Lupus erythematosus, or lupus, is a chronic disease in which inflammation can attack and damage any part of the body. Lupus can be treated, but there is no cure. Symptoms vary among people with lupus, but fatigue, facial rash (called malar rash), joint pain and swelling, and fever are among the most common. Some people with lupus develop disability and become unable to work. Eighty to 90 percent of people with lupus live a normal lifespan, but severe cases of lupus can be life-threatening.
What is lupus?
Lupus is an autoimmune disease. In other words, the damage in lupus is caused by the body’s immune system attacking its own tissues, causing inflammation and damage. Lupus can attack any type of tissue or organ. Common targets of lupus include the skin and joints. In severe cases, lupus can attack the kidneys, heart, brain, nerves, and blood vessels.
Most people with lupus experience periods of increased disease activity called flares, during which worsening inflammation causes more intense or new symptoms depending on where the inflammation is happening. Between flares, symptoms may improve or disappear. Triggers for lupus flares differ between people, but common triggers include sunlight or fluorescent light, exhaustion, emotional stress, infection, injury, and medications such as sulfa drugs, antibiotics, and diuretics (water pills).
There are several types of lupus, and lupus symptoms are different for each person. Some types of lupus are limited to the skin, while systemic lupus erythematosus (SLE) attacks other types of tissues. The type of lupus a person is diagnosed with can change over time.
The history of lupus
Writings by the Greek doctor Hippocrates described lupus-like ulcers several hundred years before the common era. The word “lupus” was first applied to a skin disease by Herbernus of Tours in 916 CE. Lupus, which is Latin for “wolf,” may have been chosen because the distinctive malar rash reminded doctors of a wolf bite, or because it resembled the facial markings of a wolf.
In the 19th century, doctors began to adopt modern scientific methods. Discoid lupus erythematosus was clearly described in 1851 and named lupus erythematosus by French dermatologist Pierre Louis Alphée Cazenave. (The Latin word “erythematosus” refers to the redness of lupus skin rash.) In 1872, Hungarian dermatologist Moritz Kaposi first differentiated between discoid (affecting the skin) and systemic (affecting other parts of the body) forms of lupus. Kaposi was the first to describe lupus as a systemic disease, associating it with joint pain, fever, and nervous system involvement that could be life-threatening. Thomas Payne, an English physician, first tried quinine (an antimalarial) as a treatment for lupus in 1894. Payne also theorized that lupus could affect the blood vessels. Over the turn of the century, Canadian physician Sir William Osler wrote one of the first complete papers on lupus. Osler described disease flares and explained how lupus could affect different organs and systems.
Through the 1920s and 30s, researchers improved their knowledge and descriptions of the disease process in lupus. In 1948, Mayo Clinic pathologist Malcolm Hargraves identified the lupus erythematosus (LE) cell, making it easier to diagnose milder cases of systemic lupus. Also at the Mayo Clinic, Philip Hench isolated the hormone cortisol and demonstrated that corticosteroids can effectively treat lupus – winning a Nobel Prize in 1950 for his efforts. In the 1950s, two more biologic markers of lupus were discovered, further improving lupus diagnosis. George Friou of the University of Southern California identified the antinuclear antibody, and researchers Cobum and Moore at Johns Hopkins established that many people with lupus produce a false positive result when a syphilis test is administered. These discoveries led to the identification of anti-double stranded DNA (anti-dsDNA) antibodies, another useful diagnostic test for lupus.
In the second half of the 20th century, researchers developed animal models of lupus that helped define lupus as an autoimmune disease. Investigating versions of lupus in mice and other animals also helped scientists understand genetic links that contribute to the development of lupus.
Since the 1990s, lupus research has focused on bringing more and better treatments to market. In 2010, the Lupus Foundation of America called a meeting to define “disease flare.” The resulting definition makes it possible for doctors to more accurately describe disease and researchers a way to begin studying whether new drugs can prevent or limit flares. In 2011, Benlysta (Belimumab) was approved by the Food and Drug Administration (FDA), making it the first new drug approved to treat lupus in more than 50 years.
How common is lupus?
Approximately 1.5 Americans and 5 million people worldwide have lupus. About 90 percent of people with lupus are women. Women of color are two to three times more likely to develop lupus than women of European descent. Most people with lupus develop their first symptoms between the ages of 15 and 44 – childbearing age for women.
How does lupus progress?
Lupus is a highly individualized disease. In mild cases, medication holds lupus in check, and symptoms do not progress, or they progress very slowly. However, permanent damage can accumulate over time; how the damage manifests depends on which tissues lupus attacks. Some severe cases of lupus progress quickly.
About 60 percent of adults with lupus, and about two-thirds of children with lupus, will develop lupus nephritis (lupus that affects the kidneys). Approximately 10 to 30 percent of people with lupus nephritis eventually progress to kidney failure, necessitating dialysis or transplant. More than half of those with lupus develop a heart abnormality – which may be mild or serious – at some point.
Can you die from lupus?
Lupus complications such as infections, kidney failure, and heart disease contribute to an early death in 10 to 15 percent of people with lupus. However, the majority of people with lupus live a normal average lifespan.
How is lupus diagnosed?
Doctors diagnose lupus with a combination of detailed patient history and laboratory tests. It can take time to receive a definitive diagnosis since lupus symptoms vary widely and can imitate those of many other diseases. Some people receive incorrect diagnoses before finding out they have lupus. Learn more about the lupus diagnosis process.
What are the symptoms of lupus?
Lupus symptoms differ extensively between types of lupus and between people. Many of the most common lupus symptoms involve fatigue, joint pain and swelling, fever, and skin rash. Learn more about lupus symptoms.
How is lupus treated?
Several different classes of medications are used to treat lupus. Nonsteroidal anti-inflammatory drugs (NSAIDs) such as Motrin (Ibuprofen) and Celebrex (Celecoxib) can help control joint pain, swelling, and fever in mild cases. Corticosteroids are effective at relieving acute disease flares. Disease-modifying antirheumatic drugs (DMARDs) are taken long-term to keep disease activity low. DMARDs can include antimalarials such as Plaquenil (Hydroxychloroquine) and immunomodulators such as Imuran (Azathioprine) and Rheumatrex (Methotrexate). Lupus symptoms such as depression and seizures can be managed respectively with antidepressants and antiepileptic drugs. Learn more about lupus treatments.
Are lupus and cancer related?
Lupus is not a type of cancer. Having lupus or another autoimmune disease may increase some people’s risk for developing certain types of cancer, including lymphoma, breast cancer, and endometrial cancer. It is unclear whether lupus or medications taken to treat it are responsible for this increased risk.