I.

INTRODUCTION

DEFINITION

Systemic Lupus Erythematosus (SLE) is a chronic, often life-long, autoimmune disease.

SLE may affect various parts of the body, but it most often manifests in the skin, joints, blood,
and kidneys. The name describes the disease:

Systemic - The disease can affect organs and tissue throughout the body.
Lupus - Latin word for wolf. It refers to the rash that extends across the bridge
of the nose and upper cheekbones and was thought to resemble a wolf bite.
Erythematosus - Greek word for red and refers to the color of the rash.

There are several different forms of lupus. SLE is the most common type and is the type
of lupus that can lead to serious systemic complications. Types of SLE include:

Subacute Cutaneous Lupus Erythematosus - Lupus that is confined to the skin

(butterfly rash) and does not affect other parts of the body. About 10% of
people with this type of lupus go on to develop SLE.
Discoid Lupus Erythematosus A type of cutaneous lupus that produces a
potentially scarring disc-shaped rash on the face, scalp, or ears.
Drug-Induced Lupus is a temporary and mild form of lupus caused by certain
prescription medications. They include some types of high blood pressure,
diuretics and some antiseizure drugs . Symptoms resolve once the medication is
stopped.
Neonatal Lupus A rare condition that sometimes affects infants born to
mothers who have SLE. Babies with neonatal lupus are born with skin rash, liver
problems, and low blood counts. This may develop to heart problems.

CAUSES
Cause of SLE is unknown. It is most likely a combination of genetic and environmental factors.
People who develop an autoimmune disease may have a genetic predisposition that is triggered
by some environmental factor such as sunlight, stress hormones, or viruses.

RISK FACTORS

Gender
About 90% of lupus patients are women, most diagnosed when they are in
their childbearing years (between the ages of 15 and 50).

Age
Most people develop SLE between the ages of 15 - 44. About 15% of patients

Family History
A family history plays a strong role in SLE. A brother or sister of a patient with
the disorder has 20 times the risk as someone without an immediate family member
with SLE.

Environmental Triggers
In genetically susceptible people, there are various external factors that
can trigger symptoms. Possible SLE triggers include colds, fatigue, stress,
chemicals, sunlight, and certain drugs.

TESTS AND DIAGNOSIS

Diagnosing lupus is difficult because signs and symptoms vary considerably from person
to person. Signs and symptoms of lupus may vary over time and overlap with those of many
other disorders. No one test can diagnose lupus. The combination of blood and urine tests,
signs and symptoms, and physical examination findings leads to the diagnosis.
A. Laboratory tests
Blood and urine tests may include:

Complete blood count. This test measures the number of red blood cells, white blood cells
and platelets as well as the amount of hemoglobin, a protein in red blood cells. Results may
indicate you have anemia, which commonly occurs in lupus. A low white blood cell or
platelet count may occur in lupus as well.

Erythrocyte sedimentation rate. This blood test determines the rate at which red blood cells
settle to the bottom of a tube in an hour. A faster than normal rate may indicate a systemic
disease, such as lupus. The sedimentation rate isn't specific for any one disease. It may be
elevated if you have lupus, another inflammatory condition, cancer or an infection.

Kidney and liver assessment. Blood tests can assess how well your kidneys and liver are
functioning. Lupus can affect these organs.

Urinalysis. An examination of a sample of your urine may show an increased protein level or
red blood cells in the urine, which may occur if lupus has affected your kidneys.

Antinuclear antibody (ANA) test. A positive test for the presence of these antibodies
produced by your immune system indicates a stimulated immune system. While most
people with lupus have a positive ANA test, most people with a positive ANA do not have
lupus. If you test positive for ANA, your doctor may advise more-specific antibody testing.

B. Imaging tests
If your doctor suspects that lupus is affecting your lungs or heart, he or she may suggest:

Chest X-ray. An image of your chest may reveal abnormal shadows that suggest fluid or
inflammation in your lungs.

Lupus can harm your kidneys in many different ways, and treatments can vary, depending on
the type of damage that occurs. In some cases, it's necessary to test a small sample of kidney
tissue to determine what the best treatment might be. The sample can be obtained with a
needle or through a small incision.


SIGNS AND SYMPTOMS
SLE can cause a wide range of symptoms, depending on the areas of the body that are
affected. The onset of this disease may be insidious or acute. Patients may present with any of
the following manifestations:

Constitutional (fatigue, fever, arthralgia, weight changes, tenderness, pain)

Musculoskeletal ( arthralgia, arthropathy, myalgia, frank arthritis, avascular necrosis)
Integumentary (malar rash, alopecia, photosensitivity, discoid lupus)
Renal (acute or chronic renal failure, acute nephritic disease)
Neuropsychiatric (seizure, psychosis, depression)
Pulmonary (pleurisy, pleural effusion, pneumonitis, pulmonary hypertension, interstitial
lung disease)
Gastrointestinal (nausea, dyspepsia, abdominal pain)
Cardiac (pericarditis, pericardial friction rub associated with myocarditis,)
Hematologic (cytopenias such as leukopenia, lymphopenia, anemia, or
thrombocytopenia

TREATMENTS AND DRUGS

There is currently no cure for SLE, but there are different medications that can help
relieve many of the symptoms and reduce the chances of organ damage.
Treatment for lupus depends on your signs and symptoms. Determining whether your signs
and symptoms should be treated and what medications to use requires a careful discussion of
the benefits and risks with your doctor. As your signs and symptoms flare and subside, you and
your doctor may find that you'll need to change medications or dosages. The medications most
commonly used to control lupus include:

Nonsteroidal anti-inflammatory drugs (NSAIDs). Over-the-counter NSAIDs, such as naproxen

sodium (Aleve) and ibuprofen (Advil, Motrin IB, others), may be used to treat pain, swelling
and fever associated with lupus. Stronger NSAIDs are available by prescription. Side effects
of NSAIDs include stomach bleeding, kidney problems and an increased risk of heart
problems.

Antimalarial drugs. Medications commonly used to treat malaria, such as

hydroxychloroquine (Plaquenil), also can help control lupus. Side effects can include
stomach upset and, very rarely, damage to the retina of the eye.

risk of infection. The risk of side effects increases with higher doses and longer term
therapy.

Immunosuppressants. Drugs that suppress the immune system may be helpful in serious
cases of lupus. Examples include azathioprine (Imuran, Azasan), mycophenolate (CellCept),
leflunomide (Arava) and methotrexate (Trexall). Potential side effects may include an
increased risk of infection, liver damage, decreased fertility and an increased risk of cancer.
A newer medication, belimumab (Benlysta), also reduces lupus symptoms in some people.
Side effects include nausea, diarrhea and fever.

NURSING CONSIDERATIONS

Watch for constitutional symptoms( joint pain or stiffness, weakness, fever, fatigue, and
chills)
Observe for dyspnea, chest pain, and edema of the extremities
Note the size, type and location of skin lesions
Check urine for hematuria, scalp for hair loss, and skin and mucous membranes for
petechiae, bleeding, ulceration, pallor, and bruising
Provide a balanced diet
- Renal involvement may mandate a low sodium, low- protein diet
Urge the patient to get plenty of rest and schedule diagnostic test and procedures to
allow adequate rest
Explain all test and procedures
Apply heat packs to relieve joint pain and stiffness
Encourage regular exercise to maintain full range of motion and prevent contractures
- Teach ROM exercises as well as body alignment and postural techniques
- Arrange for physical therapy and occupational counseling as appropriate
Administer prescribed medications
- Explain their expected benefits
- Watch for adverse effects, especially when the patient is taking high doses of
corticosteroids
- Advise the patient receiving cyclophosphamide to maintain adequate hydration
- Give Mesna(mesnex) to prevent hemorrhagic cystitis and ondansetron ( Zofran)
to prevent nausea and vomiting as prescribed
Monitor the patients vital signs, intake and output, weight, and laboratory reports
- Check pulse rate and observe for orthopnea
- Check stools and GI secretions for blood
Monitor the patient for hypertension, weight gain, and other signs of renal involvement
Assess for signs of neurologic damage, such as personality change, paranoid or psychotic
behavior, ptosis, or diplopia
- Take seizure precaution
- If Raynauds phenomenon is present, warm and protect the patients hands and
feet.
Avoid excessive sunlight exposure, and wear sunscreen (ultraviolet light is the one of the
main triggers of flares)
Get plenty of rest (fatigue is another common SLE symptom)

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

Kidneys. 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).

Lungs. 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.

Heart. 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:

Infection. 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.

Cancer. 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.

PROGNOSIS
A few decades ago SLE was regarded as a terminal condition, as many people would die of an
associated complication within a few years of being diagnosed.

However, some people with SLE are still at risk of life-threatening complications as a result of
damage to internal organs and tissues, such as heart attack or stroke. SLE, and some of the
treatments for it, can also increase your risk of developing potentially serious infections.