If you or a family member has been diagnosed with or are being evaluated for possible lupus, you will want to know as much as you can about the disease. “Understanding Lupus” will provide you with a guide to lupus so that you can better understand what lupus is.
Lupus is a chronic, autoimmune disease that can damage any part of the body (skin, joints, and/or organs inside the body). Chronic means that the signs and symptoms tend to last longer than six weeks and often for many years. In lupus, something goes wrong with your immune system, which is the part of the body that fights off viruses, bacteria, and germs (“foreign invaders,” like the flu). Normally our immune system produces proteins called antibodies that protect the body from these invaders. Autoimmune means your immune system cannot tell the difference between these foreign invaders and your body’s healthy tissues (“auto” means “self”) and creates autoantibodies that attack and destroy healthy tissue. These autoantibodies cause inflammation, pain, and damage in various parts of the body.
- Lupus is also a disease of flares (the symptoms worsen and you feel ill) and remissions (the symptoms improve and you feel better). Lupus can range from mild to life-threatening and should always be treated by a doctor. With good medical care, most people with lupus can lead a full life.
- Lupus is not contagious, not even through sexual contact. You cannot “catch” lupus from someone or “give” lupus to someone.
- Lupus is not like or related to cancer. Cancer is a condition of malignant, abnormal tissues that grow rapidly and spread into surrounding tissues. Lupus is an autoimmune disease, as described above.
- Lupus is not like or related to HIV (Human Immune Deficiency Virus) or AIDS (Acquired Immune Deficiency Syndrome). In HIV or AIDS the immune system is underactive; in lupus, the immune system is overactive.
- Our research estimates that at least 1.5 million Americans have lupus. The actual number may be higher; however, there have been no large-scale studies to show the actual number of people in theU.S.living with lupus.
- It is believed that 5 million people throughout the world have a form of lupus.
- Lupus strikes mostly women of childbearing age (15-44). However, men, children, and teenagers develop lupus, too.
- Women of color are 2-3 times more likely to develop lupus.
- People of all races and ethnic groups can develop lupus.
- More than 16,000 new cases of lupus are reported annually across the country.
What Causes Lupus? No one knows what causes lupus. Scientists think that people are born with the genes to develop lupus and that something brings on or “triggers” the disease and symptoms. However, a combination of genetics (heredity), environment, and hormones is involved.
Systemic Lupus Erythematosus
Systemic lupus is the most common form of lupus, and is what most people mean when they refer to “lupus.” Systemic lupus can be mild or severe. Some of the more serious complications involving major organ systems are:
- inflammation of the kidneys (lupus nephritis), which can affect the body’s ability to filter waste from the blood and can be so damaging that dialysis or kidney transplant may be needed
- an increase in blood pressure in the lungs (pulmonary hypertension)
- inflammation of the nervous system and brain, which can cause memory problems, confusion, headaches, and strokes
- inflammation in the brain’s blood vessels, which can cause high fevers, seizures, behavioral changes,
- hardening of the arteries (coronary artery disease), which is a buildup of deposits on coronary artery walls that can lead to a heart attack
More information about how lupus affects various organs and tissues is available in the LFA fact sheet series, “The Body & Lupus.”
Cutaneous Lupus Erythematosus
Cutaneous refers to the skin, and this form of lupus is limited to the skin. Although there are many types of rashes and lesions (sores) caused by cutaneous lupus, the most common rash is raised, scaly and red, but not itchy. It is commonly known as a discoid rash, because the areas of rash are shaped like disks, or circles. Another common example of cutaneous lupus is a rash over the cheeks and across the bridge of the nose, known as the butterfly rash. Other rashes or sores may appear on the face, neck, or scalp (areas of the skin that are exposed to sunlight or fluorescent light), or in the mouth, nose, or vagina. Hair loss and changes in the pigment, or color, of the skin are also symptoms of cutaneous lupus.
Approximately 10 percent of people who have cutaneous lupus will develop systemic lupus. However, it is likely that these people already had systemic lupus, with the skin rash as their main symptom.
Drug-induced Lupus Erythematosus
Drug-induced lupus is a lupus-like disease caused by certain prescription drugs. The symptoms of drug-induced lupus are similar to those of systemic lupus, but only rarely will any major organs be affected.
The drugs most commonly connected with drug-induced lupus are hydralazine (used to treat high blood pressure or hypertension), procainamide (used to treat irregular heart rhythms), and isoniazid (used to treat tuberculosis). Drug-induced lupus is more common in men because they are given these drugs more often; however, not everyone who takes these drugs will develop drug-induced lupus. The lupus-like symptoms usually disappear within six months after these medications are stopped.
Neonatal lupus is a rare condition that affects infants of women who have lupus and is caused by antibodies from the mother acting upon the infant in the womb. At birth, the infant may have a skin rash, liver problems, or low blood cell counts, but these symptoms disappear completely after several months with no lasting effects. Some infants with neonatal lupus can also have a serious heart defect. With proper testing, physicians can now identify most at-risk mothers, and the infant can be treated at or before birth. Most infants of mothers with lupus are entirely healthy.
Symptoms of lupus vary widely depending on the individual case and the form of lupus present. Most people with lupus do not experience all of these symptoms. The section only serves to alert people to clues that might indicate the presence of lupus in an undiagnosed person.
Because lupus can affect so many different organs, a wide range of symptoms can occur. These symptoms may come and go, and different symptoms may appear at different times during the course of the disease.
The most common symptoms of lupus, which are the same for females and males, are:
- extreme fatigue (tiredness)
- painful or swollen joints
- anemia (low numbers of red blood cells or hemoglobin, or low total blood volume)
- swelling (edema) in feet, legs, hands, and/or around eyes
- pain in chest on deep breathing (pleurisy)
- butterfly-shaped rash across cheeks and nose
- sun- or light-sensitivity (photosensitivity)
- hair loss
- abnormal blood clotting
- fingers turning white and/or blue when cold (Raynaud’s phenomenon)
- mouth or nose ulcers
Many of these symptoms occur in other illnesses besides lupus. In fact, lupus is sometimes called “the great imitator” because its symptoms are often like the symptoms of rheumatoid arthritis, blood disorders, fibromyalgia, diabetes, thyroid problems, Lyme disease, and a number of heart, lung, muscle, and bone diseases.
The form of lupus and its symptoms determine what type of doctor you will see. Most people who have mild to moderate disease will be treated by a rheumatologist, who specializes in the diseases of joints and muscles.
However, if your lupus causes kidney problems, you will also see a nephrologist, a specialist in diseases of the renal system.
If you have rashes or lesions, you will see a dermatologist, who specializes in diseases that affect the skin (including the scalp and the mouth).
Because lupus can cause damage to any part of the body, other specialists may be necessary, such as a cardiologist, who specializes in heart problems, or a neurologist, who specializes in problems that affect the brain and nervous system, or a perinatologist, who specializes in high-risk pregnancies.
More than 90 percent of people with lupus are women.
Symptoms and diagnosis occur most often when women are in their childbearing years, between the ages of 15 and 44. Symptoms of lupus will occur before age 18 in 15 percent of the people who are later diagnosed with the disease.
In theUnited States, lupus is more common in people of color — African Americans, Hispanics/Latinos, Asian Americans, Native Americans, Native Hawaiians and Pacific Islanders — than in the Caucasian population. It also appears that lupus develops at an earlier age and is more severe among members of these ethnic groups.
Relatives of people with lupus have an approximately 5-13 percent chance of developing lupus. However, only about 5 percent of children will develop lupus if their mother has lupus.
Frequently Asked Questions
Can I have my children tested? Testing isn’t advisable in individuals who do not have symptoms.
The idea that lupus is generally a fatal disease is a big misconception. In fact, the prognosis of lupus is much better today than ever before.
It is true that medical science has not yet developed a method for curing lupus. And some people do die from the disease. However, people with non-organ threatening aspects of lupus can look forward to a normal lifespan if they:
- follow the instructions of their physician,
- take their medication(s) as prescribed, and
- know when to seek help for unexpected side effects of a medication or a new manifestation of their lupus.
Although some people with lupus have severe recurrent attacks and are frequently hospitalized, most people with lupus rarely require hospitalization. There are many lupus patients who never have to be hospitalized, especially if they are careful and follow their physician’s instructions.
New research brings unexpected findings each year. The progress made in treatment and diagnosis during the last decade has been greater than that made over the past 100 years. It is therefore a sensible idea to maintain control of a disease that tomorrow may be curable.
Frequently Asked Questions
Is there a cure for lupus? At the present time there is not a cure for lupus, but there certainly is effective treatment.
Is lupus a fatal disease? Lupus is not a universally fatal disease. In fact, today with close follow-up and treatment, 80-90% of the people with lupus can expect to live a normal life span. Lupus does vary in intensity and degree, however, and there are people who have a mild case, there are those who have a moderate case and there are some who have a severe case of lupus, which tends to be more difficult to treat and bring under control. For people who have a severe flare-up, there is a greater chance that their lupus may be life-threatening. We know that some people do die of this disease and because of that we have a tremendous amount of respect for the potential of this disease. However, the majority of people living with lupus today can expect to live a normal lifespan. People frequently read in the literature that, 80-90% of people with lupus live for more than 10 years. Unfortunately, this is often misinterpreted as people with lupus live for only 10 years.
Let us clarify this.
It is important to understand that the “10 years” does not represent the number of years the person will live, but rather the number of years involved in the study. The studies followed patients with lupus from the time of diagnosis for a period of ten years. At the end of this research period they were able to conclude that 80-90% of the people enrolled were still alive. What this study did not look at is what happened in year 11, 12, 15, 20 and so on. We know there are many people who have been living with lupus for 15, 19, 25, 30 and 40 years. This is not a disease that is universally fatal to all. The majority of people with lupus today can expect to live a normal lifespan.
When people die of lupus, what do they usually die of? Overwhelming infection and kidney failure are the two most common causes of death in people with lupus. Recently there is new information which indicates heart disease may be another leading cause of death among people with lupus.
Check out our new interactive “Could you have lupus?” symptom checklist! This interactive tool was designed to help you better understand the many ways that lupus can affect your body. Each highlighted section features a text box which provides a brief description of how lupus can damage a particular organ or system, from the brain to the blood vessels. http://www.lupus.org/newsite/pages/lupusChecklist.aspx