Lupus and the Heart

 
G.W. Dennish M.D. FACC
Chairman, Cardiology Department, Scripps Memorial Hospital, La Jolla, California
Clinical Professor of Medicine, University of California, San Diego
 
Introduction
 
“Lupus,”or systemic lupus erythematosus (SLE) is a disorder that may affect many different parts of the body including the skin, joints and kidneys. It is generally not known that lupus can involve the heart in several different and important ways. The heart consists of several different types of tissue including muscle and connective tissue and each of these tissues can be involved by inflammation or scarring. In addition, the blood vessels that supply the heart (as well as other organs) can be similarly damaged leading to premature narrowing of the arteries (hardening of the arteries). Furthermore a connective tissue that surrounds and cushions the heart can become inflamed. The end result of these processed can be the development of chest pain, shortness of breath, palpitations, swelling of the extremities and other symptoms.
 
Types of Cardiac Involvement
 
Pericarditis The most common presentation of cardiac involvement in lupus is pericarditis. The pericardium is a connective tissue that surrounds the heart and cushions it in the chest. When this becomes inflamed by lupus or other inflammatory dise ases it can cause chest pain. Although the pain of pericarditis can be confused with that of a “heart attack” or myocardial infarction, it is usually quite different and is more like “pleurisy” that is, it is worsened by taking deep breaths and may be made worse by changing positions, leaning forward or lying down. Pericarditis is usually not life threatening but can be quite painful. A lupus patient who develops chest pain should seek medical attention immediately so that its cause can be differentiated from that of a true heart attack, and so that correct treatment can be initiated. Aspirin in high doses may be the first line of therapy but other more powerful agents such as corticosteroids may be necessary. Non-steroidal anti-inflammatory agents like ibuprofen are commonly prescribed. Pericarditis usually resolves with treatment but may recur requiring repeat courses of therapy. Rarely, surgery is necessary to remove the inflamed pericardium. Occasionally excessive fluid may accumulate around the heart between the pericardium and heart muscle and may lead to other problems.
 
Valvular Disorders Just as the connective tissue of the pericardium may become inflamed so too can the valves inside the heart can be affected. Up to 50% of lupus patients have some valvular involvement, but it is usually mild and only evident in 20% of patients. Inflammation of the valves usually causes no symptoms initially and most patients will have complete resolution of valvular involvement, but after a period of time scarring can occur and results in damage to the valve. This can cause “leaking” or improper blood flow inside the heart. Ultimately this can lead to excessive fluid accumulation and “heart failure”. Less than 20% of patients actually develop significant leak over a period of five years. When present, medical therapy is usually adequate to control symptoms and progression of the problem, Very rarely is more aggressive treatment including surgical valve replacement necessary. Patients who have this type of  heart damage should be given antibiotics before most dental procedure (including cleaning) and some general surgical procedures.
 
Myocarditis Occasionally the heart muscle proper can become inflamed in lupus patients. Chest pain, similar to what occurs with pericarditis, or shortness of breath may occur.
Once again, most patients will have minimal symptoms, if any, but in more severe cases the heart muscle becomes permanently damaged. The heart can enlarge and the muscle may become weakened and “flabby” leading to congestive heart failure. Medical treatment includes anti-inflammatory agents as well as specific therapy for heart failure when it occurs.  Rarely does surgical treatment become necessary.
 
Arrythmias  There are specialized tissues inside the heart that initiate and conduct electrical impulses that signal the heart muscle to contract in an organized fashion. These tissues can become inflamed and permanent damage can occur later if the structures become scarred. During the early, inflammatory phase the heart can become overly exciteable and cause extra heart beats usually perceived as an irregularity of the heart rhythm or “palpitations”. These “arrythmias” do not usually need treatment, but if very troublesome to the patient medical treatment is very effective. Very rarely are they dangerous. If the conduction system becomes damaged by scarring, signals may be blocked and the heart can beat too slowly. In very unusual circumstances this slowing might be serious enough to require treatment with a pacemaker.
 
Coronary Artery Disease Coronary artery disease (CAD) or atherosclerosis, hardening of the arteries, remains the number one killer of both men and women in western countries. Patients with lupus are now known to be at higher risk for the development of CAD. Women tend to have lupus more often than men and when CAD develops in women it tends to become symptomatic in the 3rd and 4th decades of life. It will frequently become manifest with chest discomfort sometimes pressure, shortness of breath, profuse sweating, profound nausea, vomiting, diarrhea or weakness. It is said that so called typical symptoms of severe chest pressure with radiation down the left arm are less common than in men making women less likely to seek early medical attention. The incidence of CAD in women with lupus is not known but is higher than in women without lupus of similar age. Of course men who have lupus also can develop CAD at an accelerated rate.
Risk factors for developing CAD in lupus patients include longer duration of the disease, longer duration of steroid treatment, high blood pressure that may occur from associated kidney disease or steroid treatment, abnormal blood lipids (cholesterol etc.)and obesity. Several of these risks including elevated blood pressure and cholesterol levels and obesity may be directly related to long term treatment with steroids.
An indirect complicating factor that may cause worsening of CAD symptoms is the adverse effect that lupus can have on the blood. This includes a tendency for blood to clot more easily in some patients thus leading to possible blockage in the coronary arteries.
Rarely are the coronary arteries directly involved by inflammation or “arteritis” which can also cause damage and ultimately blockage of the arteries.
 
Diagnosis
 
When a patient with lupus develops cardiac symptoms such as chest pain or shortness of breath, they should seek medical attention as soon as possible. Most of the cardiac manifestations of lupus are not immediately life threatening but may become serious if untreated. Particularly, chest pain or shortness of breath can be caused by both benign and serious complications of lupus and only a trained health care professional can properly assess these symptoms. 
Standard tests that are frequently very helpful include routine blood count and markers of inflammation as well as specific factors present in lupus that can be measured.
An electrocardiogram (EKG) may show signs of inflammation or damage but may not be specific.
Chest X Ray may be helpful to exclude other non cardiac causes of discomfort.
A very helpful and extremely specific test is the echocardiogram. During this test, sound waves are bounced off of the heart from a transmitter or transducer. When the sound wave return to the transducer which is also a receiver, the signal are reconstructed to show an extremely accurate image of the heart including the pericardial sac, heart valves, and heart muscle. There is no radiation involved the test is essentially painless.
 
Treatment
 
As listed above, most of the cardiac conditions seen in lupus patients are relatively mild and benign. Some patients never know they had any heart involvement. Those who do get symptoms such as chest pain can usually be treated successfully with standard medications such as aspirin, other non-steroidal anti-inflammatory agents and occasionally more powerful drugs such as corticosteroids or antimetabolic agents. Those patients who do require corticosteroids for long periods, either because of lupus involving the heart or any other organ, should have their risk factors for developing coronary artery disease closely monitored by their physician. These include blood pressure and levels of cholesterol and other blood lipids and markers. No patient should really use tobacco products but lupus patients, particularly if they are taking corticosteroids, should discontinue this dangerous habit. Diet should be appropriate to maintain ideal weight, something that can be quite difficult for patients taking steroids, as blood pressure and lipids are usually much better in lean individuals. A patient with a family history of premature coronary artery disease should be particularly vigilant, should be seen by their physician frequently and should probably undergo complete risk factor evaluation at an earlier age than usually recommended, probably by age 40. 
 
Patients who have lupus and one of the cardiac complications noted above can, in the vast majority of cases, have their cardiac condition controlled by standard medications and a healthy lifestyle.