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Lupus and the Heart-Joint Connection

Often described as the disease with a thousand faces, systemic lupus erythematosus (SLE) is a chronic autoimmune disorder. It typically causes fatigue and joint pain, can injure nearly any internal organ, and increases the risk of cardiovascular disease. A recent Canadian study in Arthritis and Rheumatism found that the incidence of heart attack and stroke in SLE patients was 7 to 17 times higher than what would be predicted based on traditional cardiovascular risk factors, such as elevated cholesterol, hypertension, diabetes, and smoking. Although SLE typically strikes young women in their childbearing years, a large number of older adults have the condition. About 15% of cases are first diagnosed after age 55, and more SLE patients are enjoying a lengthy life thanks to improved drug regimens—with more advances on the horizon (see box).

What Is Lupus?

Derived from the Latin term for wolf (lupus) and the Greek word for red (erythematosus), the disorder's name refers to the red facial rash (which reminded some observers of a wolf bite) that often develops in lupus patients. Systemic lupus is an immune system disorder. Normally, the immune system makes antibodies that protect the body by attacking foreign invaders such as viruses and bacteria. In SLE, however, the body produces so-called autoantibodies that attack and destroy normal cells, causing inflammation, injury, and pain in various tissues and organs.

Although the exact cause of SLE is unknown, genetic and environmental factors are thought to play a role. In addition to SLE, there are two other forms of lupus: drug-induced and cutaneous lupus. Drug-induced lupus may appear after the use of medications such as hydralazine (an antihypertensive) or procainamide (used to treat abnormal heart rhythms). Although the symptoms of this form of lupus are similar to those of SLE, they usually resolve within a few days to weeks after the offending drug is stopped. Cutaneous lupus erythematosus, a milder form sometimes manifested by coin-shaped lesions on the face, neck, and scalp, evolves into systemic disease in about 10% of patients.

Recognizing Lupus

SLE manifests itself in many ways and can affect virtually any part of the body. Onset may be sudden or gradual over years. Hallmarks of the disease include:

Joint pain. Frequently accompanied by redness and swelling, especially in the fingers, hands, wrists, and knees, joint pain occurs in 90% of patients— sometimes years before other symptoms.

Skin disorders. They may be manifested as a butterfly-shaped rash across the cheeks and bridge of the nose (50% of patients); coin-shaped lesions, often on the face (25% of patients); recurrent ulcers in the mouth and nose; and transient hair loss. About 30% develop Raynaud phenomenon, a disorder marked by poor circulation in the small blood vessels of the extremities.

Fatigue or fever. Both occur in 90% of patients. Caused by inflammation rather than infection, fever is often low grade but may rise during flare-ups.

Kidney involvement. About half of patients develop lupus nephritis, a persistent inflammation of the kidneys. Such individuals may eventually experience renal failure and require dialysis or kidney transplantation.

Blood disorders. Almost 85% of patients experience low red and white cell and platelet counts or other blood disorders. Low platelets may cause easy bleeding and easy bruising. An antibody paradoxically called the lupus anticoagulant can predispose the blood to clot, raising the risk of venous and arterial thrombosis, heart attack, and stroke.

Lung involvement. Many patients develop pleurisy (an inflammation of the membrane lining the lung) and pleural effusion (an accumulation of fluid between the lung and its lining). Symptoms include chest pain, difficulty breathing, and coughing.

Nervous system involvement. Neurologic disorders affect about a quarter of patients.

Symptoms typically include anxiety, irritability, depression, and mild impairment of memory and concentration, but seizures and psychosis can also occur. For some patients, the limitations imposed by their disease may also lead to depression.

Flare-ups interspersed with periods of improvement or remission are typical for SLE. In about 20% to 30% of patients, symptoms remain mild. Such patients may have only a skin rash and achy joints but no evidence of major organ involvement. In about 50% to 75% of patients, however, vital organs such as the kidney, heart, or lungs are affected. Symptoms can be exacerbated by infections, ultraviolet light, certain drugs, and hormones, especially estrogen—for example, symptoms have been observed to increase before menstruation and during pregnancy. In addition, both physical and emotional stress may trigger episodes.

Cardiovascular Connection

According to several recent studies, certain nontraditional risk factors—including low bone density and high homocysteine levels—may be tied to heart disease and stroke in people with lupus. A study in Arthritis and Rheumatism reported that women with SLE who had decreased bone mineral density had increased amounts of plaque in their carotid arteries and signs of calcification in their coronary arteries, raising the risk of stroke and heart disease.

Michelle Petri, M.D., an Associate Professor of Rheumatology at Johns Hopkins, and colleagues had previously uncovered a link between high homocysteine levels and an increased risk of stroke and arterial clots among patients with SLE. "Homocysteine levels are high in about 30% of lupus patients and should be checked," notes Dr. Petri.

And a recent Swedish study, reported in Circulation, also found that high homocysteine levels and osteoporosis (very low bone density) appeared to increase the risk of heart disease and stroke in people with SLE. The study also identified two other nontraditional risk factors for cardiovascular disease: the lupus anticoagulant and antibodies to oxidized low density lipoprotein (LDL, or "bad") cholesterol.

Long-term corticosteroid therapy, often needed to control disease symptoms, may also contribute greatly to the higher cardiovascular risk among SLE patients. High doses of corticosteroids for long periods can raise blood pressure, increase lipid levels, cause weight gain, and raise the risk of diabetes down the road-all factors that put lupus patients at greater risk for heart disease and stroke. In addition, prolonged corticosteroid therapy can lead to low bone density, cataracts, and mood swings. According to Dr. Petri, "The adverse effects of prolonged corticosteroid therapy are probably the number one cause of organ damage among patients with systemic lupus."

Lifestyle Measures

Conservative measures that discourage episodes include avoiding excessive sun exposure and using a sunscreen with a Sun Protection Factor (SPF) of at least 15. All patients with lupus should obtain sufficient rest (8 to 10 hours of sleep nightly and a nap, if needed, during the day), exercise regularly to help fend off muscle weakness and fatigue, and follow a healthy diet. Because lupus patients have limited energy, they should pace themselves by spreading out work and other activities over a longer period of time and slowing down before they become too tired. Immunizations to protect against influenza and pneumonia are generally recommended, and prompt treatment of infections is essential. Stress management is also important.

Drug Treatment

Early diagnosis and treatment can greatly improve outcome. With treatment, the 20-year survival rate is about 80%, and many individuals can look forward to a normal life span. Regular monitoring of organ function and heart disease risk factors is necessary. Patients with traditional and nontraditional risk factors should receive aggressive treatment to ward off cardiovascular disease.

Patients should generally be followed by a rheumatologist—even when they feel well. Although there is no cure, combination therapy can control symptoms. For some patients with mild disease, the only medication needed may be aspirin or a nonsteroidal anti-inflammatory drug (NSAID) to relieve muscle and joint pain and arthritis. For others with more severe symptoms, stronger medications may be required, at least on a short-term basis. They include:

Corticosteroids, which reduce inflammation and suppress the immune system. They are used to control severe or life-threatening complications such as kidney disease, central nervous system involvement, and hemolytic anemia. Some people need corticosteroids for brief periods; others may require long-term therapy. Combining corticosteroids with other drugs can sometimes reduce the risk of serious side effects because the corticosteroid dose can be reduced.

Cytotoxic drugs, which also suppress immune function and are most commonly used to manage widespread lupus flares and to treat serious kidney, neurologic, and arthritic symptoms. They can, however, produce anemia, a low white blood count, and an increased risk of infection; they may also increase the risk of cancer later in life, although the risk appears to be very small.

Antimalarial drugs, which are frequently given to treat the skin and joint symptoms associated with lupus. Side effects, though rare, include diarrhea and rashes. Because about 1 of every 5,000 people taking hydroxychloroquine may develop retinal changes, periodic eye examinations are recommended.

For more information:
Lupus Foundation of America

A Promising Future

Researchers are investigating more targeted SLE therapies that can avoid the potentially dangerous side effects associated with current treatments. Medications currently being investigated include dehydroepiandrosterone (DHEA), a mild male hormone; bromocriptine, which reduces prolactin levels (a pituitary hormone that is elevated in SLE); LJP 394 and leflunomide (Arava), which zero in on particular aspects of the immune system (such as the antibodies responsible for lupus kidney disease or the immune cells responsible for inflammation); genetically cloned (monoclonal) antibodies to immune system proteins, which are thought to play a role in triggering SLE; and transplantation of blood-forming stem cells (which produce red and white blood cells and platelets) in conjunction with cyclophosphamide (Cytoxan), a cytotoxic drug. It now appears that short-term high-dose cyclophosphamide therapy can be effective without stem cell transplantation.

From The Johns Hopkins Medical Letter: Health After 50, April 2002.



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