Lupus (SLE)

Systemic lupus erythematosus (SLE), commonly known as "lupus," is an autoimmune illness. The immune system, which normally protects the body from foreign invaders and infection, malfunctions and instead attacks a person's own healthy body tissues. Its cause is unknown, but most scientists believe that genetics, combined with outside triggers (such as infections, medications or other environmental factors) lead people to develop lupus. Lupus is a lifelong condition, but symptoms tend to cycle in alternate periods of "flares" (or "flares-ups") and remissions. Lupus affects women much more than men. There is no known cure, but numerous treatments are available.

If your primary care doctor suspects you may have lupus, contact a rheumatologist to confirm the diagnosis and get appropriate treatment. HSS is top ranked for rheumatology by U.S.News & World Report.

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Types of Lupus

There are several different forms of lupus

Systemic lupus erythematosus (SLE) is the most common.
Discoid lupus is marked by a scarring rash of circular-shaped lesions.
Subacute cutaneous lupus involves a more widespread rash that is often worsened by sun exposure.

Drug-induced lupus is relatively rare and is triggered by certain drugs, such as hydralazine.
Neonatal lupus occurs in infants whose mothers have specific blood abnormalities.

Lupus Symptoms

Symptoms vary from person to person, but the typical lupus patient is a young woman experiencing fever, swollen lymph nodes (glands), butterfly-shaped rash on her face, arthritis of the fingers, wrists or other small joints, hair loss, chest pain and protein in the urine. Symptoms usually begin in only one or two areas of the body, but more may develop over time. The most common signs and symptoms of lupus are:

Arthritis.
Malaise, fatigue, fevers, weight loss.
Rashes (often in response to sunlight exposure), usually located on the face, neck, chest, backs of the upper arms, fingertips and/or base of the fingernails.
Hair loss.
Swollen lymph nodes.
Kidney (renal) disease.
Blood (hematologic) disorders, such as hemolytic anemia, leukopenia, lymphopenia or thromobocytopenia.

Chest pains due to pleuritis or pericarditis.
Abdominal pain.
Headache, mood disorder, confusion, psychosis, seizure or stroke.
Immune system disorders – antibodies against your own cells, including anti-dsDNA, anti-Sm or antiphospholipid antibodies (anticardiolipin, lupus anticoagulant), or antinuclear antibodies (ANA)
Photosensitivity – in which sun exposure makes you ill (beyond just a rash), requiring the use of a high-grade sunscreen.
Small, painless ulcers in the mouth and nose.

In addition to discussing these and other symptoms with you, your doctor will order a variety of tests to confirm a diagnosis of lupus. These may include blood, urine and biochemical tests. People with lupus often have false-positive test results for syphilis.

Complications of Lupus

The severity of lupus varies from mild to life threatening. Kidney problems and neurologic complications are more dangerous than the rashes, arthritis or other symptoms. After many years of having lupus, patients may develop hypertension (high blood pressure), accelerated atherosclerosis (plaque and fat build-up in the arteries), heart and lung diseases, kidney failure or osteoporosis. With proper treatment, the majority of people diagnosed with lupus have a normal life expectancy, but many will experience disabilities. Each patient will likely have his or her own specific pattern of symptoms and flares, but the disease can change over time.

Blood Clots

Lupus patients who are positive for antiphospholipid antibody (aPL) can develop blood clots and valvular heart disease. These patients require additional medications, such aspirin, warfarin (Coumadin), heparin, or low-molecular-weight heparin (Lovenox or Fragmin) to reduce the risk of clots.

Lupus and Pregnancy

Many women with lupus ask "it safe for me to get pregnant?" or "can I have a healthy pregnancy?" Pregnancy is possible in most patients with lupus, but complications are frequent. Anyone with lupus should be considered to have a high risk for health complications during pregnancy. When a woman with lupus becomes pregnant or is planning to become pregnant, she should get a referral for appropriate, specialized care. Lupus patients who are positive for aPL are at high risk of miscarriage, while patients with anti-Ro/SSA and anti-La/SSB antibodies are at risk for delivering a child with neonatal lupus.

Kidney Disease

People with lupus are at great risk of contracting kidney disease. Kidney failure occurs in a minority of patients with lupus nephritis, despite advances in therapy. These patients must undergo dialysis. About one-third of patients who start dialysis during an acute lupus flare will be able to discontinue it within the first year. The remaining two-thirds, and those suffering gradual deterioration of kidney function over several years will require either continual dialysis for life or a kidney transplant.

Lupus Treatments

Depending on the symptoms, blood test results and the particular organs involved, a person with lupus may receive one or more of the following:

Nonsteroidal anti-inflammatory drugs (NSAIDs)
Corticosteroids
Immunosuppressive drugs such as azathioprine (Imuran), methotrexate (Rheumatrex), cyclosporine (Sandimmune, Neoral), and mycophenolate mofetil (CellCept), cyclophosphamide (Cytoxan), some of which also may be given by injection or intravenously.
Experimental approaches such as intravenous immunoglobulin and vitamins/antioxidants.