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Systemic Lupus Erythematosus

Lupus erythematosus is a chronic inflammatory autoimmune disorder that affects the connective tissues. Lupus erythematosus takes two forms: discoid lupus erythematosus (DLE) and systemic lupus erythematosus (SLE). DLE affects only the skin; SLE affects multiple organs (including the skin) and can be fatal.

SLE strikes women 8 times more often than men (15 times more often during childbearing years). It occurs worldwide but is most prevalent among Asians and blacks.

Causes

The exact cause of SLE remains a mystery, but available evidence points to interrelated immune, environmental, hormonal, and genetic factors. Scientists think that autoimmunity is the primary cause. In autoimmunity, the body produces antibodies. such as antinuclear antibodies (ANAs), against its own cells. The formed antigen-antibody complexes then suppress the body's normal immunity and damage tissues. A significant feature in patients with SLE is their ability to produce antibodies against many different tissue components. such as red blood cells (RBCs), neutrophils, platelets, lymphocytes, and almost any organ or tissue in the body.

Certain predisposing factors can make a person susceptible to SLE. They include stress, streptococcal or viral infections, exposure to sunlight or ultraviolet light, injury, surgery, exhaustion, nervous tension, emotional upsets, immunization, pregnancy, and abnormal estrogen metabolism.

Symptoms

Symptoms of the disease vary widely from person to person. They can be mild or severe, and can come and go over time. The most common symptoms include extreme fatigue, painful or swollen joints, unexplained fever, and skin rashes. A skin rash that appears on the nose and cheeks-commonly called the butterfly rash-may be a symptoms, or the rash could be on the face and ears, upper arms, shoulders, chest and hands.

Diagnostic tests

Lupus is sometimes difficult to diagnose because there is no single set of symptoms. A physical exam and a blood test can detect a group of antibodies found in the blood of almost all people with lupus. Some other lab tests include:

  • A compliment test (C3, C4, CH50, CH100) measures the amount of complementary proteins circulating in the blood.
  • A sedimentation rate (ESR) or C-reactive protein (CRP) may be used to measure inflammation levels.
  • A urine analysis issued to detect kidney problems.
  • Chest X-rays may be taken to detect lung damage.
  • An EKG can detect heart problems.

Treatment

The mainstay of SLE treatment is drug therapy. The patient with mild disease requires little or no medication. Nonsteroidal anti-inflammatory drugs (NSAIDs), including aspirin, usually control arthritis and arthralgia symptoms. Skin lesions need topical medications and protection from exposure to the sun. Recommend that the patient use sunscreening agents with a sun protection factor of at least 15. Topical corticosteroid creams, such as triamcinolone and hydrocortisone, may be prescribed for mild disease.

Fluorinated steroids may control acute or discoid lesions. Refractory skin lesions may respond to intralesional or systemic corticosteroids or antimalarials such as hydroxychloroquine and chloroquine. Because hydroxychloroquine and chloroquine can cause retinal damage, such treatment requires ophthalmologic examination every 6 months. Dapsone helps many patients.

Corticosteroids remain the treatment of choice for systemic symptoms of SLE, for acute generalized exacerbations, and for serious disease-related injury to vital organ systems from pleuritis, pericarditis, nephritis related to SLE, vasculitis, and CNS involvement. With initial prednisone doses (equivalent to 60 mg or more), the patient's condition usually improves noticeably within 48 hours. Then, with symptoms under control, the patient discontinues prednisone use or tapers off slowly. (Note: Increasing serum complement levels and decreasing anti-DNA titers indicate patient response.)

If the patient has glomerulonephritis, she requires treatment with large doses of corticosteroids. If renal failure occurs despite treatment, dialysis or kidney transplantation may be necessary.

In some patients, cytotoxic drugs, such as azathioprine, chlorambucil (Leukeran), cyclophosphamide, and methotrexate, can delay or prevent renal deterioration. Antihypertensive drugs and dietary changes can also be effective. Additionally, warfarin is indicated for antiphospholipid antibodies, which can cause clotting in vascular structures.

Joint replacement may be indicated if chronic synovitis and pain are problematic.

Prevention

There are no known ways to avoid developing SLE. However, it is possible for a patient who has been diagnosed with SLE to prevent flares of the disease. Recommendations for improving general health to avoid flares include decreasing sun exposure, getting sufficient sleep, eating a healthy diet, decreasing stress, and exercising regularly.

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