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Systemic lupus erythematosus, abbreviated as SLE, is an autoimmune disorder that affects many parts of the body. An autoimmune disorder is a condition in which the body, for reasons that medical science usually does not understand, creates antibodies against its own tissues.

What is going on in the body?

• brain and nervous system
• digestive system
• eyes
• heart
• joints and muscles
• kidney
• lung
• skin

What are the signs and symptoms of the disease?

Because SLE affects so many different parts of the body, the disease has many signs and symptoms. These are best listed by organ system.

It should be emphasized that an individual patients with SLE will exhibit some combination, not all, of these symptoms, and some have involvement of only one organ system. The symptoms come and go in an irregular time pattern. Some persons with lupus can identify "triggers" that usually bring on the symptoms.

• fatigue
• fever
• loss of appetite
• nausea
• weight loss

• hives
• loss of scalp hair
• red "butterfly rash" on the face, which extends over the cheeks and bridge of the nose in the shape of a butterfly
• red, raised rashes in areas exposed to the sun
• sensitivity to the sun
• ulcers of the mouth, nose, or vagina

• arthritis
• hand deformities
• joint pain, especially in the hands, wrists, and knees
• loss of the blood supply to bone, especially hips and shoulders
• muscle pains and weakness
• serious infection within a joint

• blood in the urine
• decrease in kidney function or kidney failure
• protein in the urine
• white blood cells in the urine

• bleeding in the brain, known as intracerebral hemorrhage
• headaches, including migraines
• loss of coordination
• memory loss, problems with concentration
• numbness, pain, or a feeling of "pins and needles" in the hands and feet
• problems with the major nerves in the head and face
• seizures
• strokes

• anemia, which is a low red blood cell count
• low blood platelet count
• low white blood cell count
• problems with both blood clotting and bleeding

• fluid collection in the sac around the heart, known as pericardial effusion
• heart attack
• heart valve problems
• inflammation and infection in the heart
• inflammation of the lining of the heart

• cough, including coughing up blood
• fluid in the lung
• inflammation in the lung
• inflammation of the lining of the lungs
• shortness of breath

• abdominal distress
• diarrhea
• enlargement of the liver
• intestinal perforation due to inflammation of blood vessels
• loss of appetite
• nausea and vomiting
• rectal bleeding caused by ulcerations

• blindness
• conjunctivitis, which is inflammation of the outer lining of the eye
• damage to the retina, which may cause visual impairments

What are the causes and risks of the disease?

The exact cause of systemic lupus erythematosus is unknown. It is believed to be an autoimmune disorder. SLE tends to run in families. Certain medications have been known to cause systemic lupus erythematosus. These include procainamide, hydralazine, isoniazid, and chlorpromazine.

Events that may trigger the disease include infection, stress, exposure to toxins, and sunlight. Women account for 80% to 90% of cases of SLE. It is more common in black women than in white women. SLE is also more common in Asian, Hispanic, and Native American women.

What can be done to prevent the disease?

Most cases of systemic lupus erythematosus cannot be prevented. It may be helpful to avoid medications that cause SLE, but not everyone who takes these medications will develop the disease.

How is the disease diagnosed?

A complete medical history and physical examination are important tools for diagnosing SLE. Blood tests also play an important role. A blood test called an ANA looks for antibodies that the person has produced against his or her own tissues.

• CT scan
• chest X-ray
• electrocardiogram
• kidney biopsy
• MRI scan
• spinal tap

What are the long-term effects of the disease?

SLE is a long-term disease that flares up, then quiets down, then flares up again. Persons with SLE often have a significant decrease in the quality of daily life. Many complications of SLE put the person at risk. Many of the medications used to treat the disease also have significant side effects. SLE can be fatal, often as a result of kidney failure, infections, or heart attack.

What are the risks to others?

There is no evidence that SLE is a contagious disease that can be passed from one person to another. However, it does tend to run in families. Pregnant women with SLE have an increased risk of miscarriages, stillbirths, and premature infants.

What are the treatments for the disease?

Although there is no cure for SLE, certain things can help prevent flare-ups. Persons with SLE should avoid intense sun exposure. Liberal use of sunscreens, long-sleeved shirts, and hats are helpful.

Since infections are common, persons with SLE should seek prompt evaluation of fevers. A yearly flu vaccine is recommended. If the spleen is removed, the person should receive the pneumococcal vaccine. Individuals should also receive antibiotics before any dental procedures.

A number of medications are used to treat SLE, including the following:

• antimalarial medications, such as hydroxychloroquine (i.e., Plaquenil) are used to treat skin problems and arthritis.
• Anti-rheumatic drugs such as methotrexate (i.e., Rheumatrex, Trexall) and cyclophosphamide (i.e., Cytoxan)
• corticosteroids, such as prednisone and methylprednisolone. These reduce the immune system response.
• nonsteroidal anti-inflammatory drugs (NSAIDs) such as ibuprofen and naproxen. These medications reduce fever and treat pain.
• powerful cytotoxic medications, which kill cells. These are used to treat nephritis, a serious kidney problem.

Individuals with end-stage kidney disease may benefit from kidney dialysis or a kidney transplant.

What are the side effects of the treatments?

The medications used to treat lupus have significant side effects. Unfortunately, some of these side effects can mimic the symptoms of the disease itself.

NSAIDs can cause stomach irritation, allergic reaction, and decreased kidney function.

Antimalarial medications can cause rash, muscle weakness, and nerve problems.

Corticosteroids have a host of possible side effects. These include weight gain, high blood pressure, osteoporosis, and increased risk of infection.

The cytotoxic medications can cause problems with the bladder and with blood cell production.

What happens after treatment for the disease?

Systemic lupus erythematosus is a chronic disease that fluctuates over time. The best way to manage it is by active collaboration between the individual and his or her healthcare professionals.

How is the disease monitored?

Periodic examinations allow the healthcare professional to monitor the activity of the disease and determine possible complications. A variety of blood tests help in the monitoring of the disease. Any new or worsening symptoms should be reported to the healthcare professional.