Systemic Lupus Erythematosus (SLE)

What is it?

Systemic lupus erythematosus, referred to as SLE or lupus, is a chronic (long-term) disease that causes inflammation — pain and swelling. It is sometimes called the “great imitator,” because people often confuse lupus with other health problems due to its wide range of symptoms.

In addition to affecting the skin and joints, it can affect other organs in the body such as the kidneys, the tissue lining the lungs (pleura) and heart (pericardium), and the brain. Most patients feel fatigue and have rashes, arthritis (painful and swollen joints) and fever.

Lupus flares vary from mild to serious.

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Complications of SLE

Inflammation caused by lupus can affect many areas of your body, including:

  • Lupus can cause serious kidney damage, and kidney failure is one of the leading causes of death among people with lupus. Signs and symptoms of kidney problems may include generalized itching, chest pain, nausea, vomiting and leg swelling (edema).
  • Brain and central nervous system. If your brain is affected by lupus, you may experience headaches, dizziness, behavior changes, hallucinations, and even strokes or seizures. Many people with lupus experience memory problems and may have difficulty expressing their thoughts.
  • Blood and blood vessels. Lupus may lead to blood problems, including anemia and increased risk of bleeding or blood clotting. It can also cause inflammation of the blood vessels (vasculitis).
  • Having lupus increases your chances of developing an inflammation of the chest cavity lining (pleurisy), which can make breathing painful. You may also be more susceptible to pneumonia.
  • Lupus can cause inflammation of your heart muscle, your arteries or heart membrane (pericarditis). The risk of cardiovascular disease and heart attacks increases greatly as well.

 

Other types of complications

Having lupus also increases your risk of:

  • People with lupus are more vulnerable to infection because both the disease and its treatments weaken the immune system. Infections that most commonly affect people with lupus include urinary tract infections, respiratory infections, yeast infections, salmonella, herpes and shingles.
  • Having lupus appears to increase your risk of cancer.
  • Bone tissue death (avascular necrosis). This occurs when the blood supply to a bone diminishes, often leading to tiny breaks in the bone and eventually to the bone’s collapse. The hip joint is most commonly affected.
  • Pregnancy complications. Women with lupus have an increased risk of miscarriage. Lupus increases the risk of high blood pressure during pregnancy (preeclampsia) and preterm birth. To reduce the risk of these complications, doctors often recommend delaying pregnancy until your disease has been under control for at least six months.

http://www.mayoclinic.org/diseases-conditions/lupus/basics/complications/con-20019676

 

What Causes it?

The immune system is the body’s defense system. When healthy, it protects the body by making antibodies (blood proteins) that attack foreign germs and cancers. With lupus, the immune system misfires. Instead of producing protective antibodies, an autoimmune disease begins and makes “autoantibodies,” which attack the patient’s own tissues. Doctors sometimes refer to this as a “loss of self-tolerance.”

As the attack goes on, other immune cells join the fight. This leads to inflammation and abnormal blood vessels (vasculitis). These antibodies then end up in cells in organs, where they damage those tissues. Why this inflammatory response begins is not clear. It most likely results from a mix of inherited tendencies and things in your environment. These include viruses, sunlight and drug allergies. People with lupus may also have an impaired process for clearing old and damaged cells from the body, which causes an abnormal immune response.

Most often, lupus starts in people in their 20s and 30s.

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How is it diagnosed?

 Lupus can be hard to detect because it is a complex disease that has many symptoms, and they can come on slowly. As experts in diagnosing and treating autoimmune diseases such as lupus, rheumatologists can best determine whether a patient has lupus and advise them about treatment options.

People with lupus often have symptoms that are not specific to lupus. These include fever, fatigue, weight loss, blood clots and hair loss in spots or around the hairline. They may also have heartburn, stomach pain, and poor circulation to the fingers and toes. Pregnant women can have miscarriages.

The American College of Rheumatology has a list of symptoms and other measures that doctors can use as a guide to decide if a patient with symptoms has lupus. If your doctor finds that you have at least four of these problems, and finds no other reason for them, you may have lupus:

  • Rashes:
    • butterfly-shaped rash over the cheeks — referred to as malar rash
    • red rash with raised round or oval patches — known as discoid rash
    • rash on skin exposed to the sun
  • Mouth sores: sores in the mouth or nose lasting from a few days to more than a month
  • Arthritis: tenderness and swelling lasting for a few weeks in two or more joints
  • Lung or heart inflammation: swelling of the tissue lining the lungs (referred to as pleurisy or pleuritis) or the heart (pericarditis), which can cause chest pain when breathing deeply
  • Kidney problem: blood or protein in the urine, or tests that suggest poor kidney function
  • Neurologic problem: seizures, strokes or psychosis (a mental health problem)
  • Abnormal blood tests such as:
    • low blood cell counts: anemia, low white blood cells or low platelets
    • positive antinuclear antibodies (ANA) result: antibodies that can cause the body to begin attacking itself that are present in nearly all lupus patients
    • certain antibodies that show an immune system problem: anti-double-strand DNA (called anti-dsDNA), anti-Smith (referred to as anti-Sm) or antiphospholipid antibodies, or a false-positive blood test for syphilis (meaning you do not really have this infection)

If your doctor suspects you have lupus from your symptoms, you will need a series of blood tests to confirm that you do have the disease. The most important blood screening test measures ANA, but you can have ANA and not have lupus. Therefore, if you have positive ANA, you may need more specific tests to prove the diagnosis. These blood tests include antibodies to anti-dsDNA and anti-Sm.

The presence of antiphospholipid antibodies can help doctors detect lupus. These antibodies signal a raised risk of certain complications such as miscarriage, difficulties with memory, or blood clots that may lead to stroke or lung injury. Doctors also may measure levels of certain complement proteins (a part of the immune system) in the blood, to help detect the disease and follow its progress.

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How is it treated?

There is no cure for lupus, and treating lupus can be a challenge. However, treatment for lupus has improved a great deal. Treatment depends on the type of symptoms you have and how serious they are. Patients with muscle or joint pain, fatigue, rashes and other problems that are not dangerous can receive “conservative” treatment.

Common treatment options include:

  • Nonsteroidal anti-inflammatory drugs (NSAIDs): NSAIDs decrease swelling, pain and fever. These drugs include ibuprofen (brand names Motrin, Advil) and naproxen (Naprosyn, Aleve). Some of these NSAIDs can cause serious side effects like stomach bleeding or kidney damage. Always check with your doctor before taking any medications that are over the counter (without a prescription) for your lupus.
  • Antimalarial drugs: Patients with lupus also may receive an antimalarial medication such as hydroxychloroquine (Plaquenil). Though these drugs prevent and treat malaria, they also help relieve some lupus symptoms, such as fatigue, rashes, joint pain or mouth sores. They also may help prevent abnormal blood clotting.
  • Corticosteroids and immune suppressants: Patients with serious or life-threatening problems such as kidney inflammation, lung or heart involvement, and central nervous system symptoms need more “aggressive” (stronger) treatment. This may include high-dose corticosteroids such as prednisone (Deltasone and others) and drugs that suppress the immune system. Immune suppressants include azathioprine (Imuran), cyclophosphamide (Cytoxan) and cyclosporine (Neoral, Sandimmune). Recently mycophenolate mofetil has been used to treat severe kidney disease in lupus – referred to as lupus nephritis.
  • Biologics: Newer treatment options include drugs called biologics that are already approved for treatment of other rheumatic diseases such as rheumatoid arthritis. Examples are rituximab (Rituxan) and abatacept (Orencia). These two drugs are not approved for treatment of lupus. In 2011, though, the FDA approved a biologic, belimumab (Benlysta), for treatment of active SLE in adult patients, but not for those with severe disease. It is the first new drug approved for lupus since 1955.See more at: http://www.rheumatology.org/I-Am-A/Patient-Caregiver/Diseases-Conditions/Lupus#sthash.EmLb3SqW.dpuf