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Systemic lupus erythematosus (SLE) is an autoimmune disease. In this disease, the immune system of the body mistakenly attacks healthy tissue. It can affect the skin, joints, kidneys, brain, and other organs.
Overview of Systemic Lupus Erythematosus (Lupus)
Systemic lupus erythematosus (lupus) is a disease that can damage many parts of the body, such as the joints, skin, kidneys, heart, lungs, blood vessels, and brain. You can’t catch lupus from another person.
If you have lupus you will have periods of illness (flares) and periods of wellness (remission).
What happens in lupus?
Lupus occurs when the immune system, which normally helps protect the body from infection and disease, attacks different parts of the body.
Who Gets Systemic Lupus Erythematosus (Lupus)?
We know that many more women than men have systemic lupus erythematosus (lupus). Lupus is more common in African American women than in white women and is also more common in women of Hispanic, Asian, and Native American descent. African American and Hispanic women are also more likely to have active disease and serious organ system involvement. In addition, lupus can run in families, but the risk that a child or a brother or sister of a patient will also have lupus is still quite low.
Although lupus usually first affects people between the ages of 15 and 45 years, it can occur in childhood or later in life as well.
Symptoms of Systemic Lupus Erythematosus (Lupus)
Each person with systemic lupus erythematosus (lupus) has slightly different symptoms that can range from mild to severe. You may have symptoms in only one or in many parts of your body. Symptoms may also come and go over time.
Some of the most common symptoms of lupus include:
- Painful or swollen joints (arthritis).
- Unexplained fever.
- Extreme fatigue.
- Red rashes, most often on the face.
- Chest pain upon deep breathing.
- Hair loss.
- Sensitivity to the sun.
- Mouth sores.
- Pale or purple fingers and toes from cold and stress.
- Swollen glands.
- Swelling in the legs or around the eyes.
Other symptoms could include:
- Anemia (a decrease in red blood cells).
- Kidney inflammation, which typically requires drug treatment to prevent permanent damage.
- Headaches, dizziness, depression, confusion, or seizures if the disease affects the central nervous system.
- Inflammation of the blood vessels.
- Decreased number of white blood cells or platelets.
- Increased risk of blood clots.
- Inflammation of the heart or the lining that surrounds it.
- Heart valve damage.
The cause of SLE is not clearly known. It may be linked to the following factors:
- Certain medicines
SLE is more common in women than men. It may occur at any age. However, it appears most often in people between the ages of 15 and 44. The disease affects African Americans and Asians more than people from other races.
Symptoms vary from person to person, and may come and go. Everyone with SLE has joint pain and swelling at some time. Some develop arthritis. SLE often affects the joints of the fingers, hands, wrists, and knees.
Other common symptoms include:
- Chest pain when taking a deep breath.
- Fever with no other cause.
- General discomfort, uneasiness, or ill feeling (malaise).
- Hair loss.
- Weight loss.
- Mouth sores.
- Sensitivity to sunlight.
- Skin rash: A "butterfly" rash develops in about half the people with SLE. The rash is mostly seen over the cheeks and bridge of the nose. It can be widespread. It gets worse in sunlight.
- Swollen lymph nodes.
Other symptoms depend on which part of the body is affected:
- Brain and nervous system: Headaches, numbness, tingling, seizures, vision problems, and personality changes
- Digestive tract: Abdominal pain, nausea, and vomiting
- Heart: Valve problems, inflammation of heart muscle
- Lung: Buildup of fluid in the pleural space, difficulty breathing
- Skin: Patchy skin color and fingers that change color when cold (Raynaud phenomenon)
- Kidney: Swelling in the legs
Some people have only skin symptoms. This is called discoid lupus.
Causes of Systemic Lupus Erythematosus (Lupus)
No one completely understands what causes systemic lupus erythematosus (lupus). Studies suggest that a number of different genes may determine your risk for developing the disease.
Some environmental factors also appear to play a role in lupus. In particular, scientists are studying the effects of sunlight, stress, hormones, cigarette smoke, certain drugs, and viruses.
Diagnosis of Systemic Lupus Erythematosus (Lupus)
Diagnosing systemic lupus erythematosus (lupus) can be difficult and may take months or even years. Although there is no single test for lupus, your doctor may do the following to diagnosis you with the condition:
- Ask you about your medical history.
- Give you a physical exam.
- Take samples of blood, skin, kidney, or urine for laboratory tests. The most useful tests look for certain antibodies in the blood.
Exams and Tests
To be diagnosed with lupus, you must have 4 out of 11 common signs of the disease. Nearly all people with lupus have a positive test for antinuclear antibody (ANA). However, having a positive ANA alone does not mean you have lupus.
The health care provider will do a complete physical exam. You may have a rash, arthritis, or edema in the ankles. There may be an abnormal sound called a heart friction rub or pleural friction rub. Your provider will also do a nervous system exam.
Tests used to diagnose SLE may include:
Tests used to diagnose SLE may include:
- Antinuclear antibody (ANA)
- CBC with differential
- Chest x-ray
- Serum creatinine
You may also have other tests to learn more about your condition. Some of these are:
- Antinuclear antibody (ANA) panel
- Complement components (C3 and C4)
- Antibodies to double-stranded DNA
- Coombs test -- direct
- ESR and CRP
- Kidney function blood tests
- Liver function blood tests
- Rheumatoid factor
- Antiphospholipid antibodies and lupus anticoagulant test
- Kidney biopsy Imaging tests of the heart, brain, lungs, joints, muscles or intestines Treatment
Treatment of Systemic Lupus Erythematosus (Lupus)
Treatments for systemic lupus erythematosus (lupus) have improved dramatically in recent decades, giving doctors more choices in how to manage the disease. Because some treatments may cause harmful side effects, you should immediately report any new symptoms to your doctor. You should also talk to your doctor before stopping or changing treatments.
Treatments for lupus include:
- Nonsteroidal anti-inflammatory drugs (NSAIDs) are used to treat joint or chest pain or fever. Ibuprofen and naproxen sodium are available over the counter, whereas other NSAIDS are available by prescription only.
- Antimalarials prevent and treat malaria, but doctors have found that they also are useful for treating fatigue, joint pain, skin rashes, and inflammation of the lungs caused by lupus. These drugs may also prevent flares from recurring.
- Corticosteroids, strong inflammation-fighting drugs, may be taken by mouth, in creams applied to the skin, by injection, or by intravenous (IV) infusion (dripping the drug into the vein through a small tube). Because they are potent drugs, your doctor will seek the lowest dose required to achieve the desired benefit.
- Immunosuppressives restrain an overactive immune system and may be prescribed if your kidneys or central nervous systems are affected by lupus. These drugs may be given by mouth or by IV infusion. The risk for side effects increases with the length of treatment.
- B-lymphocyte stimulator (BlyS)-specific inhibitors reduce the number of abnormal B cells thought to be a problem in lupus.
- Alternative and complementary therapies may improve symptoms, although research has not shown whether they help treat the disease. Examples include:
- Special diets.
- Nutritional supplements.
- Fish oils.
- Ointments and creams.
- Chiropractic treatment.
In many cases you may need to take medications to treat problems related to lupus, such as high cholesterol, high blood pressure, or infection.
Who Treats Systemic Lupus Erythematosus (Lupus)?
Most people will see a rheumatologist for their systemic lupus erythematosus (lupus) treatment. A rheumatologist is a doctor who specializes in rheumatic diseases (arthritis and other inflammatory disorders, often involving the immune system). Clinical immunologists (doctors specializing in immune system disorders) may also treat people with lupus. As treatment progresses, other professionals often help, including:
- Primary care doctors, such as a family physician or internal medicine specialist, who coordinates care between the different health providers and treats other problems as they arise.
- Mental health professionals, who help people cope with difficulties in the home and workplace that may result from their medical conditions.
- Nephrologists, who treat kidney disease.
- Cardiologists, who specialize in the heart and blood vessels.
- Hematologists, who specialize in blood disorders.
- Endocrinologists, who treat problems related to the glands and hormones.
- Dermatologists, who treat skin problems.
Living With Systemic Lupus Erythematosus (Lupus)
Dealing with a long-lasting disease like systemic lupus erythematosus (lupus) can be hard on the emotions. You might think that your friends, family, and coworkers do not understand how you feel. Sadness and anger are common reactions.
Besides working with your doctor to determine a treatment plan, there are a few things you can do to help you live with lupus:
- Learn to recognize the warning signs of a flare so that you and your doctor might reduce or prevent them. These warning signs include:
- Increased tiredness.
- Stomach discomfort.
- Eat a proper diet, exercise, and learn relaxation techniques to help cope with stress. A healthy lifestyle, as well as quitting smoking, will also reduce your risk for heart disease associated with lupus. Talk to your doctor before starting an exercise program.
- Develop and maintain a good support system of family, friends, medical professionals, community organizations, and support groups.
Pregnancy and Contraception for Women With Lupus
Although pregnancy in women with lupus is considered high risk, most women with mild to moderate lupus can have healthy pregnancies. Regular care and good nutrition during pregnancy are essential. Talk to your doctor if you are pregnant or plan to become pregnant.
Research shows that birth control pills do not increase the risk for severe flares among women with lupus. As a result, doctors are increasingly prescribing oral contraceptives to women with inactive or stable disease.
The outcome for people with SLE has improved in recent years. Many people with SLE have mild symptoms. How well you do depends on how severe the disease is. Most people with SLE will require medicines for a long time. Nearly all will require hydroxychloroquine indefinitely.
Some people with SLE have abnormal immune deposits in the kidney cells. This leads to a condition called lupus nephritis. People with this problem may develop kidney failure. They may need dialysis or a kidney transplant.
A kidney biopsy is done to detect the extent of damage to the kidney and to help guide treatment. If active nephritis is present, treatment with immunosuppressive medicines including high doses of corticosteroids along with either cyclophosphamide or mycophenolate are needed.
OTHER PARTS OF THE BODY
SLE can cause damage in many different parts of the body, including:
- Blood clots in arteries of veins of the legs, lungs, brain, or intestines
- Destruction of red blood cells or chronic anemia.
- Fluid around the heart (pericarditis), or inflammation of the heart (myocarditis or endocarditis)
- Fluid around the lungs and damage to lung tissue
- Pregnancy problems, including miscarriage
- Bowel damage with abdominal pain and obstruction
- Inflammation in the intestines
- Severely low blood platelet count (platelets are needed to stop any bleeding)
- Inflammation of the blood vessels
SLE and Pregnancy
Both SLE and some of the medicines used for SLE can harm an unborn child. Talk to your provider before you become pregnant. If you become pregnant, find a provider who is experienced with lupus and pregnancy.
- Minimal mesangial glomerulonephritis Class I (Minimal mesangial glomerulonephritis Minimal mesangial glomerulonephritis)
- Mesangial proliferative lupus nephritis Class II (Mesangial proliferative lupus nephritis Mesangial proliferative lupus nephritis)
- Focal proliferative nephritis Class III (Focal proliferative nephritis Focal proliferative nephritis)
- Diffuse proliferative nephritis Class IV (Diffuse proliferative nephritis Diffuse proliferative nephritis)
- Membranous glomerulonephritis Class V (Membranous glomerulonephritis Membranous nephritis)
- Glomerulosclerosis Class VI (Glomerulosclerosis Glomerulosclerosis)
Arthritis is often used to refer to any disorder that affects the joints. Rheumatic diseases usually affect joints, tendons, ligaments, bones, and muscles. Arthritis is often used to refer to any disorder that affects the joints. Rheumatologic diseases usually affect joints, tendons, ligaments, bones, and muscles.
|Rheumatology and||Connective Tissue Diseases|
|* Ankylosing spondylitis||* Arthritis|
|* Arthritis and Rheumatic diseases||* Autoimmune diseases|
|* Autoinflammatory diseases||* Behçet’s disease|
|* Bursitis||* Giant cell arteritis|
|* Gout||* Juvenile arthritis|
|* Knee problems||* Lupus|
|* Osteoarthritis||* Polymyalgia rheumatica|
|* Psoriatic arthritis||* Reactive arthritis|
|* Rheumatoid arthritis||* Scleroderma|
|* Sjögren’s syndrome||* Systemic lupus erythematosus (Lupus)|
|* Tendinitis||* Rheumatologic diseases|
|* Glossary of rheumatology terms|
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