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Systemic Lupus Erythematosus

Systemic lupus erythematosus (lupus) is an autoimmune disease that results in episodes of inflammation in joints, tendons, and other connective tissues and organs.

Different tissues and organs become inflamed in different people, and the severity of the disease ranges from mild to debilitating, depending on the number and variety of antibodies that appear and the organs affected. About 90 percent of the people who have lupus are young women in their late teens to 30s, but children, mostly girls, and older men and women can also be affected.
Occasionally, certain heart drugs (hydralazine, procainamide, and beta-blockers) can cause a lupus-like syndrome that disappears after the drug is discontinued.

Characteristics of Lupus

• Facial rash
• Skin rash
• Sensitivity to sunlight
• Mouth sores
• Fluid around the lungs, heart, or other organs
• Arthritis
• Kidney dysfunction
• Low white blood cell count or low platelet count
• Nerve or brain dysfunction
• Positive results of a blood test for antinuclear antibodies, followed in some cases by positive results of a more specific test for antibodies to double-stranded DNA
• Anemia

Symptoms

The number and variety of antibodies that can appear in lupus are greater than those in any other disease, and they--along with other unknown factors--determine which symptoms develop. Therefore, symptoms, and their severity and gravity, vary greatly from person to person. Lupus can be quite mild, or it can be devastating, disabling, or fatal. For example, in people who have antibodies that affect only the skin, the skin symptoms may be mild--protecting the skin from sunlight may even prevent them--or they may be severe and disfiguring.

Because symptoms vary greatly, lupus may resemble many other diseases. For example, the connective tissue of joints is commonly affected in lupus, and the arthritis that results may resemble rheumatoid arthritis. Lupus may resemble epilepsy or some psychologic disorders when the brain is affected.
Although lupus can be chronic and ongoing, it usually flares up intermittently. What triggers a flare-up of lupus in people who are predisposed to it often isn't known, although sunlight seems to be one factor.

Lupus may begin with a fever. A high fever can occur abruptly, or episodes of fever and a generally sick feeling (malaise) can come and go, sometimes for years. About 90 percent of people with lupus have joint inflammation, which ranges from intermittent mild aches to severe arthritis in several joints. Years of joint symptoms may precede other symptoms. In fact, many people who have lupus recall having growing pains as children. Long-standing joint inflammation can lead to deformity and permanent damage to the joint and surrounding tissue, but the bone doesn't erode as it does in rheumatoid arthritis.

Skin rashes are common, often occurring on the face, neck, upper chest, and elbows. The most characteristic is a red, butterfly-shaped rash that appears across the bridge of the nose and on the cheeks. Circular, raised bumps may develop. These rashes rarely blister or become raw. Mouth sores are also common. Mottled, reddish-purple areas may appear on the sides of the palms and on the fingers; swelling and redness may develop around the nails. Hair loss is common when the disease is active. In almost half the people who have lupus, the skin is highly sensitive to light; it may burn easily or a rash may develop after exposure to sunlight.

Occasionally, an inflammation develops and extra fluid accumulates in the membranes surrounding the lungs. This inflammation (pleurisy) can make deep breathing painful. Fluid may accumulate in the sac around the heart, resulting in pericarditis, which can cause severe, constant chest pain. Children, young adults, and blacks with lupus commonly develop swollen lymph nodes throughout the body, and about 10 percent of the people with lupus develop an enlarged spleen.

Sometimes the nervous system is affected, causing headaches, personality changes, seizures, and symptoms that resemble dementia, such as difficulty in thinking clearly. Strokes occur less often. Protein or red blood cells in the urine, detected by a laboratory test, indicate kidney damage caused by glomerulonephritis, an inflammation of the kidneys, which is a common consequence of lupus. If severe, progressive kidney disease develops, blood pressure can become dangerously high, and kidney failure, which may be fatal, can follow. Early detection and treatment of kidney damage in people who have lupus reduces the incidence of severe kidney disease.

Diagnosis

Lupus is diagnosed mainly on the basis of its symptoms, particularly if they occur in a young woman. Because of the wide range of symptoms, distinguishing lupus from similar diseases can be difficult at first.

Laboratory tests can help confirm the diagnosis. A blood test can detect antinuclear antibodies, which are present in almost all people who have lupus. However, these antibodies also occur in other diseases. Therefore, if antinuclear antibodies are detected, a test for antibodies to double-stranded DNA is also performed. A high level of these antibodies is almost specific for lupus, but not all people who have lupus have these antibodies. Blood tests to measure complement levels (a group of proteins that are part of the immune system) and to detect other antibodies may be performed to predict the activity and course of the disease.

Kidney damage from lupus may be detected by blood and urine tests. Sometimes a biopsy of kidney tissue must be performed to help the doctor plan treatment.

Prognosis and Treatment

Because the course of lupus is unpredictable, the prognosis varies widely. The disease tends to be chronic and relapsing, often with symptom-free periods that can last for years. Flare-ups rarely occur after menopause. The prognosis has improved markedly over the last two decades. Usually, if the initial inflammation is controlled, the long-term prognosis is good.

If the symptoms of lupus are caused by taking a drug, discontinuing the drug cures the lupus, although the recovery may take months.
Treatment depends on which organs are affected and whether the disease is mild or severe. Mild lupus is characterized by fever, arthritis, rash, mild heart and lung involvement, and headaches. Severe lupus may cause life-threatening blood disorders, massive heart and lung involvement, significant kidney damage, vasculitis of the arms and legs or gastrointestinal tract, or severe nervous system dysfunction.

Mild disease may require little or no treatment. Nonsteroidal anti-inflammatory drugs (NSAIDs) often can relieve joint pain. Aspirin is used in low doses if the person's blood has a tendency to clot, as happens in some people with lupus; doses that are too high can harm the liver. Hydroxychloroquine, chloroquine, or quinacrine, sometimes taken in combination, helps relieve joint and skin symptoms.

Severe disease is treated immediately with a corticosteroid such as prednisone. The dose and duration of treatment depend on which organs are affected. Sometimes an immunosuppressive drug such as azathioprine or cyclophosphamide is given to suppress the body's autoimmune attack. The combination of a corticosteroid and an immunosuppressive drug is most often used for severe kidney or nervous system disease and vasculitis.

Once the initial inflammation is controlled, a doctor determines the dose that most effectively suppresses the inflammation over the long term. Usually, the dose of prednisone is gradually decreased when symptoms are controlled and laboratory test results improve. Relapses or flare-ups can occur during this process. For most people who have lupus, the dose of prednisone can eventually be decreased or discontinued.

Surgical procedures and pregnancy are more complicated for people who have lupus, and they require close medical supervision. Miscarriages and flare-ups after childbirth are common.

 

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