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Systemic lupus erythematosus

Disseminated lupus erythematosus; SLE; Lupus; Lupus erythematosus; Butterfly rash - SLE; Discoid lupus

 

Systemic lupus erythematosus (SLE) is an autoimmune disease. In this disease, the body's immune system mistakenly attacks healthy tissue. It can affect the skin, joints, kidneys, brain, and other organs.

Causes

 

The cause of autoimmune diseases is not fully known.

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 often than people from other races.

Certain drugs may also cause SLE.

 

Symptoms

 

Symptoms vary from person to person, and may come and go. Almost everyone with SLE has joint pain and swelling. 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.
  • Fatigue.
  • Fever with no other cause.
  • General discomfort, uneasiness, or ill feeling (malaise).
  • Hair loss.
  • Mouth sores.
  • Sensitivity to sunlight.
  • Skin rash: A "butterfly" rash in about half the people with SLE. The rash is most often 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: Abnormal heart rhythms (arrhythmias)
  • Lung: Coughing up blood and difficulty breathing
  • Skin: Patchy skin color and fingers that change color when cold (Raynaud phenomenon)
  • Kidney: Swelling in the legs, weight gain

Some people have only skin symptoms. This is called discoid lupus.

 

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:

  • Antinuclear antibody (ANA)
  • CBC with differential
  • Chest x-ray
  • Serum creatinine
  • Urinalysis

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)
  • Coombs test - direct
  • Cryoglobulins
  • ESR and CRP
  • Kidney function blood tests
  • Liver function blood tests
  • Rheumatoid factor
  • Antiphospholipid antibodies and lupus anticoagulant test
  • Kidney biopsy

 

Treatment

 

There is no cure for SLE. The goal of treatment is to control symptoms. Severe symptoms that involve the heart, lungs, kidneys, and other organs often need treatment from specialists.

Mild forms of the disease may be treated with:

  • NSAIDs for joint symptoms and pleurisy. Talk to your provider before taking these drugs.
  • Low doses of corticosteroids, such as prednisone, for skin and arthritis symptoms.
  • Corticosteroid creams for skin rashes.
  • Hydroxychloroquine, a drug also used to treat malaria.
  • Belimumab, a biologic drug, may be helpful in some people,

Treatments for more severe SLE may include:

  • High-dose corticosteroids.
  • Immunosuppressive drugs (drugs which dampen or suppress the immune system). These medicines are used if you do not get better with corticosteroids, or if your symptoms get worse when you stop taking them.
  • Blood thinners, such as Coumadin, for clotting disorders.

If you have SLE, it is also important to:

  • Wear protective clothing, sunglasses, and sunscreen when in the sun.
  • Get preventive heart care.
  • Stay up-to-date with immunizations.
  • Have tests to screen for thinning of the bones (osteoporosis).
  • Avoid tobacco and drink minimal amounts of alcohol.

 

Support Groups

 

Counseling and support groups may help with the emotional issues involved with the disease.

 

Outlook (Prognosis)

 

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.

The disease tends to be more active:

  • During the first years after diagnosis
  • In people under age 40

Many women with SLE can get pregnant and deliver a healthy baby. A good outcome is more likely for women who receive proper treatment and do not have serious heart or kidney problems. However, the presence of SLE antibodies raises the risk of miscarriage.

 

Possible Complications

 

Some people with SLE have abnormal 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.

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 anemia of chronic disease
  • 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
  • Stroke
  • Severely low blood platelet count (platelets are needed to stop any bleeding)
  • Inflammation of the blood vessels

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.

 

When to Contact a Medical Professional

 

Call your provider if you have symptoms of SLE. Also call if you have this disease and your symptoms get worse or a new symptom occurs.

 

 

References

Arntfield RT, Jicks CM. Systemic lupus erythematosus and the vasculitides. In: Marx JA, Hockberger RS, Walls RM, eds. Rosen's Emergency Medicine. Philadelphia, PA: Elsevier Saunders; 2014:chap 118.

Crow MK. Etiology and pathogenesis of systemic lupus erythematosus. In: Firestein GS, Budd RC, Gabriel SE, McInnes IB, O'Dell JR et al, eds. Kelley's Textbook of Rheumatology. 9th ed. Philadelphia, PA: Elsevier Saunders; 2013:chap 79.

Crow MK. Systemic lupus erythematosus. In: Goldman L, Schafer AI. Goldman's Cecil Medicine. Philadelphia, PA: Elsevier Saunders; 2016:chap 266.

Lisnevskaia L, Murphy G, Isenberg D. Systemic lupus erythematosus. Lancet. 2014;384(9957):1878-1888. PMID: 24881804 www.ncbi.nlm.nih.gov/pubmed/24881804.

 
  • Systemic lupus erythematosus

    Systemic lupus erythematosus

    Animation

  •  

    Systemic lupus erythematosus - Animation

    When your joints are sore and achy, you might assume you have arthritis. But if that joint pain strikes when you're still in your 30's, or even your 20's, it might be another condition entirely. You might have an autoimmune disease called systemic lupus erythematosus, or lupus, for short. An autoimmune disease means that your immune system, which normally serves as your body's first defense against infections, mistakenly attacks your own tissues. Imagine if you hit your hand over and over and over again. The skin would turn red and swell up, and it would probably hurt quite a bit. Well, the same kind thing happens inside your body when your immune system attacks your tissues. They swell up, and they hurt. Almost everyone with lupus has joint pain and swelling, but depending on what part of your body the lupus is attacking, you could have other symptoms too. If it's your skin, you might have a rash on your face and body. If lupus attacks your digestive tract, you might feel sick to your stomach. If it attacks your brain or nervous system, you may have numbness, tingling, vision problems, and headaches. So, how do you know that you have lupus? Your doctor will ask about your symptoms, listen to your heartbeat, and examine your nervous system. Doctors often use a test to check for antinuclear antibodies, the immune substances that attack your tissues. You'll likely also need other blood or urine tests, and perhaps an x-ray, CT, ultrasound or biopsy, depending on your symptoms. Taken together, your symptoms and the results of these tests can help your doctor determine whether you have lupus. If you do have lupus, lupus is a chronic condition, but, you can control its symptoms. For example, taking steroid medicines by mouth might help control the overactive immune response that's causing your lupus. Steroid creams can treat skin rashes. For achy joints, non-steroidal anti-inflammatory medicines like ibuprofen, and anti-malaria drugs might help. You may need stronger drugs if these medicines alone don't control your lupus symptoms. When you have lupus, you need to be extra careful about your health. Wear sunscreen and protective clothing whenever you're out in the sun, so your skin doesn't get even more irritated. Stop smoking and make sure you're up-to-date on your vaccines. Have your heart checked regularly because lupus can cause heart complications. Lupus can be a lifelong journey, but life with lupus is a lot better today than it was just a few decades ago. Improved treatments can help control your joint pain and other symptoms so you can live a pretty normal life. To improve your outlook with lupus, stay on top of your health care, and do call your doctor right away if your symptoms get worse or you develop any new symptoms.

  • Systemic lupus erythematosus

    Systemic lupus erythematosus - illustration

    Systemic lupus erythematosus is a chronic inflammatory autoimmune disorder which may affect many organ systems including the skin, joints and internal organs. The disease may be mild or severe and life-threatening. African-Americans and Asians are disproportionately affected.

    Systemic lupus erythematosus

    illustration

  • Lupus, discoid  - view of lesions on the chest

    Lupus, discoid - view of lesions on the chest - illustration

    Lupus, discoid - view of lesions on the chest: This close-up picture of the neck clearly shows the typical rounded appearance of discoid lupus. The whitish appearance is caused by scaling. The two dark spots are biopsy sites and are not part of the disease.

    Lupus, discoid - view of lesions on the chest

    illustration

  • Lupus, discoid on a child's face

    Lupus, discoid on a child's face - illustration

    The round or disk shaped (discoid) rash of lupus produces red, raised patches with scales. The pores (hair follicles) may be plugged. Scarring often occurs in older lesions. The majority (approximately 90%) of individuals with discoid lupus have only skin involvement as compared to more generalized involvement in systemic lupus erythematosis (SLE).

    Lupus, discoid on a child's face

    illustration

  • Systemic lupus erythematosus rash on the face

    Systemic lupus erythematosus rash on the face - illustration

    This is a picture of a systemic lupus erythematosis rash on the face. Lupus erythematosis often produces a "butterfly rash" or malar rash. Typically, the rash also appears on the nose.

    Systemic lupus erythematosus rash on the face

    illustration

  • Antibodies

    Antibodies - illustration

    Antigens are large molecules (usually proteins) on the surface of cells, viruses, fungi, bacteria, and some non-living substances such as toxins, chemicals, drugs, and foreign particles. The immune system recognizes antigens and produces antibodies that destroy substances containing antigens.

    Antibodies

    illustration

  • Systemic lupus erythematosus

    Animation

  •  

    Systemic lupus erythematosus - Animation

    When your joints are sore and achy, you might assume you have arthritis. But if that joint pain strikes when you're still in your 30's, or even your 20's, it might be another condition entirely. You might have an autoimmune disease called systemic lupus erythematosus, or lupus, for short. An autoimmune disease means that your immune system, which normally serves as your body's first defense against infections, mistakenly attacks your own tissues. Imagine if you hit your hand over and over and over again. The skin would turn red and swell up, and it would probably hurt quite a bit. Well, the same kind thing happens inside your body when your immune system attacks your tissues. They swell up, and they hurt. Almost everyone with lupus has joint pain and swelling, but depending on what part of your body the lupus is attacking, you could have other symptoms too. If it's your skin, you might have a rash on your face and body. If lupus attacks your digestive tract, you might feel sick to your stomach. If it attacks your brain or nervous system, you may have numbness, tingling, vision problems, and headaches. So, how do you know that you have lupus? Your doctor will ask about your symptoms, listen to your heartbeat, and examine your nervous system. Doctors often use a test to check for antinuclear antibodies, the immune substances that attack your tissues. You'll likely also need other blood or urine tests, and perhaps an x-ray, CT, ultrasound or biopsy, depending on your symptoms. Taken together, your symptoms and the results of these tests can help your doctor determine whether you have lupus. If you do have lupus, lupus is a chronic condition, but, you can control its symptoms. For example, taking steroid medicines by mouth might help control the overactive immune response that's causing your lupus. Steroid creams can treat skin rashes. For achy joints, non-steroidal anti-inflammatory medicines like ibuprofen, and anti-malaria drugs might help. You may need stronger drugs if these medicines alone don't control your lupus symptoms. When you have lupus, you need to be extra careful about your health. Wear sunscreen and protective clothing whenever you're out in the sun, so your skin doesn't get even more irritated. Stop smoking and make sure you're up-to-date on your vaccines. Have your heart checked regularly because lupus can cause heart complications. Lupus can be a lifelong journey, but life with lupus is a lot better today than it was just a few decades ago. Improved treatments can help control your joint pain and other symptoms so you can live a pretty normal life. To improve your outlook with lupus, stay on top of your health care, and do call your doctor right away if your symptoms get worse or you develop any new symptoms.

  • Systemic lupus erythematosus

    Systemic lupus erythematosus - illustration

    Systemic lupus erythematosus is a chronic inflammatory autoimmune disorder which may affect many organ systems including the skin, joints and internal organs. The disease may be mild or severe and life-threatening. African-Americans and Asians are disproportionately affected.

    Systemic lupus erythematosus

    illustration

  • Lupus, discoid  - view of lesions on the chest

    Lupus, discoid - view of lesions on the chest - illustration

    Lupus, discoid - view of lesions on the chest: This close-up picture of the neck clearly shows the typical rounded appearance of discoid lupus. The whitish appearance is caused by scaling. The two dark spots are biopsy sites and are not part of the disease.

    Lupus, discoid - view of lesions on the chest

    illustration

  • Lupus, discoid on a child's face

    Lupus, discoid on a child's face - illustration

    The round or disk shaped (discoid) rash of lupus produces red, raised patches with scales. The pores (hair follicles) may be plugged. Scarring often occurs in older lesions. The majority (approximately 90%) of individuals with discoid lupus have only skin involvement as compared to more generalized involvement in systemic lupus erythematosis (SLE).

    Lupus, discoid on a child's face

    illustration

  • Systemic lupus erythematosus rash on the face

    Systemic lupus erythematosus rash on the face - illustration

    This is a picture of a systemic lupus erythematosis rash on the face. Lupus erythematosis often produces a "butterfly rash" or malar rash. Typically, the rash also appears on the nose.

    Systemic lupus erythematosus rash on the face

    illustration

  • Antibodies

    Antibodies - illustration

    Antigens are large molecules (usually proteins) on the surface of cells, viruses, fungi, bacteria, and some non-living substances such as toxins, chemicals, drugs, and foreign particles. The immune system recognizes antigens and produces antibodies that destroy substances containing antigens.

    Antibodies

    illustration

A Closer Look

 

Tests for Systemic lupus erythematosus

 

     

    Review Date: 1/16/2016

    Reviewed By: Gordon A. Starkebaum, MD, Professor of Medicine, Division of Rheumatology, University of Washington School of Medicine, Seattle, WA. Also reviewed by David Zieve, MD, MHA, Isla Ogilvie, PhD, and the A.D.A.M. Editorial team.

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