What is Lupus?
Lupus, also known as systemic lupus erythematosus (or SLE), is a chronic, autoimmune disorder that causes persistent inflammation that can affect organs throughout the body, including the skin and musculoskeletal, cardiovascular, renal, and neurological systems.
The precise cause of lupus is unknown. What is known is that the disease is characterized by antibodies formed by the immune system that attack the body’s own cells. Inflammation typically occurs in the joints, and inflammation of the skin may cause a characteristic butterfly-shaped rash on the face, as well as an increased sensitivity to the sun. Severe cases of lupus may lead to kidney or heart failure and can be fatal. However, lupus is often mild; many people never experience the most severe symptoms of the disease and, with treatment, are able to pursue a wide range of normal activities.
Who Gets Lupus?
Women are affected eight to 10 times more frequently than men. An increased incidence of lupus is found among those of Asian or African descent and members of some Native American tribes. Lupus usually develops between the ages of 15 and 35, although it may occur at any age.
Symptoms vary widely depending upon which organ systems are involved, and they occur in a series of flare-ups or episodes separated by periods of normal or near-normal health. The most common symptoms include:
- General feeling of poor health.
- Loss of appetite and weight loss.
- Abdominal pain, nausea, and vomiting.
- Joint pain and swelling.
- A butterfly-shaped rash over both cheeks and the bridge of the nose, as well as rashes elsewhere on the body.
- Increased skin sensitivity to sun exposure.
- Small, painless ulcers in the mucous membranes of the nose and mouth.
- Blurred or worsening vision.
- Unusual bruising or bleeding.
- Jaundice; dark urine or decreased urine.
- Palpitations or irregular heartbeat.
- Enlarged glands or lymph nodes.
- Swelling of the abdomen, the tissues around the eyes, and the ankles; weight gain; shortness of breath
- Cough and chest pain.
- A tingling sensation or pain in the muscles, stiffness, spasms, seizures, numbness, or temporary paralysis.
- Depression, anxiety, mental confusion.
The cause of lupus is not yet known. However, certain exposures may have a connection to lupus and may trigger a flare up. Possible risk factors include:
- Family history.
- Hormones: lupus symptoms may worsen during pregnancy, the menstrual cycle or during other hormonal fluctuations.
- Ultraviolet radiation.
- Childbirth, abortion or stress.
- Certain drugs have been associated with inducing lupus symptoms, including hydralazine, an antihypertensive; procainamide, a drug used to treat heart rhythm disturbances; and chlorpromazine, which is a tranquilizer. Although rare, drug-induced lupus has a genetic risk factor, causes general symptoms such as fever, malaise, joint pain (arthralgia) and inflammation (polyarthritis), and inflammation of the membranes surrounding the lungs (pleurisy) and heart (pericarditis).
- Certain foods, such as alfalfa sprouts, may cause a temporary lupus-like illness in some
people; the illness reportedly disappears when the medication or food is discontinued.
What If You Do Nothing?
Treatment may not be necessary for mild symptoms. However, it is important to get evaluated by a doctor to rule out the possibility of symptoms being caused by other disorders. If symptoms grow more severe, or if new symptoms appear, treatment is necessary to control disease activity and to avoid some of the potentially serious complications of the disease.
There is no single, specific test for lupus. Instead, doctors use clinical observations and a variety of tests to reach a diagnosis. The American College of Rheumatology has established criteria for the classification of lupus. Lupus is diagnosed if more than 4 of the following are present:
- Malar rash (butterfly-shaped rash across the cheeks and nose)
- Discoid lupus rash (scarring, round rashes found most commonly on the face and scalp)
- Photosensitivity (rash on sun exposed areas of the body due to exposure to sunlight)
- Mouth or nose ulcers (usually painless)
- Nonerosive arthritis(does not destroy the bones surrounding the joints), which occurs in 2 or more joints and causes tenderness, swelling, or effusion (increased amounts of fluid)
- Cardiopulmonary involvement (e.g., inflammation of the lining around the heart [pericarditis] and/or the lungs [pleurisy])
- Neurological disorder (e.g., seizures, psychosis with delirium and/or hallucinations)
- Kidney (renal) disorder (e.g., excessive protein in the urine [proteinuria], cellular casts in the urine)
- Blood disorder (e.g., hemolytic anemia, low white blood cell count, low platelet count)
- Immunologic disorder (e.g., antibodies to double stranded DNA, antibodies to Sm, antibodies to cardiolipin, and lupus anticoagulant)
- Antinuclear antibodies (ANA; positive test in absence of drugs known to induce a positive test)
- Although there is no specific test that is used to diagnose lupus, blood tests to detect autoimmune antibodies are often used. More specifically, most patients with lupus test positive for anti-nuclear antibody (ANA), though the test is not definitive for lupus.
- Urine tests for excessive protein and red blood cells can also be used to detect abnormalities that may be linked to lupus.
- A kidney biopsy may be performed.
There is no cure for lupus, and the goals of treatment are to reduce the severity of episodes, to reduce inflammation in the body, to prevent damage to organs of the body, and to maintain function. Lifestyle changes and medication can both assist in managing symptoms.
Because the course of the disease varies from patient to patient, there are a number of different treatment plans. In many cases, preventive treatment can reduce the frequency and severity of symptom flare-ups.
- Wear sunscreen and avoid excessive sun exposure. Ultraviolet (UV) rays of the sun that causes lupus to become more active. Although the sun is the major source of UV rays, UV light also is produced by many other sources, including lights commonly found in homes and work places. Therefore, it is recommended that patients who have lupus wear sunscreen every day, even if they don't go outside, and even in the winter. On sunny days, patients should apply sunscreen two to three times a day.
- Dietary restrictions have an impact. Avoid eating bean sprouts or garlic and don’t take Echinacea supplements. These substances can cause an increase in lupus activity by stimulating the immune system.
- Do not smoke. Patients with lupus have an increased risk for heart attack at a younger age that people who do not have the condition, and smoking prevents Plaquenil, the most commonly prescribed drug for treating lupus, from working.
- Have regular medical checkups.
- Limit alcohol intake.
Drugs. The types of medications used to treat lupus depend on which organs are affected and on the severity of symptoms. In some cases, women with lupus are unable to tolerate oral contraceptives or estrogen replacement therapy. Patients should check with their rheumatologist to see if it is safe to take hormones or not. Sulfonamide, which is present in some antibiotics, also has been shown to cause flares in patients with lupus. Patients who have lupus should always list sulfa antibiotics, such as Septra and Bactrim, as medicines to which they are allergic. Medications that are commonly used include the following:
- Non-steroidal anti-inflammatory drugs (NSAIDs) include aspirin, ibuprofen, such as Advil or Motrin, naproxen or Naprosyn, indomethacin known as Indocin. NSAIDsare used to treat muscle and joint pain and inflammation. These drugs may cause gastrointestinal side effects can include stomach pain and gastrointestinal bleeding, and should be taken with meals. Antacids and other medications, such as Cytotec and Prilosec may further reduce the risk for these side effects. Some NSAIDs, such as Arthrotec and Celebrex, are less likely to cause ulcers. Kidney function and blood pressure must be monitored regularly while taking these medications.
- Acetaminophen (Tylenol).
- Corticosteroid, such as prednisone, reduce inflammation and suppress the immune system. Side effects include weight gain, high blood pressure, risk for infection, osteoporosis, cataracts, and diabetes. The risks are higher when the medication is taken for a long period of time or at high doses.
- Antimalarials such as chloroquine (Aralen) or hydroxychloroquine (Plaquenil) may be prescribed for skin and joint symptoms of lupus. These medications rarely cause side effects, but they may affect the eyes. Patients who are taking antimalarials should have their eyes examined regularly (usually once or twice yearly). Plaquenil is the most commonly prescribed antimalarial medications, and fortunately, it is very rare to develop eye problems from it.
- Immunomodulating drugs include azathioprine, known as Imuran, and cyclophosphamide, known as Neosar). Specifically intravenous immunoglobulin injections block production of specific antibodies, may be given to lupus patients on a regular basis to increase platelets, the blood cells that aid in coagulation.
- In 2011, the U.S. Food and Drug Administration (FDA) approved belimumab (Benlysta) to be used with standard lupus treatment. This drug, which is a monoclonal antibody, is the first new treatment for lupusin over 50 years. Belimumab is administered intravenously to help reduce levels of abnormal B cells in patients with lupus and lessen the severity of symptoms. Side effects include diarrhea, nausea, and fever. A study involving treatment with belimumab in African Americans with lupus is ongoing because these patients did not experience the expected results in earlier studies.
- If renal failureoccurs, hemodialysisis the preferred treatment over peritoneal dialysis. Patients who experience kidney failure can be considered for renal transplant because the risk for development of the disease in the transplanted kidney is low.
- Severe cases of inflammation in and around the lungs and heartmay require surgical drainage, especially if fluid surrounding the heart decreases its ability to pump blood called cardiac tamponade.
There is no known way to prevent lupus.
When To Call A Doctor
Make an appointment with a doctor if you develop symptoms of lupus. See your doctor if, after being diagnosed with lupus, symptoms worsen or new symptoms develop.
Reviewed by Diane M. Horowitz, M.D., Rheumatologist, North Shore Long Island Jewish Health System, Great Neck, NY. Review provided by VeriMed Healthcare Network.