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What is lupus?

Systemic Lupus Erythematosus (also known as lupus or SLE) is a chronic disease that can affect any part of the body. Lupus is an autoimmune disorder, which means your body's immune system attacks healthy cells and tissue. In lupus the overactive immune system produces antibodies that attack various tissue and organs, including the skin, joints, kidneys, heart, and brain. Some people experience only mild rashes and arthritis. Others suffer debilitating fever, fatigue, joint pain, or severe organ and/or life-threatening disease.

Lupus is named for the Latin word for wolf. The doctor who first described lupus noted a distinctive rash across the face that looked like the bite of a wolf.

Ninety percent of lupus patients are women of child-bearing age. The disease is also more common in Hispanics, African-Americans, and Asians than in Caucasians.

Lupus can be hard to diagnose. Almost all people with lupus have joint pain and develop arthritis. Frequently affected joints are the fingers, hands, wrists, and knees. Rarely, lupus complications can be fatal.

What causes lupus?

The simple answer is we don't know. Researchers suspect a complicated relationship between an individual's inherited genes, hormones, and environment that works against a patient over time to cause lupus. The genetic link is not completely understood, but a person's risk for developing lupus is 20 times higher than the general population if they have a brother or sister with the disease. Women are nine times more likely than men to develop lupus, leading researchers to believe there is a hormonal component to developing the disease as well. Finally, researchers are also looking into the association between exposure to certain environmental toxins such as cigarette smoke, silica, and mercury to better understand the link between exposure and lupus.

What are the symptoms of lupus?

Lupus is different for each patient; no two lupus patients experience the same pattern of symptoms. Even the severity of lupus symptoms can vary; some people experience mild, moderate, or severe symptoms that can be temporary, while others experience chronic symptoms. Symptoms depend on where in your body inflammation is occurring.

The most common symptoms include:

  • Fatigue
  • Joint pain, stiffness, and swelling
  • Muscle pain
  • Skin rashes across the cheeks and nose or elsewhere on the body
  • Sun sensitivity
  • Chest pain, shortness of breath
  • Swelling in legs
  • Fever
  • Hair loss (alopecia)

Make an appointment with your doctor if you develop an unexplained rash; have an ongoing fever, or a persistent ache or unexplained fatigue.

How is lupus diagnosed?

There is no one single diagnostic test for lupus; your rheumatologist will evaluate your symptoms, examine your body, check blood and urine tests, and eventually decide if you have lupus. Lupus is diagnosed when four clinical and laboratory features are present:

  • Malar rash
  • Discoid rash
  • Photosensitivity (development of a rash after sun exposure)
  • Oral or nasal ulcers
  • Arthritis of multiple joints
  • Serositis (inflammation of the lining around the lungs or heart)
  • Neurological disorders such as seizures and psychosis
  • Kidney disease indicated by protein or casts in the urine
  • Blood disorders such as hemolytic anemia, leukopenia, and lymphopenia
  • Positive ANA test: ANA stands for "anti-nuclear antibody," which means that your immune system is producing an antibody against the nucleus of your cells. A positive ANA test by itself does not necessarily mean you have lupus. Many healthy people have a positive ANA. That is why the combination of your laboratory tests, your symptoms, and your physical exam is important and will help your rheumatologist decide whether your not you have lupus.
  • Other autoantibodies: Antibodies normally are protective. Lupus antibodies damage the healthy tissues and organs.

Your rheumatologist will likely look at your fingers and toes for discoloration, which is indicative of circulation problems.

How is lupus treated?

While there is no cure for lupus, many treatments help to manage symptoms and reduce complications. They are:

  • Corticosteroids (such as Prednisone)
  • Hydroxychloroquine (also known as Plaquenil)
  • Immunosuppressants: These medications can suppress the immune system. Azathioprine (Imuran) and CellCept (Mycophenolate Mofetil) are both organ transplant medications that can control lupus. Chemotherapy can also be used as an immunosuppressive medication. Methotrexate is used for arthritis and rashes. Cyclosphosphamide (Cytoxan) is used for severe organ or life-threatening lupus.
  • Biological agents, such as Rituximab (Rituxan) and Belimumab (Benlysta), which is a specific inhibitor of soluble B-lymphocyte stimulator, a blood factor that worsens lupus symptoms.

Patients with lupus have normal fertility and can have children. If you are considering pregnancy or trying to get pregnant, your rheumatologist may suggest delaying pregnancy until your disease has been under control for at least six months. Delaying pregnancy can help to reduce the risk of lupus-related pregnancy complications. Frequent monitoring and visits with a rheumatologist are important.

Complications of Lupus

Long-term lupus complications include lupus- or medication-related organ and tissue damage. Complications can affect different organs in the body.

  • Pregnancy complications: Lupus increases the risk of pregnancy-related complications such as preeclampsia, preterm birth, and miscarriage.
  • Infection: Lupus and its treatments can weaken the immune system, making people more susceptible to many infections.
  • Early atherosclerosis and heart disease: People with lupus are more likely to have heart attacks and strokes than those without lupus. Your doctor should check for risk factors and treat high blood pressure, cholesterol, and other risk factors early and aggressively.
  • Kidney damage: Without proper treatment, lupus can cause kidney damage leading in some people to kidney failure, the leading cause of death in people with lupus.
  • Osteoporosis: Some lupus medications can ause bone thinning. This should be prevented and treated before fractures and damage to the bones occur.