History of Lupus

From its discovery to the present day, scientists have learned a great deal about the complexities of this disease. Although references to this condition have been noted even in medieval times, perhaps the first comprehensive description of the disease was advanced by Pierre Cazenave in the mid 1800s. The name “lupus” is Latin for wolf, because patients often get a "wolf-bite like" malar rash on their face. (Some people call this a "butterfly rash".) In the 1950s, antinuclear antibodies associated with lupus were discovered. Antinuclear antibodies are triggers that cause an autoimmune attack of self tissues by the immune system. Studies in murine (mouse) models over the past 40 years have helped us to understand lupus better.

   Some common questions about lupus:

What is lupus?

A person's immune system is responsible for defending the body against bacteria, viruses, and other disease-causing germs. The cells of the immune system are what normally prevent us from getting sick. When a person has lupus, though, the good disease-fighting cells have begun to attack their own body cells instead of the germs. This is called an autoimmune response (or attack). Autoimmune responses damage healthy organs like the skin, joints, blood, kidneys, brain, spinal cord, liver and lungs. Lupus affects people differently; some people may have damage to many of their organs while others may only have damage to one or two. Sometimes there is only a little damage, but at other times it can be very serious, making the person very sick. Because lupus is such a complex disease, the diagnosis, treatment, and monitoring of this condition are sometimes difficult.

What causes lupus?

Researchers are still trying to find the exact cause of lupus. The disease is probably caused by a combination of genetic and environmental factors. Scientists are working hard to identify the genetic risk factors for lupus both in humans and in animal models.

We know that lupus is caused by abnormalities in the immune system in which the body's disease-fighting cells recognize healthy body cells in an aberrant fashion. The disease-fighting cells then make thousands of copies of themselves in order to attack the body cells. To a scientist, this means that there is a break in tolerance to self antigens, leading to hyperactivity of self-reactive immune cells. We also know that in lupus, there are problems with excessive inflammation in the damaged organs. Normally, inflammation is a healthy process that helps our bodies fight infection and heal faster. However, in the target organs such as skin and kidneys, excessive inflammation can cause dysfunctioning of these organs, leading to the clinical signs of the disease.

Unfortunately, we don't yet know what specific combinations of genetic, hormonal, and environmental factors (like infection, UV light, and diet) are most likely to cause lupus. This is the target of ongoing research in many laboratories around the world, including ours at UT Southwestern Medical Center in Dallas, Texas, USA.

How common is lupus?

Over 1 million Americans have been diagnosed with lupus; and 16,000 new patients are diagnosed every year. This disease is present in every ethnic group, although certain populations like African Americans and Hispanic Americans are more affected. Lupus is about ten times more common in women. It afflicts 1 out of 100 women, and about 1 out of 60 African American & Hispanic women. Lupus is more common than AIDS, sickle cell anemia, muscular dystrophy, cerebral palsy, multiple sclerosis, and cystic fibrosis combined; in fact, 20,000 Americans die from lupus each year. Lupus ranges from mild to life-threatening, in terms of its clinical presentation. In most people with lupus, the disease can be controlled with proper treatment.

Lupus affects all ethnic groups albeit to different extents. Listed below is a brief survey of the prevalence rates of lupus in different parts of the world.

Region/Population	Prevalence of Lupus
China	40-70 per 100,000
Polynesians	50 per 100,000
USA	15-50 per 100,000
Iceland, Sweden	36 per 100,000
Malays	30 per 100,000
United Kingdom	28 per 100,000
New Zealand Whites	15 per 100,000
India	3-10 per 100,000

How is lupus diagnosed?

There is no simple test for diagnosing lupus. The most widely recognized list of unique symptoms and characteristics for systemic lupus erythematosus was revised in 1997 by the American College of Rheumatology (ACR). Although this classification criteria is not the only way to diagnose lupus, these criteria are often used to “classify” or "diagnose" patients as having systemic lupus erythematosus.


To see the 1982 ACR classification criteria for lupus:
http://www.rheumatology.org/research/classification/sle.html

To see a description of the 1997 classification criteria revision:
http://www.rheumatology.org/research/classification/1982SLEupdate.html


It is important however that the diagnosis of lupus is made by a trained physician. For more information, please see Patient Care - Patient Information.

How is lupus treated?

Because lupus affects different organs in different people, its treatment depends on the symptoms and severity of the disease in the patient. Here are the names of some drugs used to treat lupus: corticosteroids (like prednisone), antimalarials (like hydroxychloroquine), immune modulating medications (like mycophenolate mofetil or methotrexate), and cytotoxic medicines (like cyclophosphamide). The goal of lupus research today is to find better medicines and treatments for this disease.

It is important however that the condition is treated by a trained physician. For more information, please see Patient Care - Patient Information.