systemic lupus erythematosus (sle) elizabeth boldon rn, msn · systemic lupus erythematosus (sle)...

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Elizabeth Boldon RN, MSN

Liz Boldon is a Nurse Education Specialist at Mayo Clinic in Rochester,

Minnesota. She received a BSN from Allen College in Waterloo, Iowa in 2002

and an MSN with a focus in education from the University of Phoenix in 2008.

She has bedside nursing experience in medical neurology and the

neuroscience ICU.

Abstract:

Systemic Lupus Erythematosus (SLE) is a chronic inflammatory autoimmune disease of

unknown cause that affects multiple organ systems. Lupus is associated with significant

mortality and morbidity. There is currently no cure for lupus and treatments vary widely.

This course will discuss the symptoms, causes, risk factors and complications related to

lupus as well as its diagnosis, common tests and treatments both traditional and

complimentary. This course will also address the important aspect of coping and

support of those who are dealing with lupus.

Systemic Lupus

Erythematosus (SLE)


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Continuing Nursing Education Course Director & Planners

William A. Cook, PhD, Director, Douglas Lawrence, MS, Course Planner Webmaster

Susan DePasquale, CGRN, MSN, FPMHNP-BC, Lead Nurse Planner

Accreditation Statement

This activity has been planned and implemented in accordance with the policies of

NurseCe4Less.com and the continuing nursing education requirements of the American

Nurses Credentialing Center's Commission on Accreditation for registered nurses.

Credit Designation

This educational activity is credited for 3 hours. Nurses may only claim credit

commensurate with the credit awarded for completion of this course activity.

Course Author & Planner Disclosure Policy Statements

It is the policy of NurseCe4Less.com to ensure objectivity, transparency, and best

practice in clinical education for all CNE educational activities. All authors and course

planners participating in the planning or implementation of a CNE activity are expected

to disclose to course participants any relevant conflict of interest that may arise.

Statement of Need

Systemic Lupus Erythematosus affects individuals differently and nurses will care for

individuals that present with varying symptoms. Although there is no cure for SLE, there

are effective treatments for it. Nurses need to have a good understanding of the disease

to provide individuals diagnosed with SLE safe and appropriate care and to educate

them about how to maintain qualify of life and wellness.

Course Purpose

This course will increase learners’ knowledge and skills related to Systemic Lupus

Erythematosus.


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Learning Objectives

Describe the symptoms of lupus.

List some of the tests & procedures used to diagnose lupus.

Discuss medications and guiding principles used in treating lupus.

Explain what a flare is and its significance.

Identify resources for further support and information related to lupus.

Target Audience

Advanced Practice Registered Nurses, Registered Nurses, Licensed Practical Nurses,

and Associates

Course Author & Director Disclosures

Elizabeth Boldon, RN, MSN, William S. Cook, PhD, Douglas Lawrence, MS,

Susan DePasquale, CGRN, MSN, FPMHNP-BC – all have no disclosures

Acknowledgement of Commercial Support:

There is no commercial support for this course.

Activity Review Information:

Reviewed by Susan DePasquale, CGRN, MSN, FPMHNP-BC

Release Date: 1/1/2015 Termination Date: 8/1/2016

Please take time to complete the self-assessment Knowledge Questions before

reading the article. Opportunity to complete a self-assessment of knowledge

learned will be provided at the end of the course.


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1. Skin problems that are common features of lupus include:

a. rashes

b. oral ulcers

c. alopecia

d. all of the above

2. An SLE flare is diagnosed based on:

a. increased signs and symptoms interfering with the patient’s quality of life

b. laboratory indicators

c. imaging studies

d. all of the above

3. Risk factors for SLE include:

a. one’s gender – is more common in men

b. age – generally diagnosed between ages of 15 and 40

c. racial origin – is most common in Caucasions

d. geographic location – is most common in European countries

4. True or False. There is no risk to pregnancy during active disease.

a. True

b. False

5. True or False. Quality of life (QOL) is generally compromised in the lupus

patient, due to everyday symptoms and the problems of flares, depression,

treatment, frequent hospital visits and restrictions on many aspects of life

such as travel and pregnancy.

a. True

b. False


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INTRODUCTION

Systemic lupus erythematosus (SLE) is a chronic inflammatory autoimmune disease of

unknown cause that affects multiple organ systems. SLE, also simply referred to as

Lupus, is associated with significant mortality and morbidity. There is currently no cure

for lupus and treatments vary widely. This course will discuss the symptoms, causes,

risk factors and complications related to lupus as well as its diagnosis, common tests

and treatments both traditional and complimentary. This course will also address the

important aspect of coping and support of those who are dealing with lupus.

Lupus is prevalent worldwide but the proportion of patients with lupus varies between

different ethnic groups. Lupus affects approximately 40 of every 100,000 northern

Europeans, more than 200 out of every 100,000 black African and black Caribbean

people and about 100 of every 100,000 Asian people. Lupus mostly affects women of

childbearing age (15-50 years.) The ratio of females to males with lupus is 9:1.

(Ferenkeh-Koroma, 2012.) The Lupus Foundation of America estimates that 1.5 million

Americans have some form of lupus and that 90% of those diagnosed are women

(Mendelson, C., 2009.)

The following is a quote from a lupus patient that helps illustrates the complexity of this

disease:

“So I was on this low-level of not quite feeling right, but not sure what was wrong

with me type of feeling for quite a long time. I had some other weird ‘medical

oddities’ show up – swollen lymph nodes under my arm pit, low grade fever,

chest pain. Horrible hip pain that required the use of a cane – nothing was really


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checked out, just more drugs prescribed to ease the symptoms.” (Mendelson, C.,

2008.)

No two cases of lupus are exactly alike. Signs and symptoms may come on suddenly or

develop slowly, may be mild or severe, and may be temporary or permanent. Most

people with lupus have mild disease characterized by episodes, or flares, when signs

and symptoms worsen for a while, then improve or even disappear completely for a

time. Some individuals may have multiple-organ involvement, while others only have

one system of the body involved, for example the skin. Lupus is often called the great

imitator because signs and symptoms may be vague and nonspecific and mimic other

disorders such as:

Fibromyalgia

Sjögren syndrome

Dermatomyositis

Hematologic problems

Drug-induced lupus

This quote from a lupus patient illustrates the way the disease is not always recognized

immediately:

“After a while, I would just keep going even though I felt so horrible because my

family doctor kept asking me if I was depressed. When I starting having

abdominal pain, difficulty swallowing, and nausea, he said it was stress induced.

He told me the same thing when my hair began to thin… The doctor just kept

trying to push anti-depressants and I kept refusing even though I was beginning


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to think that maybe I could use some. So I stayed away from the doctor for quite

some time.” (Mendelson, C., 2008.)

The signs and symptoms of lupus that one may experience will depend on which body

systems are affected by the disease. The next section of this course will describe some

of the common symptoms of lupus by system.

SYSTEMIC SIGNS AND SYMPTOMS

Dermatologic

Skin problems including photosensitivity, rashes, oral ulcers, and alopecia are a

common feature of lupus. Besides the characteristic butterfly-shaped facial rash (see

picture below), patients may develop flat or raised rashes or lesions everywhere on the

body. A definitive diagnosis requires a biopsy. Although skin problems may not be life

threatening, they can be uncomfortable or painful and decrease the patient’s quality of

life due to disfigurement and altered body image. Skin conditions may occur alone or

accompany a more serious inflammatory response that affects vital organs.

webmd.com


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Musculoskeletal

Most patients with lupus have nonerosive arthritis, including joint tenderness, edema,

and effusions that are symmetric and usually nondeforming. The nondeforming arthritis

of lupus differentiates it from rheumatoid arthritis, which causes progressive joint

destruction. The arthritis of lupus frequently involves the joints of the hands as well as

the wrists and knees. Joint symptoms occur in over 90% of lupus patients at some time

during the illness and are often seen as the earliest manifestation. Arthritis, with

inflammation, occurs in 65 to 70% of patients and tends to migratory and symmetrical

(Schur, P. & Gladman, D., 2013.)

Neurologic

Patients with lupus may be depressed because of cerebral vasculitis or because they

are trying to cope with the stress of a chronic illness. In some patients, central nervous

system involvement causes strokes, seizures, cognitive impairment, headaches, visual

disturbance, or psychosis. Other less common problems are movement disorders,

cranial neuropathies, myelitis, and meningitis (Schur, P. & Gladman, D., 2013.)

Gastrointestinal (GI)

Patients with lupus may have abdominal discomfort or pain, diarrhea, anorexia, or

nausea. Vasculitis of the intestine can cause acute abdominal pain with cramps,

vomiting, and diarrhea. These problems may result from pancreatitis, vasculitis, and

bowel infarction secondary to lupus vasculitis. Other potential neurologic symptoms

include cognitive defects, organic brain syndromes, delirium, psychosis, seizures,

headache, and/or peripheral neuropathies.


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Hematologic

Autoantibodies may be produced against cell surface antigens of blood cells, including

red blood cells, and platelets. This results in hemolytic anemia, leukopenia, or

thrombocytopenia.

Renal

About 50% of patients with lupus have renal involvement such as glomerulonephritis,

usually from deposition of immune complexes and resultant inflammation and tissue

damage. Most of the remaining 50% of lupus patients have subclinical disease that can

be demonstrated if renal biopsy is performed. Renal involvement usually develops in the

first few years of illness and should be detected early by periodic urinalysis, and lab

tests (Schur, P. & Gladman, D., 2013.)

Cardiopulmonary

Pericarditis and pleuritis, the most common cardiopulmonary disorders in lupus occur

when immune complexes are deposited in the pericardial and pleural spaces producing

an inflammatory response. Other potential pulmonary symptoms include pleurisy,

pleural effusions, pneumonitis, lung disease, pulmonary hypertension, and alveolar

hemorrhage (Schur, P. & Gladman, D., 2013.)

Other Common Symptoms:

Other common symptoms of lupus include fatigue, myalgia, weight changes and fever.

Fatigue is the most common complaint and is occasionally the most debilitating. It

occurs in 80 to 100% of patients, and its presence is not clearly correlated with other


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measures of disease activity. Fatigue is strongly associated with diminished exercise

tolerance. Fatigue, however, may not be related to active lupus, but to one or more of

the following: increased work load, depression, unhealthful habits, stress, anemia,

hypothyroidism, use of certain medications, any inflammatory and/or infectious disease,

coexistent fibromyalgia, sleep disturbances, deconditioning, or a perception of poor

social support. Fatigue due to lupus may respond to glucocorticoids, antimalarials, and,

in some studies, to exercise and psychosocial interventions.

Weight changes are frequent in patients with lupus and may be related to the disease or

its treatment. Weight loss often occurs prior to the diagnosis of lupus. Unintentional

weight loss may be due to decreased appetite, to the side effects of medications, and to

gastrointestinal disease. Weight gain in lupus is usually due to one of two factors: salt

and water retention associated with hypoalbuminemia or increased appetite associated

with the use of glucocorticoids.

Fever that is thought to be due to active disease is seen in over half of lupus patients.

Fever may also represent infection or a drug reaction.

Because of the complexity and variety of symptoms, patients with lupus often find it

takes time to receive a proper diagnosis. Interviews conducted with 147 SLE patients

revealed that on average, patients spend two to four years and see three physicians

before the disease is correctly diagnosed (Manzi, S., 2009.)

Patients with lupus can be classified into three basic categories, as below:

Quiescent


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Lead a normal life

May only present with a rash and/or fatigue

Treatment may NSAIDs and topical applications

Stable with occasional flare

May present with rash and/or chest pain

Occasionally calls the nurse advice line

Treatment may include intravenous or oral corticosteroids

Serious

Presents with kidney, hematological and/or brain involvement

Treatment may include continuous corticosteroids and immunosuppressive drugs

Most patients with lupus can expect to live a normal lifespan with appropriate treatment

and lifestyle changes, but exacerbations or flares can be life-threatening (Ferenkeh-

Koroma, 2012.)

Lupus Flares

Lupus flares are exacerbation of disease. A flare is diagnosed based on an increase in

signs and symptoms that interfere with the patient’s quality of life, lab indicators, and

imaging studies. Sometimes, however, a flare may be underway when the lab and

imaging studies are normal. At other times, the patient may not have an increase in

symptoms, but their lab values may indicate increased inflammation.


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Flares are common and can be triggered by many factors: too much work or not enough

rest, stress an emotional crisis, an infection, an injury, surgery, pregnancy, the

postpartum period, ultraviolet light exposure, or sudden discontinuation of lupus

medications.

Signs of a flare include increasing fatigue, pain, headache, fever, general ill feeling, and

onset of new rash. Even a low-grade fever or new rash can signal that a flare is under

way. Patients with lupus should pay close attention to any signs or symptoms, however

subtle, as they may indicate an increase in disease activity. A lupus flare can affect

different organs and may cause serious damage if left untreated. Flares may occur

several times a year and last days or weeks. During a flare, patients may need to use

analgesics for pain syndromes associated with the joints, skin, or other areas of the

body. For mild to moderate pain, an NSAID, such as ibuprofen, or tramadol (Ultram) are

often effective. If the flare causes more intense pain, a more potent opioid analgesic

such as hydrocodone, oxycodone, or fentanyl may be necessary (Pullen, R., Brewer, S.

& Ballard, A., 2009.)

Patients with lupus should follow these guidelines (Pullen, R., 2008) to minimize flares

and reduce negative outcomes from flares:

Recognize the symptoms of a flare

Protect themselves from light exposure

Prevent and recognize infection

Take medications as prescribed

Be aware of pregnancy risks


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Avoid undue emotional and physical stress and get sufficient rest

Don’t smoke and limit alcohol intake

Chose a healthy and balanced diet

Ask questions of healthcare providers if things are not understood

Be aware of available support groups for patients and families

SLE CAUSES AND RISK FACTORS

Lupus is an autoimmune disease. The immune system is designed to protect the body.

In patients with lupus, the immune system malfunctions and attacks the healthy cells of

the body. The exact cause of lupus, the resulting autoimmune inflammatory response

and the risks of inheriting the disease are not clear. It is likely that lupus results from a

combination of genetics and environment. It appears that people with an inherited

predisposition for lupus may develop the disease when they come into contact with

something in the environment that can trigger lupus. The cause for lupus in most cases,

however, is unknown. Some potential triggers include the following:

Sunlight

Exposure to the sun may bring on lupus skin lesions or trigger an internal response in

susceptible people. A helpful education tool for health professionals to share with

patients is the Lupus Foundation of America (2013) website, which informs on exposure

to ultraviolet rays and reactions that can take many forms; available at

http://www.lupus.org/webmodules/webarticlesnet/templates/new_newsroom.aspx?articl

eid=3940&zoneid=8.

http://www.lupus.org/webmodules/webarticlesnet/templates/new_newsroom.aspx?articleid=3940&zoneid=8

http://www.lupus.org/webmodules/webarticlesnet/templates/new_newsroom.aspx?articleid=3940&zoneid=8


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Medications

Certain types of anti-seizure medications, blood pressure medications and antibiotics

can trigger Lupus. People who have drug-induced lupus usually see their symptoms go

away when they stop taking the medication. Approximately 15,000 cases of drug-

induced lupus are reported each year (Ferenkeh-Koroma, 2012.)

Stress Stress has been implicated in causing flares, particularly of mild disease.

Unfortunately, stress as an entity has never been clinically defined, except as it relates

to its psychosomatic effects (Schur & Gladman, 2013.)

Occupational exposure to toxins / pesticides

These environmental contributors are difficult to isolate, but researchers have

established links between lupus and a variety of toxins, such as cigarette smoke, silica,

and mercury (Schur & Gladman, 2013.)

Trauma

Tissue damage associated with a severe injury can result in profound inflammatory

responses that may trigger autoimmune development in lupus-prone individuals (Anam,

Amare, Naik, Szabo & Davis, 2009.)

Illness or Infection Infectious disease agents such as the Epstein-Barr Virus

(EBV, which causes mononucleosis or “mono”), herpes zoster virus (the virus

that causes shingles), and cytomegalovirus have also been implicated in

exacerbating SLE (Schur & Gladman, 2013.)


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Surgery

Surgery is another insult that can increase lupus activity. The mechanism is unclear

but may be related to the release of nuclear and other antigens into the bloodstream,

in which they bind to circulating antinuclear antibodies to form immune complexes

(Schur and Gladman, 2013.)

Pregnancy

Pregnancy can cause an exacerbation or can even trigger the first symptoms of lupus; a

relapse is more likely to develop in the immediate postpartum period. The hormonal

adjuvants that are used during ovulation induction and during ovarian stimulation in

preparation for in vitro fertilization may also cause exacerbations of SLE. Therapeutic

abortions can also induce a relapse, perhaps via mechanisms related to pregnancy or

to the surgery itself (Schur and Gladman, 2013.)

Risk Factors

Risk factors of lupus include the following:

Gender-specific - lupus is more common in women.

Age Specific - although lupus affects people of all ages, it's most often

diagnosed between the ages of 15 and 40.

Race - lupus is more common in African Americans, Hispanics and Asians.


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COMPLICATIONS OF SLE

Inflammation caused by lupus can affect many areas of the body; these areas can

include the following systems:

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

If the brain is affected by lupus, one 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 vasculitis.

Lungs

Having lupus increases the chances of developing pleurisy, which can make breathing

painful.


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Heart

Lupus can cause inflammation of the heart muscle, the arteries or heart membrane

(pericarditis). The risk of cardiovascular disease and heart attacks increases greatly as

well.

Other types of complications

Having lupus also increase the risk of the following comorbid conditions:

Infection

Individuals 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 the 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.


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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 recommend delaying

pregnancy until the disease has been under control for at least six months.

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. Best practice for the testing patients to rule in or out SLE will vary

depending on the constellation of symptoms for each patient. Providers should be

knowledgeable of the signs and symptoms of SLE and consider it in the differential

diagnosis for patients as appropriate. As discussed above, patients often have delays in

receiving an appropriate diagnosis of SLE, however, a timely diagnosis and initiation of

treatment is in the best interest of all patients.

Laboratory tests

Blood and urine tests may include:

Complete Blood Count (CBC)


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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 anemia, which commonly occurs in lupus. A low white blood cell or

platelet count may occur in lupus as well. (Schur & Wallace, 2013.)

Erythrocyte Sedimentation Rate (ESR)

This blood test determines the rate at which red blood cells settle to the bottom of

a tube with one 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 one has lupus, another inflammatory condition, cancer or an

infection (Schur & Wallace, 2013.)

C-reactive protein (CRP). A protein that is elevated when inflammation is found

in the body. Although ESR and CRP reflect similar degrees of inflammation,

sometimes one will be elevated when the other is not. This test may be repeated

to test response to medications (Schur & Wallace, 2013).

Kidney and Liver Assessment

Blood tests can assess how well the kidneys and liver function. Lupus can affect

these organs.

Urinalysis (UA)

Because the body’s waste is processed by the kidneys, testing a sample of urine

can reveal any problems with the way the kidneys are functioning. Lupus can


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attack the kidneys without any warning signs, so these tests can be very

important. The most common urine tests look for cell casts (bits of cells that

normally would be removed when the blood is filtered through your kidneys), and

proteinuria (protein being spilled into the body because the kidneys are not

filtering the waste properly). An examination of a sample of urine may also show

an increased protein level or red blood cells in the urine, which may occur if lupus

has affected the kidneys.

24-Hour Urine Collection

This test is done for calculation of creatinine clearance and for quantitation of

proteinuria or protein/creatinine ratios.

Antinuclear Antibody (ANA) Test

A positive test for the presence of these antibodies — produced by the 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

one tests positive for ANA, a provider may advise more-specific antibody testing.

Antinuclear antibodies (ANA) are antibodies that connect, or bind, to the nucleus

-- the "command center" -- of the cell. This process damages, and can destroy,

the cells. The ANA blood test is a sensitive test for lupus, since these antibodies

are found in 97 percent of people with the disease. When three or more typical

features of lupus are present -- such as involvement of the skin, joints, kidneys,

lungs, heart, blood, or nervous system -- a positive ANA test will confirm a


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diagnosis of lupus. However, a positive ANA test result does not always mean

one has lupus. The ANA can be positive in people with other illnesses, or positive

in people with no illness. The ANA can also change from positive to negative, or

negative to positive, in the same person. Still, lupus is usually the diagnosis when

these antinuclear antibodies are found in the blood (Schur & Wallace, 2013.)

Comprehensive Metabolic Panel (CMP)

A comprehensive metabolic panel is a blood test that measures one’s sugar

(glucose) level, electrolyte and fluid balance, kidney function, and liver function.

This panel measures the blood levels of sodium, potassium, calcium, chloride,

carbon dioxide, glucose, blood urea nitrogen, creatinine, protein, albumin,

bilirubin, and liver enzymes. This test may be used to monitor the effects of

medications, or to monitor the effects and potential complications of SLE itself

(Schur & Gladman, 2013.)

Imaging tests

If a provider suspects that lupus is affecting the lungs or heart, he or she may suggest:

X-ray

An x-ray of involved body areas affected may be ordered, such as joints and

especially a chest x-ray. The test ordered would depend on the body part being

affected. An image of the chest may reveal abnormal shadows that suggest fluid

or inflammation in the lungs.

http://www.webmd.com/hw-popup/electrolytes-and-electrolyte-imbalances

http://www.webmd.com/hw-popup/kidneys-7905

http://www.webmd.com/a-to-z-guides/liver-function-test-lft

http://www.webmd.com/vitamins-and-supplements/lifestyle-guide-11/supplement-guide-potassium

http://www.webmd.com/vitamins-supplements/ingredientmono-781-calcium.aspx?activeingredientid=781&activeingredientname=calcium

http://www.webmd.com/a-to-z-guides/chloride-cl

http://www.webmd.com/a-to-z-guides/creatinine-and-creatinine-clearance-blood-tests

http://www.webmd.com/digestive-disorders/bilirubin-15434


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Echocardiogram

The electrocardiogram uses sound waves to produce real-time images of the

beating heart. It can check for problems with the valves and other portions of the

heart.

Renal Ultrasonography

Ultrasonography is used to assess kidney size and to rule out urinary tract

obstruction when there is evidence of renal impairment.

Computed Tomography (CT)

The CT is indicated for abdominal pain, suspected pancreatitis, interstitial lung

disease.

Magnetic Resonance Imaging (MRI)

MRI may be ordered to rule out focal neurologic deficits or cognitive dysfunction.

Contrast angiography

This may be helpful if vasculitis affecting medium sized arteries is suspected.

Biopsy

Lupus can harm the 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


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of organ tissue (i.e. skin or kidney) to determine what the best treatment might be. The

sample can be obtained with a needle, or through a small incision.

The American College of Rheumatology (ACR) has produced a set of 11 classification

criteria used in the diagnosis of lupus (see Table 1). Typically, the ACR classification

criteria are used for research purposes. Individuals fulfilling any of the 11 criteria can be

included in lupus studies. Individuals fulfilling two or three of the criteria, especially

immunological, antinuclear antibody and hematological blood tests, may develop lupus,

but do not yet fulfill sufficient criteria to make a formal diagnosis of the disease. To be

diagnosed with lupus, a patient must present with four or more of the ACR classification

criteria. Because of the complexity of the disease and its symptoms, diagnosing lupus

can be a challenge to even the most experienced clinician. The average time between

initial presentation and definitive diagnosis is between two and four years (Weinstein,

P., 2012) and patients see an average of three physicians during that time.

Table 1 – Classification Criteria used in the Diagnosis of SLE

TREATMENT AND MEDICATIONS

1. Malar rash – a rash over the cheeks and nose, often in the shape of a butterfly 2. Discoid rash – a rash that appears as red, raised, disk-shaped patches 3. Photosensitivity – a reaction to sun or light that causes a skin rash to appear or get worse 4. Oral ulcers – sores appearing in the mouth 5. Arthritis – joint pain and swelling of two or more joints in which the bones around the joints

do not become destroyed 6. Serositis – inflammation of the lining around the lungs (pleuritis) or inflammation of the lining

around the heart that causes chest pain which is worse with deep breathing (pericarditis) 7. Kidney disorder – persistent protein or cellular casts in the urine 8. Neurological disorder – seizures or psychosis 9. Blood disorder – anemia (low red blood cell count), leukopenia (low white blood cell count),

lymphopenia (low level of specific white blood cells), or thrombocytopenia (low platelet count)

10. Immunologic disorder – abnormal anti-double-stranded DNA or anti-Sm, positive antiphospholipid antibodies

11. Abnormal antinuclear antibody (ANA)


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Morbidity and mortality associated with lupus is significant with a fourfold increased risk

of death reported in patients with lupus compared with the general population. In 1955,

the reported survival rate of lupus was around 50% at 5 years; and, more recently,

survival was 80% at 15 years. Survival has improved significantly over the past 50 years

with 93% of patient with lupus surviving for 5 years and 92% surviving for more than 10

years. These improved survival rates may be related to advanced medical therapy in

general (notably renal transplantation and the use of immunosuppressive drugs) along

with a better understanding of the pathogenesis of the disease and early diagnosis

(Ferenkeh-Koroma, 2012.)

Despite improved life expectancy of lupus patients during the past few decades,

available evidence suggests that the quality of this extended life remains poor

compared with that of the general population and worse than that of individuals with

some other chronic diseases (Toloza, S., Sequeira, W. & Jolly, M., 2011.)

Early on, patients tend to die of active disease (manifestations of vasculitis, pulmonary

hemorrhage, kidney problems) or infection. Over time, cardiovascular disease and

osteoporosis become more of a problem. Patients also have a higher risk of cancer

throughout life. Lupus has an unpredictable course, with flares and remissions. But

underlying the reversible inflammatory changes is irreversible organ damage caused by

the disease itself and, possible, by treatment. Preventing bone disease, heart disease,

and cancer now play more prominent roles in managing lupus (Manzi, S., 2009.)

Treatment of lupus aims to reduce the over-activity of the immune system to reduce the

associated inflammation, and to control symptoms. Immunosuppression may be


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required on either a short or long-term basis, and high doses of corticosteroids are often

required as well as low maintenance doses for some patients. Many treatments for

lupus also require the use of prophylactic medications, particularly for the prevention of

osteoporosis commonly seen with the long-term use of corticosteroids.

An individual’s treatment for lupus depends on their signs and symptoms. Determining

whether the signs and symptoms should be treated and what medications to use

requires a careful discussion of the benefits and risks with a care provider. There are

some general guidelines all patients with lupus should follow, as specified below (Schur,

P. & Wallace, D., 2013):

Lifestyle and Prevention

Sun protection

Individuals with lupus should avoid exposure to direct or reflected sunlight and

other sources of ultraviolet (UV) light (e.g. fluorescent and halogen lights.) They

should use sunscreens, preferably those products that block both UV-A and UV-

B, with a high skin protection factor (SPF). A sunscreen with an SPF of 55 or

greater is recommended.

Diet and nutrition

A balanced diet is recommended, consisting of carbohydrates, proteins, and fats.

Exercise

Inactivity produced by acute illness causes a rapid loss of muscle mass, bone

demineralization, and loss of stamina resulting in a sense of fatigue. This can


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usually be treated with isometric and graded exercise. In selected refractory

cases, relief can be obtained with antimalarial drugs.

Smoking Cessation

Cigarette smoking may increase the risk of developing lupus and smokers in

general have more active disease. Patients should be counseled not to smoke or

to quit smoking and should be provided with help to do so.

Immunizations

Patients with lupus should receive appropriate immunizations prior to the

institution of immunosuppressive therapies.

Avoidance of specific medications

Some data suggest that sulfonamide-containing antibiotics may cause

exacerbations and should, therefore, be avoided.

Pregnancy and contraception

Pregnancy should be avoided during active disease (especially with significant organ

impairment) due to the high risk of miscarriage and exacerbation of lupus. Women with

lupus should be counseled not to become pregnant until the disease has been

quiescent for at least six months. Thus, contraception and family planning are

particularly important for women with SLE. Maternal health and fetal development

should be monitored frequently during pregnancy. If possible, delivery should occur in a

controlled setting. In addition, many women with SLE should be followed by an

obstetrician knowledgeable in high-risk pregnancies.


27

Patients with SLE with one or more of the following features should be followed by an

obstetrician experienced in management of high risk pregnancies:

Prior history of poor obstetric outcomes

Renal involvement

Cardiac involvement

Pulmonary hypertension

Interstitial lung disease

Evidence of active lupus

High-dose glucocorticoid therapy

Immunosuppressive therapy

Antiphospholipid antibodies/syndrome

Antibodies to Ro/La (predisposing to neonatal lupus)

Multiple gestation

The following characteristics are associated with high maternal and fetal risk:

Severe pulmonary hypertension (mean pressure >50 mmHg)

Restrictive lung disease (forced vital capacity <1 liter)

Heart failure

Chronic renal failure (creatinine >2.8 mg/dL)

Active renal disease

History of severe preeclampsia or HELLP syndrome

Stroke within the previous six months

Severe lupus flare within the previous six months


28

If any of the listed high risk factors for poor maternal/fetal outcomes are present, a

decision to pursue pregnancy should be carefully considered and made with an

awareness of the potentially serious consequences (Schur & Bermas, 2013.)

There are two issues related to therapy of women with lupus who become pregnant:

monitoring of disease activity in both asymptomatic and symptomatic patients and

treatment of active disease. Mothers should be assessed for disease activity at least

once each trimester and more often if they have active lupus. Treatment of SLE during

pregnancy is associated with some unique problems. Consideration must be given to

the following issues:

Medications used to treat SLE may cross the placenta and cause fetal harm.

Thus, the risks and benefits of treatment during pregnancy must be

repeatedly weighed against the risk of activity of SLE having a deleterious

effect on the mother and the fetus.

Nephritis in pregnancy requires special consideration because of its potential

morbidity and possible confusion with preeclampsia (Schur & Bermas, 2013).

Pregnant women with SLE should be aware of the risks and signs and symptoms of

complications some resources available to them; helpful resources include:

The Lupus Foundation of America -

http://www.lupus.org/webmodules/webarticlesnet/templates/new_donate.aspx?a=314&z

=6&page=1

Web MD - http://lupus.webmd.com/guide/pregnancy-lupus

http://www.lupus.org/webmodules/webarticlesnet/templates/new_donate.aspx?a=314&z=6&page=1

http://www.lupus.org/webmodules/webarticlesnet/templates/new_donate.aspx?a=314&z=6&page=1

http://lupus.webmd.com/guide/pregnancy-lupus


29

Medication

As signs and symptoms flare and subside, medications or dosages may need to be

altered. The medications most commonly used to control lupus include:

Nonsteroidal anti-inflammatory drugs (NSAIDs)

Over-the-counter NSAIDs, such as naproxen (Aleve) and ibuprofen (Advil,

Motrin, 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.

Corticosteroids

Prednisone and other types of corticosteroids can counter the inflammation of

lupus, but often produce long-term side effects — including weight gain, easy

bruising, osteoporosis, hypertension diabetes and increased risk of infection. The

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


30

Immune suppressants

Drugs that suppress the immune system may be helpful in serious cases of

lupus. Examples include cyclophosphamide (Cytoxan), 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.

Belimumab

In March of 2011, the FDA granted approval for this new targeted therapy for the

treatment of lupus. This was the first such medication to receive such a

designation in over 50 years (Lo, M. & Tsokos, G., 2011.)

Alternative and Complementary Medicine (CAM) Therapies

Those who suffer from lupus should take steps to care for their bodies. Simple

preventative measures can help reduce the incidence of lupus flares and, should they

occur, help the individual better cope with the signs and symptoms they experience.

These individuals should try to perform the following:

Adequate rest

People with lupus often experience persistent fatigue that's different from normal

tiredness and that isn't necessarily relieved by rest. For that reason, it can be


31

hard to judge when one needs to slow down. Those with lupus should get plenty

of sleep at night and naps or breaks during the day as needed.

Be sun smart

Because ultraviolet light can trigger a flare, those with lupus should wear

protective clothing, such as a hat, long-sleeved shirt and long pants, and use

sunscreens with a sun protection factor (SPF) of at least 55 every time they go

outside.

Regular exercise

Exercise can help one recover from a flare, reduce the risk of heart attack, help

fight depression and promote general well being.

Avoid Smoking

Smoking increases the risk of cardiovascular disease and can worsen the effects

of lupus on the heart and blood vessels.

Eat a healthy diet

A healthy diet emphasizes fruits, vegetables and whole grains. Sometimes one

with lupus may have dietary restrictions, especially if they have hypertension,

kidney damage or gastrointestinal problems.

Additionally, there are CAM therapies that may benefit people with lupus. However,

these therapies are usually used with conventional medications, as adjuncts or in


32

combination. These treatments should be discussed with a provider before initiating

them. The provider can help weigh the benefits and risks and advise if the treatments

will interfere with current lupus medications.

It is important for clinicians to create a therapeutic relationship with patients, inform

them of all treatment options, explain one’s own bias as a provider, and form an

agreement to collaborate to avoid working at cross purposes to medical therapies.

Healthcare consumers are increasingly savvy and with all the resources available via

technology, they are likely to discover and learn about CAM therapies on their own if not

through a clinician. They may begin CAM therapies on their own due to their anxiety to

feel better, and not readily disclose this information unless asked directly.

CAM treatments for lupus include the following:

Dehydroepiandrosterone (DHEA)

Supplements containing this hormone have been shown to reduce the dose of

steroids needed to stabilize symptoms in some people who have lupus.

Flaxseed

Flaxseed contains a fatty acid called alpha-linolenic acid, which may decrease

inflammation in the body. Some studies have found that flaxseed may improve

kidney function in people who have lupus that affects the kidneys. Side effects of

flaxseed include bloating and abdominal pain.


33

Fish oil

Fish oil supplements contain omega-3 fatty acids that may be beneficial for

people with lupus. Preliminary studies have found some promise, though more

study is needed. Side effects of fish oil supplements can include nausea,

belching and a fishy taste in the mouth.

Vitamin D

There is some evidence to suggest that people with lupus may benefit from

supplemental vitamin D to manage osteoporosis secondary to lupus and

corticosteroid use.

B12 and folic acid

This can be effective in managing anemia.

St. John’s wort

This has mild serotonin-boosting properties, and may be used to fight fatigue and

depression.

COPING AND SUPPORT

Individuals with lupus are likely to have a range of painful feelings about their condition,

from fear to extreme frustration. The challenges of living with lupus increase the risk of

depression and related mental health problems, such as anxiety, stress and low self-

esteem. Things that may help those with lupus and families cope include:


34

Learn as much as possible about lupus

Write down questions about lupus and ask them at doctor appointments. Ask

doctors or nurses for reputable sources of further information. The more one

knows about lupus, the more confident they'll feel in their treatment choices.

Gather support among friends and family

Talk about lupus with friends and family and explain ways they can help out

during flares. Lupus can be frustrating for loved ones because they usually can't

see it and the person may not appear sick. They can't tell if the individual is

having a good day or a bad day unless they communicate that. Those with lupus

should be open about what they're feeling so that friends and family know what to

expect.

Take time out

Those with lupus can cope with stress in life by taking time for themselves. They

can use that time to read, meditate, listen to music or write in a journal. They

should find activities that calm and renew themselves.

Connect with others who have lupus

It may be helpful for those with lupus to talk to other people who have lupus.

They can connect with other people who have lupus through support groups in

the community or through online message boards. Other people with lupus can


35

offer unique support because they're facing many of the same obstacles and

frustrations that the individual is facing.

A final quote that illustrates the frustration that can accompany the journey to diagnosis

of lupus is as follows (Mendelson, C., 2008):

“I complained and complained about tiredness, swollen glands, fevers,

migraines, coldness. I was sick with pneumonia/pleurisy at least once a year. My

tendons swelled and weakened. Periods of illness grew longer. Doctor shopping,

always trying to find an answer – but mostly being diagnosed with Disaffected

Housewife Syndrome, may name for what male doctors see as a women’s

problem of complaining of being ick when he believed they really weren’t (often

on first sight).”

Quality of life (QOL) is generally compromised in the lupus patient, due to everyday

symptoms and the problems of flares, depression, treatment, frequent hospital visits

and restrictions on many aspects of life such as travel and pregnancy. The Quality of

Life Scale (QOLS), created originally by American psychologist John Flanagan in the

1970's, has been adapted for use in chronic illness groups. Although the definition of

QOL is still evolving, one researcher defines QOL as "a broad range of human

experiences related to one's overall well-being. It implies value based on subjective

functioning in comparison with personal expectations and is defined by subjective

experiences, states and perceptions. Quality of life, by its very natures, is idiosyncratic

to the individual, but intuitively meaningful and understandable to most people." This

definition denotes a meaning for QOL that transcends health. Flanagan’s Quality of Life


36

Scale (QOLS), befits this definition of QOL (Burckhardt & Anderson, 2003.) Table 2

shows this QOLS.

Table 2 – The Quality of Life Scale

The Quality of Life scale (QOL) in Table 2 involves asking the person with lupus to

answer questions according to the following instructions:

Read each item and circle the number that best describes how satisfied you are

at this time.

Answer each item even if you do not currently participate in an activity or have a

relationship. You can be satisfied or dissatisfied with not doing the activity or

having the relationship.

The QOLS is scored by adding up the score on each item to yield a total score for the

instrument. Scores can range from 16 to 112. The QOLS scores are summed so that a

higher score indicates higher quality of life. Average total score for healthy populations

is about 90.

For rheumatic disease groups, the average score ranges are 83 for rheumatoid arthritis,

84 for systemic lupus erythematosus, 87 for osteoarthritis, and 92 for young adults with

juvenile rheumatoid arthritis (Burckhardt & Anderson, 2003.)


37

Living with lupus can have a profound effect on a person’s mental and emotional well-

being. Patients are likely to experience mental and physical problems such as difficulty

concentrating or sleeping. Emotions such as grief, fear, anxiety, and depression are

also common. The feelings associated with lupus can have multiple causes, including:

Outward effects of the disease or its treatment

http://www.webmd.com/balance/tc/grief-and-grieving-topic-overview

http://www.webmd.com/anxiety-panic/default.htm

http://www.webmd.com/depression/default.htm


38

Work and activity limitations

Pain, fatigue and other physical symptoms

Social isolation

Uncertainty about the future

Difficulty with family relationships

Sometimes the mental and emotional effects of lupus can be related to the disease

process itself or medications used to treat it. Common problems that may be associated

with the disease include:

Cognitive dysfunction

Depression and anxiety

Mood swings and personality changes

If patients are experiencing any of these problems, it’s important to discuss them with a

healthcare provider, who can help to find solutions. This may include a change in

medication to control the lupus, or the provider may add medications to treat problems

like anxiety and depression. Other options may include a referral to a mental health

professional, who will be able to help identify additional ways to cope with lupus (Schur

& Wallace, 2013.)

Lupus is not a well-known disease to the general public; sufferers may find it difficult to

explain their limitations to friends and colleagues. Family life can suffer too, particularly

as this disease often strikes women who are mothers of young children. Chronic sleep

disturbance affected 72% of lupus outpatients in one study (Wheeler, T., 2010), which

also showed severe anxiety and depression in about 25% of this cohort. The sleep


39

disturbance contributes to, but is by no means the only factor in, the chronic debilitating

fatigue that is a major feature of the disease for so many; varying estimates put the

incidence between 50 and 100% of sufferers (Wheeler, T., 2010.) This is why having

support from family, friends and support groups is so important for those who suffer

from lupus. Some of those support groups and reputable sources of information include:

The Lupus Foundation of America - www.lupus.org

Lupus Research Institute – www.lupusresearchinstitute.org

Dorough Lupus Foundation – www.doroughlupusfoundation.org

S.L.E. Lupus Foundation – www.lupusny.org

Lupus Alliance of America – www.lupusalliance.org

Lupus International - www.lupusinternational.com

Molly’s Fund – www.mollysfund.org

WebMD - http://lupus.webmd.com/default.htm

MayoClinic.com - http://www.mayoclinic.com/health/lupus/DS00115

Medline Plus - http://www.nlm.nih.gov/medlineplus/lupus.html

There are numerous other helpful websites related to lupus for health providers and

individuals affected by lupus; these may be found at http://cure4lupus.org .

SUMMARY

Systemic lupus is an incurable and potentially debilitating disease that carries with it the

risk of serious multi-organ complications and early death. Understanding the basics of

the this disease and related treatments not only helps healthcare workers to interact

http://www.lupus.org/

http://www.lupusresearchinstitute.org/

http://www.doroughlupusfoundation.org/

http://www.lupusny.org/

http://www.lupusalliance.org/

http://www.lupusinternational.com/

http://www.mollysfund.org/

http://lupus.webmd.com/default.htm

http://www.mayoclinic.com/health/lupus/DS00115

http://www.nlm.nih.gov/medlineplus/lupus.html

http://cure4lupus.org/


40

with these patients more sensitively and competently, but also brings educational

benefits since this is a multi-system disease that involves grappling with the

complexities of the immune system. This course has discussed the symptoms, causes,

risk factors and complications related to lupus as well as its diagnosis, common tests

and treatments both traditional and complimentary. This course also addressed the

important aspect of coping and support of those who are dealing with lupus, offering

additional resources for those who are interested.

Please take time to help the NURSECE4LESS.COM course planners evaluate nursing

knowledge needs met following completion of this course by completing the self-

assessment Knowledge Questions after reading the article.

Correct Answers, page 42.


41

1. Skin problems that are common features of lupus include:

a. rashes

b. oral ulcers

c. alopecia

d. all of the above

2. An SLE flare is diagnosed based on:

a. increased signs and symptoms interfering with the patient’s quality of life

b. laboratory indicators

c. imaging studies

d. all of the above

3. Risk factors for SLE include:

a. one’s gender – is more common in men

b. age – generally diagnosed between ages of 15 and 40

c. racial origin – is most common in Caucasians

d. geographic location – is most common in European countries

4. True or False. There is no risk to pregnancy during active disease.

a. True

b. False

5. True or False. Quality of life (QOL) is generally compromised in the

lupus patient, due to everyday symptoms and the problems of flares,

depression, treatment, frequent hospital visits and restrictions on many

aspects of life such as travel and pregnancy.

a. True

b. False


42

Correct Answers:

1. d

2. d

3. b

4. b

5. a

Footnotes:

1. Anam, K., Amare, M, Naik, S, Szabo, K.A. & Davis, T.A. (2009.) Severe tissue trauma triggers the

autoimmune state systemic lupus erythematosus in the MRL/++ lupus-prone mouse. Lupus, April

18(4), 318-331.

2. Burckhardt, C.S. & Anderson, L.A. (2003.) The Quality of Life Scale (QOLS): Reliability, Validity,

and Utilization. Health and Quality of Life Outcomes, 1(60), 1-7.

3. Dall’Era, M. & Chakravarty, E.F. (2011.) Treatment of Mild, Moderate, and Severe Lupus

Erythematosus: Focus on New Therapies. Current Rheumatology Reports, 13, 308-316.

4. Ferenkeh-Koroma A. (2012.) Systemic lupus erythematosus: nurse and patient education.

Nursing Standard, 26 (39), 49-57.

5. Lo, M.S. & Tsokos, G.C. (2012.) Treatment of systemic lupus erythematosus: new advances in

targeted therapy. Annals of the New York Academy of Sciences, The Year in Immunology, 1247,

138-152.

6. Mayo Clinic Staff. (October 26, 2011.) Lupus. Retrieved April 14, 2013, from


7. Manzi S. (2009.) Lupus update: Perspective and clinical pearls. Cleveland Clinic Journal of

Medicine, 76 (2), 137-142.

8. Mendelson, C. (2009.) Diagnosis: A Liminal State for Women Living With Lupus. Health Care for

Women International, 30, 390-407.

9. Pullen, R.L. (2008.) Stay in the loop about lupus. Nursing made incredibly easy!,

January/February, 44-54.

10. Pullen, R.L., Brewer, S. & Ballard, A. (2009.) Putting a face on systemic lupus erythematosus.

Nursing 2009, 23-28.

11. Schur, P.H. & Bermas, B.L. (2013.) Pregnancy in women with systemic lupus erythematosus. In

D.S. Pisetsky (Ed.),UpToDate. Available from http://www.uptodateonline.com.

http://www.ncbi.nlm.nih.gov/pubmed?term=Anam%20K%5BAuthor%5D&cauthor=true&cauthor_uid=19276300

http://www.ncbi.nlm.nih.gov/pubmed?term=Amare%20M%5BAuthor%5D&cauthor=true&cauthor_uid=19276300

http://www.ncbi.nlm.nih.gov/pubmed?term=Naik%20S%5BAuthor%5D&cauthor=true&cauthor_uid=19276300

http://www.ncbi.nlm.nih.gov/pubmed?term=Szabo%20KA%5BAuthor%5D&cauthor=true&cauthor_uid=19276300

http://www.ncbi.nlm.nih.gov/pubmed?term=Davis%20TA%5BAuthor%5D&cauthor=true&cauthor_uid=19276300


http://www.uptodateonline.com/


43

12. Schur, P.H. & Gladman, D.D. (2013). Overview of the clinical manifestations of systemic

erythematosus in adults. In D.S. Pisetsky (Ed.),UpToDate. Available from

http://www.uptodateonline.com.

13. Schur, P.H. & Wallace, D.J.. (2013). Diagnosis and differential diagnosis of systemic



14. Schur, P.H. & Wallace, D.J.. (2013). Overview of the therapy and prognosis of systemic



15. Toloza S.M.A., Sequeira, W. & Jolly, M. (2011.) Treatment of Lupus; Impact on Quality of Life.

Current Rheumatology Reports, 13, 324-337.

16. Weinstein, P.K. (2012.) The Face of Lupus. The Nurse Practitioner, 37 (12), 38-45.

17. Wheeler, T. (2010.) Systemic lupus erythematosus: the basics of nursing care. British Journal of

Nursing, 19 (4), 249-253.

The information presented in this course is intended solely for the use of healthcare professionals taking

this course, for credit, from NurseCe4Less.com.

The information is designed to assist healthcare professionals, including nurses, in addressing issues

associated with healthcare.

The information provided in this course is general in nature, and is not designed to address any specific

situation. This publication in no way absolves facilities of their responsibility for the appropriate

orientation of healthcare professionals.

Hospitals or other organizations using this publication as a part of their own orientation processes

should review the contents of this publication to ensure accuracy and compliance before using this

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Hospitals and facilities that use this publication agree to defend and indemnify, and shall hold

NurseCe4Less.com, including its parent(s), subsidiaries, affiliates, officers/directors, and employees from

liability resulting from the use of this publication.

The contents of this publication may not be reproduced without written permission from

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