Solving the Riddle of Lupus

People with systemic lupus erythematosus (SLE) face a brighter future thanks to growing knowledge about this chronic disease that drives the body's autoimmune system haywire.

In terms of research and treatment, "more will be done on lupus in the next 10 years than has been done in the previous five decades," says Sandra C. Raymond, president of the Lupus Foundation of America (LFA).

Jane E. Salmon, M.D., a New York rheumatologist who has studied SLE since 1981, says the contrast between our awareness then and now is like night and day.

"We still don't have answers, but we have many, many potential targets now to see whether or not we can correct the disease," says Dr. Salmon. "Now we at least have places to look to intervene. We also have a much better understanding of who is genetically at risk for the disease."

Immune overdrive

Unlike AIDS, which weakens the immune system, lupus kicks it into overdrive. The result? The body targets healthy tissues and cells as invaders and tries to destroy them.

Lupus can attack almost every organ system, Raymond says. Patients with SLE can end up with kidney, heart, and blood disorders or organ failures of all kinds.

Yet much is not known about the disease. There are no definitive warning signs, no sure-fire test, and no cure.

No one is even sure how many people have lupus, says Chad Helmick, M.D., an epidemiologist for the CDC. The LFA estimates that 1.5 million Americans have the disease, but some estimates range up to 4 million. Congress recently approved funding for a lupus registry to help pin down the number.

Experts do know that nine out of 10 people with lupus are women. African Americans and Hispanics are hit hardest.

From 1979 to 1998, the latest statistics available, the CDC reports 22,860 deaths from SLE. African American women ages 45 to 64 had the highest death rate with the biggest rise: 70 percent in 20 years. Why isn't clear, Dr. Helmick says.

The CDC also says:

• More than a third of SLE deaths took place among people 15 to 44 years old. Death rates rose with age.

• Death rates were more than five times higher for women than for men and more than three times higher for blacks than for whites.

Difficult diagnosis

Lupus is hard to diagnose because it can mimic other diseases or lead to other problems. It's often described as a type of arthritis. The disease, which is not contagious, can be mild to severe. With the right treatment, many patients lead long, healthy lives. That includes the ability to have children, adds Dr. Salmon.

Lupus takes several forms. Symptoms vary and often come and go, making lupus difficult to diagnose. In general, it can cause extreme joint and muscle pain, prolonged and extreme exhaustion, fevers, skin rashes, and organ failure. Patients also are more likely to have heart and kidney problems.

Studies show genetics plays a role in causing lupus coupled with environmental triggers, says Emily Baechler Gillespie, Ph.D., an autoimmune disease specialists and lead author of a study from the University of Minnesota and other groups that links genetic signatures and lupus symptoms. The triggers may include exposure to ultraviolet light or certain viral infections, she says. The study may help lead to more targeted therapies.

Treatments to ease inflammation and other symptoms vary with the patient and with lupus' effects. Medication can range from ibuprofen to corticosteroids and even to chemotherapy. But in this time of rapid growth in lupus knowledge, Dr. Salmon adds, trials of new therapies are under way.

Common lupus symptoms

The LFA lists the most common symptoms and the percentage of patients who have them. See your doctor if you have any of them.

 

Lupus symptoms	Symptom frequency
Achy joints	95%
Frequent fevers over 100 degrees	90%
Arthritis (swollen joints)	90%
Prolonged or extreme fatigue	81%
Skin rashes	74%
Anemia	71%
Kidney trouble	50%
Pain in the chest on deep breathing	45%
Butterfly-shaped rash across cheek, nose	42%
Sun or light sensitivity	30%
Hair loss	27%
Abnormal blood clotting	20%
Raynaud's phenomenon (fingers turn white or blue when cold)	17%
Seizures	15%
Mouth or nose ulcers	12%