupus myocarditis usually includes corticosteroids. Immunosuppressive drugs such as Cytoxan (cyclophosphamide) and Imuran (azathioprine) may be added of the inflammation is not completely controlled with corticosteroids. Myocarditis can lead to tissue damage that replaces heart tissue with scar tissue.

Endocarditis

When lupus causes inflammation of the endocardium (endocarditis), the heart valves can be damaged, but this condition rarely affects the pumping efficiency of the heart. The surface of the valves may become thickened or develop wart-like growths called Libman-Sacks lesions. Although these growths may cause heart murmurs, it is uncommon for them to seriously affect the function of the valves. If bacteria lodge in the growths, infection (bacterial endocarditis) can occur. This too is uncommon, but is potentially very serious and requires hospitalization. Rarely does the inflammation and scarring of valves lead to a deformity requiring valve replacement.

Coronary Artery Disease

The coronary arteries can become prematurely narrowed in people with SLE. These arteries deliver blood and oxygen to the heart muscle and are vital to the heart’s pumping function. Narrowing or blockage of an artery (coronary artery disease) can lead to chest pain and a heart attack. The narrowing of the coronary arteries in people with lupus may be due to inflammation of the artery wall (arteritis), cholesterol deposits inside the wall (atherosclerosis), arterial spasm, or blood clots.

Atherosclerosis is the most common cause of coronary artery disease in lupus. Studies suggest that people with lupus are more likely to develop premature atherosclerosis if they are on corticosteroids or have kidney involvement. Prevention is the primary treatment of coronary artery disease. Controlling cardiac risk factors and lupus disease activity and carefully monitoring corticosteroid use are all required to prevent heart attacks in people with lupus.

Heart damage can develop from inflammation in active lupus or from medications. Treatment of cardiac problems must be individualized for each person and for each problem. Early and accurate diagnosis, combined with aggressive therapy to reduce organ damage, is crucial in order to minimize permanent heart damage. Typical tests include a chest x-ray, EKG, echocardiogram, and blood tests to evaluate lupus activity.

Pulmonary (Lung) Involvement

Lupus can affect the lungs in many ways. Pleuritis (pleurisy) is the most common pulmonary manifestation of SLE. The pleura is a membrane that covers the outside of the lung and the inside of the chest cavity. It produces a small amount of fluid to lubricate the space between the lung and the chest wall. When lupus activity generates immune complexes, they start an inflammatory response at this membrane, a condition called polaritis. Sometimes an excessive amount of fluid builds up in the pleural space between the lung and the chest wall. This is called a pleural effusion and occurs less often then pleuritis. If the effusion is large enough, it can be seen on a chest x-ray. Since infection or conditions other than lupus can cause pleural effusions, the physician may need to take a sample of the fluid and perform tests to help determine its cause.

Pleural Disease

Symptoms of pleuritis include severe, often sharp, stabbing pain that may be pinpointed to a specific area or areas of the chest. Sometimes the pain is made worse by taking a deep breath, soughing, sneezing, or laughing. Analgesics, non-steroidal anti-inflammatory drugs (NSAIDs), and/or corticosteroids may be used to treat pleuritis. Pleural effusions will usually respond to these medications or clear by themselves with time.

Lupus Pneumonitis

Pneumonitis is inflammation within the lung tissue. Infection is the most common cause of pneumonitis in people with lupus. Bacteria, virus, or fungi are organisms that can cause infection in the lung. Sometimes pneumonitis may occur without infection and is then called non-infectious pneumonitis. Since both forms of pneumonitis have the same symptoms (e.g., fever, chest pain, shortness of breath, and cough), the person is assumed to have an infection until proven otherwise. The diagnosis of pneumonitis requires blood tests, sputum tests, and x-rays. Bronchoscopy (a visual inspection of the inside of the lungs) and/or lung biopsy may also be necessary to determine if infection is the cause of the pneumonitis.

Treatment of pneumonitis initially includes a course of antibiotics. If laboratory and other diagnostic tests show no proof of infection, then the diagnosis is likely lupus pneumonitis. This non-infectious pneumonitis is treated with high doses of corticosteroids. Immunosuppressive drugs such as Imuran (azathioprine) may be added if the inflammation is not controlled with corticosteroids.

Chronic Diffuse Interstitial Lung Disease

Chronic diffuse (widespread) interstitial lung disease is relatively uncommon in people with SLE. The symptoms include: gradual onset of a chronic, dry cough; pleuritic-like chest pains; and difficulty breathing during physical activity. Besides lupus, there are other reasons for this condition. To determine the cause, special procedures are required, such as bronchoscopy, bronchoalveolar lavage, and/or lung biopsy. Correct identification of the cause is necessary in order to chose the proper treatment. Chronic interstitial lung disease scars the lung. This scarred tissue acts as a barrier to the oxygen that normally moves easily from the lung into the blood.

The progression of chronic interstitial lung disease can be measured. The pulmonary function test assesses the ability of the lungs to receive, hold and use air. The oxygen saturation test measures how readily oxygen moves through the lung and into the blood stream. Oxygen saturation is usually reduced in chronic interstitial lung disease. High-resolution CT scans are used to assess disease activity and the person’s response to therapy. Chronic lupus interstitial lung disease is primarily treated with corticosteroids, with varying results. In general, the lung function can be stabilized with treatment.

Pulmonary Hypertension

Occasionally, people with lupus develop pulmonary hypertension of high blood pressure in the blood vessels within the lung. If severe, this can be life-threatening, and there tends to be little chance for improvement. There is no uniformly successful medical treatment for pulmonary hypertension. Heart-lung transplants may be an option for some people with pulmonary hypertension caused by SLE.

Pulmonary Emboli

Pulmonary emboli are blood clots that block the pulmonary arteries. At first, they cause pleuritis (lung) pain and shortness of breath. These clots can lead to abnormal oxygen exchange in the lung and even death. Tests for the presence of pulmonary emboli include ventilation-perfusion (breathing and blood flow) scans of the lung, and angiography (dye injected into an artery). There may also be an evaluation for thrombophlebitis (inflammation of a vein due to a blood clot). Risk factors in those with SLE are antiphospholipid antibodies, decreased blood levels of protein S, possible vascular damage, and prolonged bed rest.

Pulmonary Hemorrhage

Pulmonary hemorrhage, or bleeding into the lung, is a rare but potentially fatal complication of SLE. Typically, individuals have fever, shortness of breath, a cough, and blood-tinged sputum. This is usually seen in the setting of multi-organ system involvement from SLE and a rapidly falling red blood count. Treatment usually includes high-dose corticosteroids with immunosuppressive agents. Aggressive supportive care is also crucial to maximize chance for recovery.

The wide range of pulmonary manifestations associated with SLE needs prompt evaluation and close monitoring. Diagnostic tools can include: chest x-ray; ventilation-perfusion scan of the lung; gallium scan; high-resolution CT scan; bronchoalveolar lavage; or pulmonary function tests. Although the usefulness of all these tests varies depending on the type of lung involvement, close follow-up and monitoring are crucial to minimize, close follow-up and monitoring are crucial to minimize long-term complications.

Conclusion

The broad array of cardiopulmonary problems associated with SLE requires a close working relationship between patient and physician. Preventive measures to reduce the number of flares and rapid evaluation of new or changing symptoms are crucial to minimize long-term problems. Treatment is always individualized to the type of heart and/or lung involvement. Ongoing medical supervision is essential to optimize therapy and prevent long-term side effects.