Lupus, an autoimmune disease, is caused by vascular inflammation (vasculitis) that results in significant damage to a number of different body systems and organs, including the joints, skin, kidneys, heart, lungs, and brain. For this reason it is sometimes called a multisystem disease. Its symptoms vary from patient to patient, ranging from mild conditions that can be managed by medications to life-threatening emergencies.
There are four major types of lupus:
- Systemic lupus erythematosus or SLE. This is the form of the disease most commonly meant by lupus. SLE can occur in childhood but is most common in people between the ages of fifteen and forty-five.
- Discoid lupus. Discoid lupus is a skin disorder in which the patient develops thick raised patches of scaly reddened skin on the face or scalp. A small percentage of patients with discoid lupus later develop SLE.
- Drug-induced lupus. This is a form of lupus triggered by medications. It goes away when the patient stops taking the drugs. Drug-induced lupus is more common in men than in women.
- Neonatal lupus. This is a rare form of lupus that sometimes occurs in babies born to mothers with SLE, Sjögren syndrome, or no disease at all.
The causes of lupus, a complex disease, are not understood. It is difficult to diagnose because its symptoms are easy to confuse with those of many other disorders and because there is no symptom profile that applies to all patients with lupus. What is known is that lupus is a chronic inflammatory disease in which the body'simmune system turns against its own tissues, producing what are called autoantibodies. Autoantibodies are protein molecules that target the person's own cells, tissues, or organs, causing inflammation and tissue damage. In lupus, the autoantibodies damage the blood vessels in such vital organs as the kidneys.
The inflammation of body tissues in lupus leads to a variety of symptoms that may come and go over time as well as vary from patient to patient. These include aches and pains in joints and muscles, skin rashes, sensitivity to sunlight, unexplained fever, swollen glands, extreme fatigue, hair loss, mouth ulcers, chest pains, easily bruised tissues, and emotional disorders. Periods when the symptoms are absent or low-key are called remissions, and periods when the symptoms return or increase in severity are called flares.
Lupus is primarily a disease of women of childbearing age. It is rarely diagnosed in children except for the neonatal form. In the United States, lupus affects about one person in every 2,000. According to the Lupus Foundation of America, between 1.5 and 2 million people in the United States may have a form of lupus. The actual number may be higher because the diagnosis is often missed by doctors. Around the world, the rate of lupus varies from country to country, from twelve cases per
100,000 people in Great Britain to thirty-nine per 100,000 in Sweden. In New Zealand, there are fifty cases per 100,000 population among Polynesians, compared with only 14.6 cases per 100,000 among white New Zealanders.
In the United States, lupus is three times more common among African Americans than among Caucasians. It is also more common among Hispanics, Asian Americans, and Native Americans. Like many other autoimmune diseases, lupus strikes women nine times more frequently than men. Among males with lupus, older men are more likely to get the disease than younger men. The fact that women of childbearing age are the group most likely to develop lupus is the reason why some researchers think that female sex hormones may be involved in the disease.
Researchers believe that lupus is the end result of a combination of genetic, hormonal, and environmental factors. At least ten different genes have been identified that increase a person's risk of developing lupus, and the disease is known to run in families. There is no single lupus gene, however. Other factors that are being studied as possible triggers of lupus include sunlight, stress, certain drugs, and viruses.
Men and Lupus
Like osteoporosis, breast cancer, and rheumatoid arthritis, lupus is a disease that is largely considered a problem for women. But the 10 percent of lupus patients who are male have some difficulties that female patients do not, precisely because of the gender ratio. According to the Lupus Foundation of America, men with lupus are often worried about being seen as less masculine because of the diagnosis, even though they can still be sexually active and have children. In addition to its feminine image, lupus can cause emotional distress for men by limiting their ability to earn a living or to do chores around the house requiring physical labor. Loss of independence, coupled with such physical changes as hair loss and weight gain, can be a heavy blow to a man's self-esteem.
Researchers are also looking into whether lupus in older men is more severe than in women in the same age groups. Some studies have suggested that men with SLE have a higher risk than women of severe damage to the blood vessels, nerves, and kidneys. Currently male patients with lupus are given the same therapies as women.
The symptoms of lupus may appear in almost any body system:
- Skin: Butterfly-shaped facial rash (also called a malar rash); rash elsewhere on body; ulcers in the mouth, nose, or vagina; loss of hair on head; sensitivity to sun exposure. About 90 percent of patients with lupus have symptoms affecting the skin and hair.
- Bones and muscles: Arthritis, muscle cramps, pains in the hands and wrists.
- Blood: Anemia, low white blood cell count, problems with normal blood clotting, Raynaud's phenomenon (loss of blood flow to fingers and toes due to stress or cold exposure).
- Heart and lungs: Inflammation of the lining of the heart (pericariditis) and lungs (pleuritis). About 50 percent of patients with lupus develop some form of lung disease.
- Nervous system: Seizures, psychotic episodes, memory loss, depression, anxiety. These affect about 15 percent of patients with lupus.
- Kidneys and liver: Kidney disease and eventual kidney failure. About 50 percent of patients with lupus have kidney problems.
- Other: Unexplained fever, weight loss or gain, fatigue.
There is no single test that can provide a definitive diagnosis of lupus. The disease is not easy to diagnose because it usually develops slowly over a period of years, its symptoms often come and go, and none of them are unique to lupus. The American College of Rheumatology compiled a list of eleven criteria in 1982 to help distinguish lupus from other diseases. Seven of these are symptoms:
- Butterfly (malar) rash on face
- The raised red patches of discoid lupus
- Skin rash triggered or worsened by sun exposure
- Ulcers in the nose or mouth
- Inflammation of the tissues lining the inside of the lungs or heart
- Seizures or convulsions in the absence of other causes for these events
The other four criteria are test results:
- Abnormally high levels of protein and red or white blood cell fragments in the urine
- Abnormally low red or white blood cell counts
- Presence of antinuclear antibodies (ANA) and anti-double strand DNA in the blood
- Other positive blood tests that indicate an autoimmune disorder
A person should meet four or more of these criteria for the doctor to suspect lupus. The symptoms do not all have to occur at the same time.
There is no cure for lupus. The symptoms of the disease are managed by medications tailored to the location and the severity of the individual patient's symptoms. The patient's doctor may prescribe drugs from any of the following groups:
- Nonsteroidal anti-inflammatory drugs (NSAIDs). These drugs include aspirin and such non-aspirin pain relievers as Aleve and Motrin. They can be used to treat joint pain, bring down fever, and other inflammatory symptoms of mild lupus.
- Antimalarial drugs. There is no known connection between lupus and malaria; however, some drugs used to treat malaria appear to prevent lupus flares as well as treat symptoms.
- Corticosteroids. This group of drugs includes prednisone, one of the drugs prescribed most frequently to treat lupus. Corticosteroids act to bring down inflammation rapidly. Unfortunately, they also have serious side effects, including weight gain resulting from increased appetite, weakened bones, high blood pressure, damage to the arteries, and an increased risk of infections and diabetes. Doctors try to minimize these side effects by prescribing the lowest dose necessary to control symptoms for the shortest possible time.
- Immunosuppressants. These are drugs that work by reducing the overactivity of the immune system that is involved in lupus. Immunosuppressants are generally given only to patients with severe flares that are damaging organ function, or in order to reduce a patient's dose of corticosteroids.
The prognosis for lupus is variable depending on the severity of the symptoms. In general, patients whose kidneys or central nervous systems are affected by the disease have a worse prognosis. Men with lupus have a slightly worse prognosis than women.
The overall life expectancy for patients with lupus has improved since the 1950s, when less than 50 percent of patients were still alive five years after diagnosis. For patients diagnosed with SLE in the United States, Canada, and Europe, 95 percent are alive at five years after diagnosis, 90 percent at ten years, and 78 percent at twenty years. The overall death rate for patients diagnosed with lupus is three times that of the general American population.
There is no way to prevent lupus because the causes of the disease have not been clearly identified.
Researchers are focusing on new treatments for lupus as well as genetic studies of ethnic groups and families at increased risk of the disease. The Lupus Foundation of America has links to registries for individuals and families with lupus and lupus-related conditions at http://www.lupus.org/webmodules/webarticlesnet/templates/new_aboutfaq.aspx?articleid=384…3x0026;zoncid=19. The National Insitute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS) is presently conducting studies of lupus in African Americans and Native Americans to look for possible genetic factors associated with the high rates of lupus in these groups.
SEE ALSO Chronic fatigue syndrome; Fibromyalgia; Sjögren syndrome
WORDS TO KNOW
Flare: A period of worsened symptoms in lupus.
Malar rash: The medical term for the butterfly-shaped facial rash found in lupus.
Neonatal: The medical term for newborn.
Raynaud's phenomenon: Discoloration of the fingers and toes caused by blood vessels going into spasm and decreasing the flow of blood to the affected digits.
Remission: A period of decreased or absent lupus symptoms.
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"Unknown: Lupus." UXL Encyclopedia of Diseases and Disorders. . Encyclopedia.com. (September 19, 2018). http://www.encyclopedia.com/medicine/encyclopedias-almanacs-transcripts-and-maps/unknown-lupus
"Unknown: Lupus." UXL Encyclopedia of Diseases and Disorders. . Retrieved September 19, 2018 from Encyclopedia.com: http://www.encyclopedia.com/medicine/encyclopedias-almanacs-transcripts-and-maps/unknown-lupus