University of Iowa Health Science Relations and
Richard D. Sontheimer, MD
Professor of Dermatology
First Published: November 2000
Last Revised: September 2003
Peer Review Status: Internally Peer Reviewed

According to the Lupus Foundation of America, an estimated 16,000 Americans develop erythematosus ("lupus") every year and 500,000 to 1.5 million Americans have been diagnosed with the disease.

Lupus is a chronic inflammatory disease that can affect various parts of the body, especially skin, joints, blood, and kidneys. Although lupus can affect any part of the body, most people with the disease experience only a few symptoms. Some of the most common are achy joints, skin rashes, fatigue, loss of hair, sores in the mouth, inflammation of the lining of the lungs, and decreased kidney function.

The causes of the disease are relatively unknown, but environment and genetic factors are involved. Richard Sontheimer, MD, professor and head of dermatology at the University of Iowa, said that although the disease is mainly hereditary, environmental factors may induce the disease.

"Sunlight exposure can really bring out lupus in someone who hasn't been yet diagnosed with the disease," Sontheimer said. "UV (ultraviolet) rays really aggravate lupus, especially for those people in which the disease affects the skin."

According to Dr. Sontheimer: Lupus is an illness that can cause problems in the body in many different ways. However, there are several general patterns of illness into which most lupus patients fall. In one pattern, patients experience only lupus skin disease ("cutaneous lupus"). An example would be patients who have only discoid LE skin lesions.

In another pattern, patients experience problems from the way that lupus can attack one or more of the vital organs inside the body. Examples would include lupus kidney disease and lupus brain disease. This pattern of lupus is called systemic lupus or SLE (systemic lupus erythematosus) for short.

A third pattern is where lupus patients experience problems both in their skin and in their internal vital organs at the same time. Such patients can be referred to as having systemic lupus with lupus skin lesions. Examples would include systemic lupus patients who also have discoid LE skin lesions or other types of lupus skin lesions such as subacute cutaneous lupus erythematosus or acute cutaneous lupus erythematosus (butterfly rash).

In some patients, lupus skin disease or lupus internal organ disease can be triggered by reactions to certain drugs. Hydrochlorothiazide and diltiazem (for high blood pressure) as well as terbinafine (for skin fungus infections), can trigger the appearance of certain types of lupus skin disease such as subacute cutaneous lupus erythematosus. Other drugs such as hydralazine (for high blood pressure) and procainamide (for heart rhythm irregularities) can trigger the appearance of some symptoms of internal lupus (fever, loss of energy, joint pain, chest pain). In such "durg-induced" lupus patients, simply stopping the offending drug can result in complete resolution of the lupus skin and internal symptoms without further treatment.

It is estimated that there is one lupus patient for every 3,000 U.S. citizens. Each year, approximately one in 20,000 U.S. citizens will be newly diagnosed with lupus. Lupus is often called a women's disease, affecting females eight times more frequently than males.

The disease can occur at any age and seems to target African-American females living in the United States most often. "In fact, approximately one in 7,500 African-Americans will develop the disease each year in the U.S.," Sontheimer said.

Treatment and medications often depend on the organs involved and the severity of the symptoms. "Treatment depends on the type of lupus, the stage of the disease and the individual patient," Sontheimer said. "Treatment really needs to be individualized."

Treatment approaches are based on the specific needs and symptoms of each person. For most people diagnosed with lupus, effective treatment only minimizes symptoms, reduces inflammation, and maintains normal body functions.

"Treatments for lupus are directed at trying to settle down the overactive immune system that is seen in lupus patients," Sontheimer said. "The most common form of treatment today for severe cases continues to be systemic immuno-suppresive drugs, such as corticosteroids (cortisone), used in high doses."

Other classes of drugs, such as antimalarials, can also be used to minimize the amount of corticosteroids a lupus patient must take (there can be many side effects associated with taking high doses of corticosteroids internally for prolonged periods of time). Sontheimer also said that people at risk for lupus, such as those who have a family member with the disease, should try to stay out of the sun or use sunscreen whenever possible.

Preventive measures have been focused on the environmental effects that trigger lupus.

"We try to prevent or minimize the onset of symptoms of lupus by telling people to decrease exposure to sunlight and artificial forms of ultraviolet light, use sunscreens, use corticosteroid creams for skin rashes and practice stress management," Sontheimer said. "Psychological and physical stress can aggravate both internal and skin forms of lupus."

Although there is no cure, lupus is generally not a fatal disease. In fact, the prognosis for the disease is better than ever. Ninety percent of people with lupus live 10 years after their diagnosis.