When it comes to lupus, one size doesn’t fit all. Because lupus is a complex disease whose course can vary so much from person to person, it is important to work with knowledgeable physicians and other health professionals. Here is where you can learn some of what you need to know to stay as healthy as possible and live well despite lupus.
What is lupus?
A disease that “attacks” your immune system. Your immune system is your body’s natural defense against disease. When you have lupus, your immune system has stopped working properly, and antibodies that normally protect you against infection are misdirected and react against substances in your cells or tissues. This may cause inflammation, injury and pain much like a splinter in your toe causes it to become swollen, tender and red. In lupus, the inflammation occurs inside your body to vital organs and systems such as your skin, joints, kidneys, brain, heart, lungs and blood. The targets vary from patient to patient, but most people with the disease have symptoms in only a few parts of the body.
What are the different types of lupus?
Systemic lupus can affect almost any part of your body. The most common type of lupus is systemic lupus erythematosus (S.L.E.). It can cause inflammation almost anywhere in your body. You may only have symptoms involving your skin and joints, or you may have inflammation of your joints, lungs, kidneys, blood, organs and/or other tissues. You may have periods of remission with few or no symptoms, and you may also experience “flares” when the disease gets more active.
Cutaneous lupus affects your skin. There are many different types of lupus skin rashes. Sometimes patients can have a rash without any other manifestations of lupus. One of the common types of skin lupus is called discoid lupus erythematosus (D.L.E.) and is identified by a rash that may appear on your face, neck or scalp, and by hair loss. It is not lifethreatening. The rash may be more noticeable when your skin is exposed to sunlight or fluorescent light. About 1 in 10 people with discoid lupus eventually develop systemic lupus, and about 3 in 10 people with systemic lupus have discoid lupus rashes.
Drug-induced lupus can develop with certain medicines. In a very small number of people, certain prescription medicines lead to the development of S.L.E. symptoms. These medicines include hydralazine (for high blood pressure), procainamide (for irregular heart rhythms), and certain anticonvulsants (for seizures). Sometimes the symptoms appear even after taking the medicines for months or more. The lupus symptoms usually disappear some time after the medicines are stopped.
What causes lupus?
Lupus is a mystery. Researchers think genetic, environmental and possibly hormonal factors combine in some way to cause the condition. You can’t “catch” lupus from someone else, and it can’t be sexually transmitted. While lupus can run in families, it most likely takes external factors to trigger the illness, such as overexposure to ultraviolet rays or the use of certain drugs. Infection, trauma, stress, surgery and hormones may also trigger lupus.
Who is most likely to get lupus?
Females are at greater risk. Lupus may occur in males or females and can happen at any age, but it is young women between the ages of 15 and 44 who are most likely to first develop symptoms of the disease. Some researchers think that female sex hormones— particularly estrogen—affect the immune system in a way that makes females more susceptible.
Lupus in the U.S. Approximately 1.5 million Americans have lupus.
- Nine in 10 people with lupus are female.
- Lupus is more prevalent in women who areAfrican American, Hispanic/Latina, Asian, or Native American.
- Lupus is a leading cause of kidney disease,stroke, and heart disease in women of childbearing age.
What are the most common symptoms?
Most people with lupus have symptoms in only a few organs. If you have not already been diagnosed, the following table may alert you to the possibility of lupus. If you have already been diagnosed, these signs and symptoms may indicate increased activity of the disease, known as a “flare.” You may also have periods of “remission” in which few or no symptoms are present. Some people with lupus experience serious, life-threatening problems. But in most cases, lupus affects only a few organs and can be controlled with medicines and lifestyle changes.
Lupus Signs and Symptoms
- Achy joints (arthralgias)
- Fever over 100 degrees F
- Swollen and painful joints (arthritis)
- Prolonged fatigue
- Skin rashes
- Swollen ankles (kidney involvement)
- Chest pain upon deep breathing (pleurisy)
- Butterfly-shaped rash across cheeks and nose
- Sensitivity to sun (photosensitivity)
- Unusual hair loss
- Problems with abnormal blood clotting
- Pale or purple fingers from cold or stress
- Mouth ulcers (often painless, at roof of mouth)
How is lupus diagnosed?
No single lab test can determine if you have lupus. Many symptoms of lupus are very similar to those of other diseases, and tend to come and go. Your primary care doctor or rheumatologist will use your medical history, a physical exam, and many routine as well as special tests to determine if you have lupus.
Four or more of the following “Eleven Criteria of Lupus” established by the American College of Rheumatology are usually present before a diagnosis of lupus is made.
The “Eleven Criteria of Lupus”
1. Malar rash – butterfly-shaped rash across cheeks and nose
2. Discoid (skin) rash – raised red patches
3. Photosensitivity – skin rash as a result of unusual reaction to sunlight or sometimes a flare of other lupus symptoms after sun exposure
4. Mouth or nose ulcers – usually painless
5. Nonerosive Arthritis – (bones around joints do not get destroyed) in 2 or more joints with tenderness, swelling or effusion
6. Cardio-pulmonary involvement - inflammation of the lining around the heart (pericarditis) and/or lungs (pleuritis)
7. Neurologic disorder - seizures and/or psychosis
8. Renal (kidney) disorder - excessive protein in the urine, or cellular casts in the urine
9. Hematologic (blood) disorder - hemolytic anemia, low white blood cell count, or low platelet count
10. Immunologic disorder - antibodies to double stranded DNA, antibodies to Sm, or antibodies to cardiolipin
11. Antinuclear antibodies (ANA) – positive test in absence of drugs known to induce it.
Does a positive Antinuclear Antibody (ANA) test signal lupus?
No, it doesn’t. Your immune system is your body’s natural defense against disease. A “positive” ANA blood test indicates that your immune system is making an antibody (a protein) that reacts with components of cells in your body—a condition called ”autoimmunity” that may or may not be harmful. As many as 2 out of every 10 people have a positive ANA test. In addition to lupus and other autoimmune conditions, a positive ANA test can indicate abnormalities such as thyroid disease and certain liver illnesses.
What is the treatment for lupus?
While there is no cure, there are treatments. Quick diagnosis and appropriate treatment can help keep the symptoms of the disease under control and lessen the chance of permanent damage to organs or tissues. Once a diagnosis is made, it’s very important that major organs (the central nervous system, kidneys, heart, lungs) are regularly monitored for problems. Treatment depends on the activity and extent of the disease—no two cases of lupus are alike. Researchers are actively looking for new medicines to add to the benefits of those already available or to replace ones that currently exist but which cause unpleasant side effects and reactions in many people.
Medicines used for lupus include:
- NSAIDS – nonsteroidal anti-inflammatory drugs to relieve achy joints and arthritis in mild S.L.E. when pain is limited and organs are not affected.
- Antimalarial drugs – such as hydroxychloroquine, often prescribed for arthritis or skin problems.
- Corticosteroids - usually prednisone, used for major organ involvement. The dosage prescribed depends on which organs are involved, the severity of symptoms, and blood test results.
- Other immunosuppressive agents – such as azathioprine, methotrexate, cyclophosphamide, cyclosporine, and mycophenolate mofetil. These very potent drugs help control the overactive but misdirected immune system in lupus patients. They help limit damage to major organs and are closely monitored to counter the potentially serious side effects and complications.
Lifestyle changes that may help with lupus:
- During a “flare” – get plenty of rest.
- When in remission – exercise to increase joint flexibility and muscle strength.
- Avoid sunlight – since the sun can trigger a flare, always apply sunblock to your face and other exposed skin (365 days a year) and regularly wear hats and other protective clothing.
- Make de-stressing a priority – support groups, professional counseling, and talking with friends, family and physicians can help a lot. Exercise when you can. Learn relaxation techniques like deep breathing. Find what works for you.
- For fever over 100 degrees F – call your doctor.
- Get regular checkups – these usually include blood and urine tests.
- Ask questions – when in doubt, call your doctor.
- Tell your doctor right away about side effects or new symptoms – help her know when a change in therapy might be needed.
What is the outlook for people with lupus?
There is no cure for lupus, but researchers are making promising new findings each year, and there is growing hope that much more targeted and less toxic treatments will be developed soon. Improved methods for detecting, diagnosing, treating and monitoring lupus have enabled patients and doctors to more effectively manage and live with this chronic disease. Just 25 years ago, only 40 percent of people with lupus were expected to live more than 3 years following the diagnosis. Today most people with lupus can look forward to a normal lifespan.