WHAT IS LUPUS?
The immune system is designed to attack foreign substances in the body. If you have lupus, something goes wrong with your immune system and it attacks healthy cells and tissues. This can damage many parts of the body such as the:
- Blood vessels
There are many kinds of lupus. The most common type, systemic lupus erythematosus, affects many parts of the body. Other types of lupus are:
- Discoid lupus erythematosus—causes a skin rash that doesn’t go away (DLE)
- Subacute cutaneous lupus erythematosus—causes skin sores on parts of the body exposed to sun (SLE)
- Drug-induced lupus—can be caused by medications
- Neonatal lupus—a rare type of lupus that affects newborns.
WHO GETS LUPUS?
Approximately 1.5 million people in the U.S. have lupus. People of African, Asian, and Native American descent are more likely to develop lupus than are Caucasians. Although it can occur in both men and women, 90% of people diagnosed with the disease are women. Women of childbearing age (14 to 45 years old) are most often affected and as many as 1 in 250 people may develop lupus.
WHAT ARE THE SYMPTOMS OF LUPUS?
The symptoms of lupus differ from one person to another. Some people have just a few symptoms, while others have many. In addition, there are many different symptoms of lupus because the disease can affect any part of the body. Some of the more common symptoms include:
- Achy joints (arthralgia)
- Unexplained fever (more than 100 F)
- Swollen joints (arthritis)
- Prolonged or extreme fatigue
- Skin rash
- Ankle swelling and fluid accumulation
- Pain in the chest when breathing deeply (pleurisy)
- A butterfly-shaped rash across the cheeks and nose
- Hair loss
- Sensitivity to the sun and/or other light
- Mouth or nose sores
- Pale or purple fingers or toes from cold or stress (Raynaud’s Phenomenon)
WHAT PROBLEMS WITH LUPUS CAN PEOPLE HAVE?
Many people with active lupus feel ill in general and complain of fever, weight loss, and fatigue. People with lupus also develop specific problems when the immune system attacks a particular organ or area in the body. The following areas of the body can be affected by lupus:
- Skin . Skin problems are a common feature of lupus. Some people with lupus have a red rash over their cheeks and the bridge of their nose—called a “butterfly” or malar rash. Hair loss and mouth sores are also common. One particular type of lupus that generally affects only the skin is called “discoid lupus.” With this type of lupus, the skin problems consist of large red, circular rashes that may scar. Skin rashes are usually aggravated by sunlight. A common lupus rash called subacute cutaneous lupus erythematosus is often worse after exposure to the sun. This type of rash can affect the arms, legs, and torso. An uncommon but serious form of lupus rash results in the development of large blisters and is called a “bullous” lupus rash.
- Joints. Arthritis is very common in people with lupus. There may be pain, with or without swelling. Stiffness and pain may be particularly evident in the morning. Arthritis may be a problem for only a few days or weeks, or may be a permanent feature of the disease. Fortunately, the arthritis usually is not crippling.
- Kidneys. Kidney involvement in people with lupus can be life threatening and may occur in up to half of those with lupus. Kidney problems are more common when someone also has other lupus symptoms, such as fatigue, arthritis, rash, fever, and weight loss. Less often, kidney disease may occur when there are no other symptoms of lupus.
- Blood. Blood involvement can occur with or without other symptoms. People with lupus may have dangerous reductions in the number of red blood cells, white blood cells, or platelets (particles that help clot the blood).
- Sometimes, changes in blood counts (low red cell count, or anemia), may cause fatigue, serious infections (low white cell count), or easy bruising or bleeding (low platelet count). Many patients do not have symptoms from low blood counts, however, so it is important for people with lupus to have periodic blood tests in order to detect any problems.
- Blood clots are more common in people with lupus. Clots often occur in the legs (called deep venous thrombosis or DVT) and lungs (called pulmonary embolus or PE) and occasionally in the brain (stroke). Blood clots that develop in lupus patients may be associated with the production of antiphospholipid (APL) antibodies. These antibodies are abnormal proteins that may increase the tendency of the blood to clot. Blood can be tested for these antibodies.
- Brain and Spinal Cord. Brain involvement is, fortunately, a rare problem in people with lupus. When present, it may cause confusion, depression, seizures, and, rarely, strokes. Involvement of the spinal cord (transverse myelitis) can cause numbness and weakness.
- Heart and Lungs. Heart and lung involvement often is caused by inflammation of the covering of the heart (pericardium) and lungs (pleura). When these structures become inflamed, patients may develop chest pain, irregular heartbeat, and accumulation of fluid around the lungs (pleuritis or pleurisy) and heart (pericarditis). The heart valves and the lung itself can also be affected by lupus, resulting in shortness of breath.
WHAT CAUSES LUPUS?
No gene or group of genes has been proven to cause lupus. Lupus does, however, appear in certain families, and when one of two identical twins has lupus, there is an increased chance that the other twin will also develop the disease. These findings, as well as others, strongly suggest that genes are involved in the development of lupus. Although lupus can develop in people with no family history of lupus, there are likely to be other autoimmune diseases in some family members. Certain ethnic groups (people of African, Asian, Hispanic/Latino, Native American, Native Hawaiian, or Pacific Island descent) have a greater risk of developing lupus, which may be related to genes they have in common.
While a person’s genes may increase the chance that he or she will develop lupus, it takes some kind of environmental trigger to set off the illness or to bring on a flare. Examples include:
- Ultraviolet rays from the sun
- Ultraviolet rays from fluorescent light bulbs
- Sulfa drugs, which make a person more sensitive to the sun, such as: Bactrim® and Septra® (trimethoprim-sulfamethoxazole); sulfisoxazole (Gantrisin®); tolbutamide (Orinase®); sulfasalazine (Azulfidine®); diuretics
- Sun-sensitizing tetracycline drugs such as minocycline (Minocin®)
- Penicillin or other antibiotic drugs such as: amoxicillin (Amoxil®); ampicillin (Ampicillin Sodium ADD-Vantage®); cloxacillin (Cloxapen®)
- An infection
- A cold or a viral illness
- An injury
- Emotional stress, such as a divorce, illness, death in the family, or other life complications
- Anything that causes stress to the body, such as surgery, physical harm, pregnancy, or giving birth
Although many seemingly unrelated factors can trigger the onset of lupus in a susceptible person, scientists have noted some common features among many people who have lupus, including:
- Exposure to the sun
- An infection
- Being pregnant
- Giving birth
- A drug taken to treat an illness
However, many people cannot remember or identify any specific factor that occurred before they were diagnosed with lupus.
EXPLAINING LUPUS TO OTHERS
Perhaps a good way to start to explain lupus is to make clear what lupus is not.
- Lupus is not contagious. 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 not like or related to HIV (Human Immune Deficiency Virus) or AIDS (Acquired Immune Deficiency Syndrome). In HIV or AIDS, the immune system is under-active; in lupus, the immune system is overactive.
You can then talk about what lupus is.
- Lupus is an autoimmune disease. In some ways, lupus represents a kind of allergic reaction by the body, in which the immune system sees the body’s own healthy tissues and cells as foreign.
- Lupus is a chronic disease. This means that anyone who develops lupus will have lupus for the rest of his or her life. As with other chronic illnesses such as diabetes or asthma, there is no cure for lupus yet. However, there are medications and lifestyle adjustments that help most people live active and productive lives in spite of these illnesses.
- Lupus has many different symptoms, and because of that, affects each person differently. Lupus can cause a mild skin rash or achy joints, or can involve the kidneys, heart, lungs, brain, or other internal organs. What most people do not realize, however, is how much effort it may take you to function day-to-day when you have to cope with extreme fatigue, chronic pain, memory loss, medication side effects, and/or visible skin lesions.
- Lupus is unpredictable because it is a disease of flares (the symptoms worsen and you feel ill) and remissions (the symptoms improve and you feel better). Knowing that lupus is unpredictable may help other people understand your physical and emotional ups and downs as well as the changes that you may have to make to schedules, plans, and commitments.
HOW IS LUPUS TREATED?
Lupus symptoms vary from one person to another. In many cases, the best treatment approach is with a health care team that will tailor treatment to your specific condition.
Today, physicians treat lupus using a wide variety of medicines, ranging in strength from mild to extremely strong. Prescribed medications will usually change during a person’s lifetime with lupus. However, it can take months—sometimes years—before your health care team finds just the right combination of medicines to keep your lupus symptoms under control.
There are many categories of drugs physicians use to treat lupus. However, the U.S. Food and Drug Administration or “FDA” has approved only a few specifically for lupus, which include:
- Corticosteroids, including prednisone, prednisolone, methylprednisolone, and hydrocortisone
- Antimalarials, such as hydroxychloroquine (Plaquenil®) and chloroquine
- The monoclonal antibody belimumab (Benlysta®)
- A rheumatologist, a doctor who specializes in diseases of the joints and muscles, generally treats people with lupus. If lupus has caused damage to a particular organ, other specialists will be consulted. For instance, a dermatologist for cutaneous lupus (skin disease), a cardiologist for heart disease, a nephrologist for kidney disease, a neurologist for brain and nervous system disease, or a gastroenterologist for gastrointestinal tract disease. A woman with lupus who is considering a pregnancy needs an obstetrician who specializes in high-risk pregnancies.
Once you have been diagnosed with lupus, your doctor will develop a treatment plan based on your age, symptoms, general health, and lifestyle. The goals of any treatment plan are to:
- Reduce inflammation caused by lupus
- Suppress your overactive immune system
- Prevent flares, and treat them when they occur
- Control symptoms like joint pain and fatigue
- Minimize damage to organs
People with lupus often require other drugs to treat conditions commonly seen with the disease. Examples include:
- Diuretics for fluid retention
- Antihypertensive drugs for high blood pressure
- Anticonvulsants for seizure disorders
- Antibiotics for infections
- Bone-strengthening drugs for osteoporosis