Lupus (LOO-pus) is a chronic (long-lasting) disease that causes inflammation of connective tissue, the material that holds the various structures of the body in place.
Lupus is one of several diseases that are grouped under the collective term collagen vascular diseases. Collagen vascular diseases are those that affect the connective tissues, materials that hold the various structures of the body in place. Specifically, lupus causes inflammation of connective tissues in the body. The medical name for lupus is lupus erythematosus (er-i-thee-ma-TO-sus).
Lupus is divided into two main types:
A third condition, called lupus vulgaris, is unrelated to lupus erythematosus. It is a rare form of tuberculosis that typically produces nodules, or small lumps, on the skin.
Lupus is a disorder that occurs worldwide and in people of all ages. In the United States, the Lupus Foundation of America estimates that 1.5 to 2 million (and possibly more) Americans have some form of the disease. In the United States, lupus is more prevalent among people of African, Native American, Asian, and Latino ancestry than in people of European descent.
Although members of both sexes can get lupus, more than 9 out of 10 people who have the disease are women. Like Julia, most women show their first signs of the disorder between the ages of 15 and 25.
Lupus is not a contagious disease. One person cannot catch it from another, and nobody needs to avoid being near someone who has lupus.
In 5 to 10 percent of cases, certain prescription drugs, such as those used for irregular heartbeat or high blood pressure, can cause symptoms of SLE. (The symptoms usually go away when the drugs are discontinued.) Medical professionals most often see this condition, which they call drug-induced lupus, in elderly patients.
For the remaining 90 percent of SLE cases and in all instances of DLE, the cause or causes of the disorder remained unclear as of 2015. Although many people who have studied lupus agreed that it is an autoimmune disorder, which is a reaction of the immune system * against one's own body, they still had many questions because the causes of immune disorders were not well understood. Researchers and medical professionals did, however, believe that some factors can play a role in the development of lupus.
One of those factors is heredity. Researchers determined that a person with a close relative who has SLE is slightly more likely to develop the disease than someone who does not. For instance, about 5 percent of children born to a parent with lupus develop the disease themselves. Scientists explored the genetic connection and found genes on two chromosomes that show connections to lupus among some families.
Certain environmental influences may play a part in the onset of lupus. Researchers believe that bacteria, viruses, extreme stress, sunlight, certain antibiotics, or food additives may trigger autoimmune responses in some individuals. Nonetheless, not everyone gets lupus because they caught a particular virus or bacterium, are stressed, took a certain antibiotic, or ate a certain food.
In addition, the fact that women are much more likely to get lupus than men suggests that hormones may also be associated with the disorder.
The signs and symptoms of lupus vary greatly in different individuals, both in the parts of the body involved and in the degree of severity. The symptoms may also come and go and may disappear for weeks or months at a time. Although lupus occurs much more frequently in women than in men, the symptoms in males are no less severe than in females.
The mildest and most common form of lupus, DLE, usually involves only the skin. It produces a rash of thickened, scaly, reddish patches on the face and sometimes other parts of the body. Often the rash spreads in a characteristic butterfly-shaped pattern over the cheeks and bridge of the nose.
After a few weeks or months, when healing has taken place, darkcolored or pale scars may remain. If the condition extends to the scalp, the person may lose some of his or her hair.
Sunlight tends to trigger and worsen the rash of lupus. In DLE, the patches sometimes nearly disappear during the winter months. Most people who have DLE are otherwise in good health. The condition only rarely progresses to SLE.
People with SLE may have the same type of rash as those with DLE, and they may lose hair as well. In SLE, however, the skin lesions may spread and cause damage to the mucous membranes * , such as those in the nose or under the eyelids, and other tissues. Some SLE patients, however, do not get these skin problems.
Arthritis * may be the first symptom in some people with SLE. In fact, SLE is medically classified in the same family of diseases as rheumatoid arthritis; they are both collagen vascular diseases. Rheumatoid arthritis is a disorder that causes painful inflammation in the joints. Other early symptoms of SLE may include weakness, extreme fatigue, fever, sensitivity to sunlight, and loss of weight. In addition, about 40 percent of people who have lupus get Raynaud's disease, which can cause sudden numbness or coldness in the fingers or toes. Although it is less common, some patients with SLE also have another collagen vascular disease, called scleroderma. Doctors sometimes call this combination mixed connective tissue disease, or MCTD. Unlike lupus, scleroderma is characterized by a thickening and, in some cases, a hardening of the skin.
SLE may also affect internal organs, which frequently causes serious disorders. People with SLE commonly have problems with kidney function, and if the kidneys fail, they may experience uremia (yoo-REE-me-a), or the buildup of toxic substances in the blood. Uremia can be fatal. SLE may also have an impact on the nervous system and cause psychological problems, seizures * , or other symptoms. It can also involve the lungs, heart, liver, and blood cells.
Medical professionals often have a difficult time diagnosing lupus, especially if the patient has only a few symptoms, because no single sign or symptom unquestionably means someone has lupus, and no single laboratory test is able to diagnose it either. For these reason, doctors must rely on a combination of a thorough medical history, observations, and tests to make a definite diagnosis of lupus.
To diagnose systemic lupus erythematosus (SLE), doctors may order blood tests to check for certain antibodies that attack the nucleus of cells, and for specific white blood cells, called LE (lupus erythematosus) cells, that destroy other blood cells and are an indication of lupus. They may also perform a skin biopsy * (removal of a small sample) to examine it for antibodies active in lupus.
These tests can help doctors make an early diagnosis, which is important so that treatment can begin as soon as possible.
Depending on the particular needs and symptoms of a lupus patient, a doctor may select from numerous treatment options, including prescribing one or more drugs to counteract pain, inflammation, and related problems.
Nonsteroidal anti-inflam-matory drugs (NSAIDs), such as aspirin, ibuprofen * , and naproxen, are common choices for lessening pain and inflammation in the joints and muscles. Another group of medications, called corticosteroids * , helps reduce inflammation and activity of the immune system. A third group, called antimalarials because they are also used to treat malaria, can help with skin and joint symptoms. A doctor may also recommend ointments and creams that contain corticosteroids and sunscreens to treat skin eruptions.
In the early 2000s, doctors sometimes also prescribed immunosuppressive drugs, such as mycophenolate mofetil (MMF) and azathioprine (AZA), and chemotherapy * drugs, called cyclophosphamide, to treat lupus. These drugs help fight the kidney inflammation associated with lupus.
Some of the drugs prescribed to treat lupus can cause unwanted side effects. For this reason, and because symptoms may change, patients need ongoing medical advice and regular checkups to stay on top of the disease, its symptoms, and possible unwanted drug reactions.
No specific preventive measure, such as vaccination * , was available to help a person avoid getting lupus as of 2015. People who were diagnosed with lupus, however, were advised to take steps to reduce the likelihood of “flares,” or sudden worsening of symptoms. Patients who were sensitive to sunlight could help prevent rashes by avoiding excessive exposure to the sun, using sunscreens, or wearing broad-brimmed hats. Although no lupus vaccination existed, doctors recommended immunizations against other infections.
Lifestyle changes help patients with lupus avoid or control symptoms as well. Regular exercise can prevent some muscle weakness and fatigue. Quitting smoking or resisting excessive alcohol consumption can bring about general health improvements. Joining support groups and talking with family, friends, and physicians can ease the effects of stress.
See also Arthritis • Autoimmune Disorders: Overview • Kidney Disease • Raynaud's Disease • Scleroderma
Office on Women's Health. “Lupus Fact Sheet.” WomensHealth.gov . http://womenshealth.gov/publications/our-publications/fact-sheet/lupus.html (accessed November 17, 2015).
Lupus Foundation of America. 2000 L St. NW, Suite 410, Washington, DC 20036. Telephone: 202-349-1155. Website: http://www.lupus.org (accessed November 17, 2015).
National Institute of Arthritis and Musculoskeletal and Skin Diseases. 1 AMS Cir., Bethesda, MD 20892-3675. Toll-free: 877-226-4267. Website: http://www.niams.nih.gov (accessed November 17, 2015).
* rheumatologist (roo-ma-TOLojist) is a doctor who specializes in disorders involving the connective tissue structures of the body.
* immune system (im-YOON SIS-tem) is the system of the body composed of specialized cells and the substances they produce that helps protect the body against disease-causing germs.
* mucous membranes are the thin layers of tissue found inside the nose, ears, cervix (SER-viks) and uterus, stomach, colon and rectum, on the vocal cords, and in other parts of the body.
* arthritis (ar-THRY-tis) refers to any of several disorders characterized by inflammation of the joints.
* seizures (SEE-zhurs) are sudden bursts of disorganized electrical activity that interrupt the normal functioning of the brain, often leading to uncontrolled movements in the body and sometimes a temporary change in consciousness.
* miscarriage (MIS-kare-ij) is the end of a pregnancy through the death of the embryo or fetus before birth.
* biopsy (BI-op-see) is a test in which a small sample of skin or other body tissue is removed and examined for signs of disease.
* ibuprofen (eye-bew-PRO-fin) is a nonsteroidal anti-inflammatory drug (NSAID) used to reduce fever and relieve pain or inflammation.
* corticosteroids (kor-tih-ko-STIRoyds) are chemical substances made by the adrenal glands that have several functions in the body, including maintaining blood pressure during stress and controlling inflammation. They can also be given to people as medications to treat certain illnesses.
* chemotherapy (KEE-mo-THER-apee) is the treatment of cancer with powerful drugs that kill cancer cells.
* vaccination (vak-sih-NAY-shun), also called immunization, is giving, usually by an injection, a preparation of killed or weakened germs, or a part of a germ or product it produces, to prevent or lessen the severity of the disease caused by that germ.