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SYSTEMIC LUPUS
ERYTHEMATOSUS
Dana Bartlett, BSN, MSN, MA, CSPI
Dana Bartlett is a professional nurse and
author. His clinical experience includes 16
years of ICU and ER experience and over
20 years of as a poison control center
information specialist. Dana has published
numerous CE and journal articles, written
NCLEX material and textbook chapters, and
done editing and reviewing for publishers
such as Elsevier, Lippincott, and Thieme. He has written widely on the
subject of toxicology and was recently named a contributing editor,
toxicology section, for Critical Care Nurse journal. He is currently employed
at the Connecticut Poison Control Center and is actively involved in lecturing
and mentoring nurses, emergency medical residents and pharmacy students.
ABSTRACT
Systemic lupus erythematosus is a rare autoimmune disease, chronic
in nature, which primarily affects women of childbearing age. Systemic
lupus erythematosus can cause damage to essentially every organ
system, and the pathogenesis of this disease is a complex, and
incompletely understood process involving genetic, environmental, and
immunologic factors. Episodic flares and remission are characteristic of
the disease. Understanding how to prevent and treat flares when they
occur helps people with systemic lupus erythematosus maintain better
health. Pharmacological treatments and prevention as well as lifestyle
changes such as cessation of smoking, diet and exercise are discussed.
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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. Pharmacology content is 0.5 hours (30 minutes).
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 continuing
nursing education (CNE) 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 Learning Need
Health clinicians are required to know about the diagnosis and
treatment of patients with systemic lupus erythematosus, as well as
the risk factors, prevention strategies and treatments to promote
quality of life and to reduce early mortality.
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Course Purpose
To provide clinicians with an up-to-date review of the literature about
systemic lupus erythematosus in the areas of epidemiology,
pathogenesis, clinical picture, diagnosis, prevention and treatment of
symptoms.
Target Audience
Advanced Practice Registered Nurses, Registered Nurses, Licensed
Vocational Nurses
Course Author & Director Disclosures
Dana Bartlett, BSN, MA, MSN, CSPI, William S. Cook, PhD,
Douglas Lawrence, MA, Susan DePasquale, MSN, FPMHNP-BC - all
have no disclosures.
Acknowledgement of Commercial Support: There is none.
PLEASE TAKE TIME TO COMPLETE A SELF-ASSESSMENT OF KNOWLEDGE, ON
PAGE 4, SAMPLE 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.
Systemic lupus erythematosus (SLE) is a rare autoimmune
disease, chronic in nature, that primarily affects
a.
b.
c.
d.
2.
Native Americans.
women of childbearing age.
elderly Caucasian women and men.
post-menopausal women.
True or False: Systemic lupus erythematosus is an
autoimmune disease caused by genetic predisposition and
exposure to environmental factors such as nutrition,
infection, and certain chemicals.
a. True
b. False
3.
Environmental risk factors that definitively cause systemic
lupus erythematosus include
a.
b.
c.
d.
4.
A 2014, meta-analysis of risk factors for systemic lupus
erythematosus found an increased risk for SLE associated
with
a.
b.
c.
d.
5.
post-menopausal hormone therapy.
cigarette smoking.
use of oral contraceptives.
None of the above
the use of oral contraceptives.
post-menopausal hormone therapy.
pregnancy.
Answers a., and b., above
Which of the following statements is/are true about
systemic lupus erythematosus?
a.
b.
c.
d.
More than 90% of patients with SLE are women
SLE is less common in African Americans
SLE is more common in Caucasians than Native Americans.
All of the above
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Introduction
Systemic lupus erythematosus is a rare autoimmune disease, chronic
in nature, which primarily affects women of childbearing age. Systemic
lupus erythematosus (SLE) can cause damage to essentially every
organ system, and the pathogenesis of SLE is a complex, and
incompletely understood process involving genetic, environmental, and
immunologic factors. Symptomatic therapy for SLE can be very
effective and advances in treatment have dramatically increased
survival rates. However, there is no cure and patients diagnosed with
SLE have a significantly higher risk for early mortality.
Epidemiology And Pathogenesis Of SLE
More than 90% of patients who have SLE are women.1,2 Systemic
lupus erythematosus is more common in African Americans than in
Caucasians and African American women are particularly susceptible.
People of Asian, Afro Caribbean, and Native American heritage are also
more likely to have SLE than Caucasians and the severity and the
progress of the disease are worse in these populations.3,4 The annual
incidence of SLE has been estimated to be 1–10 per 100,000.5
Systemic lupus erythematous is an autoimmune disease, and the
pathogenesis of SLE is both simple and complex. The simplicity lies in
the basic process of how the disease happens. Patients who have a
genetic susceptibility to SLE are exposed to environmental triggers,
these induce an abnormal immune response, and the immune
response results in the clinical signs and symptoms of SLE.1 The
complexity lies in the interaction between these three factors and as
Long, et al. (2106) wrote: “Both genetic predisposition and
environmental factors such as nutrition, infection, and chemicals are
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implicated in the pathogenic process of autoimmunity, however, how
much and by what mechanisms each of these factors contribute to the
development of autoimmunity remain unclear.”6
Genetic Influences
Systemic lupus erythematous is a heritable disease; there is a high
concordance rate (the presence of the same trait) in monozygotic
twins than dizygotic twins or siblings, and someone who has a sibling
with SLE is more likely to develop the disease.7 Although research has
identified susceptibly genes for SLE, it is unclear how and for whom
these result in the active disease;6,7 and, these genetic variations
appear to account for only a small part of the heritability of systemic
lupus erythematosus.8
Environmental Influences
There are many environmental exposures that may be risk factors for
the development of SLE. Not surprisingly, the evidence that supports a
causative role for these environmental risk factors in the development
of SLE is sometimes strong and at other times weak and conflicting.
Environmental risk factors that are likely to increase the risk for SLE
are cigarette smoking, female gender, oral contraceptives and
postmenopausal hormone therapy, silica exposure, drug use, and
other environmental factors, which are further discussed below.
Cigarette Smoking
Cigarette smoking is associated with a moderate increase in the risk
for developing systemic lupus erythematous,9,10 but it is not clear how
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tobacco exposure contributes as a cause of SLE. Cigarette smoking
may initiate autoimmune processes, causes inflammation and
oxidative stress, which may in turn damage DNA, and it may expose
the body to antigens that are normally suppressed.9,11 Cigarette
smoking may increase disease activity.12
Female Gender
As mentioned previously, the great majority of adult cases of systemic
lupus erythematous are seen in women. Research has shown that
female sex hormones and the X chromosome contribute to the
development of SLE but how and why these factors influence
development and expression of the disease is not known.13,14
Oral Contraceptives and Postmenopausal Hormone Therapy
Oral contraceptives have long been thought of a risk factor for
developing SLE. However, the evidence linking oral contraceptives and
SLE is conflicting, and a recent (2014) meta-analysis did not find an
association between the use of oral contraceptives and an increased
risk for SLE.15 The same meta-analysis did find an association between
postmenopausal hormone therapy and an increased risk for SLE.
Female sex hormones influence immune system function and this may
explain the association between therapeutic use of hormones, female
gender and systemic lupus erythematosus. Additionally, although SLE
is a disease that predominantly affects women of childbearing age,
there is no conclusive evidence that pregnancy is a risk factor for new
onset of the disease.16
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Silica Exposure
Silica is a naturally occurring compound found in sand, quartz and
other minerals. Occupational exposure to silica in industries such as
construction, ceramic or glass manufacturing, and mining that use
silica have been identified as a risk factor for developing SLE.17,18 and a
dose-response relationship has been established.9 The likely
mechanism of action is inflammation that initiates autoimmune
processes.18
Drug-induced SLE
Drug-induced SLE is a rare, self-limiting, idiosyncratic reaction to
certain medications that is characterized by: 1) Delayed onset, from
one month to > three years;22 2) Mild signs and symptoms that are
typical of SLE; and, 3) Complete resolution within days or weeks of
discontinuing the drug therapy. Commonly used drugs such as antiepileptics, proton pump inhibitors, and statins have been implicated as
causes of drug-induced SLE,23 but aside from hydralazine and
procainamide, which have a 5-8% and 20% risk, respectively, of
causing SLE, drug-induced SLE is rare. Interested learners are referred
to Rubin (2015)22 for a list of drugs that may cause this phenomenon.
Other Environmental Risk Factors
Air pollution, diet, exposure to heavy metals, pesticides and solvents,
infections, ultraviolet radiation, and vitamin D have been examined for
their role as risk factors for systemic lupus erythematous. There is
evidence that suggests that exposure to these possible risk factors
may worsen the symptoms of systemic lupus erythematous (i.e.,
exposure to ultraviolet radiation through sunlight) but no conclusive
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evidence that they increase the risk of developing SLE or cause the
disease.9
The relationship between vitamin D and SLE is an example of the
complexity of the cause-effect relationship of environmental exposures
and SLE. Sunlight can aggravate the symptoms of SLE, often causing
what is commonly called a flare. But adequate exposure to sunlight is
needed for dermal production of vitamin D. Vitamin D may be
immunosuppressive; and, people who have SLE often have low levels
of vitamin D. Several small studies have shown that vitamin D
supplementation is helpful for people who have SLE,19,20 but the
primary question remains unanswered: are low levels of vitamin D a
cause or effect of SLE?9,21 The topic of vitamin D and SLE will be
discussed in more detail later in this learning module.
Immune Response of SLE
An in-depth discussion of the complex immune response of SLE is
beyond the scope of this course. The result of this immune system
response is the production of auto-antibodies (which are active against
the organism’s own tissues) and immune complexes (a combination of
a disease-causing antigen and its corresponding antibody) that bind
to, inflame, and damage specific tissues and organs, producing the
signs, symptoms and complications of SLE.1
Clinical Presentation Of SLE
Systemic lupus erythematosus can run a varied clinical course, ranging
from a relatively benign illness to a rapidly progressive disease with
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fulminant organ failure and death.24 The onset of SLE is usually during
the reproductive years of women, but late onset SLE does occur and
the disease affects children, as well.
There are three patterns of clinical presentation of SLE: chronic,
relapsing-remitting, and remission.25 Most patients have some
symptoms most of the time. Complete or prolonged remissions are
rare and uncommon, respectively;1,25,26 the median duration of periods
of remission has been reported to be three months.25 Additionally, the
pattern of disease activity that is seen in the first few years after onset
of SLE will tend to prevail.27
The clinical picture of SLE and the
severity of the signs and symptoms
are quite variable. Many patients
have dermatologic, mild
hematologic, musculoskeletal, and
serological effects. While others
have predominantly central
nervous system and renal
problems, and the disease is mild
in some cases and severe in
others.1,27 Disease presentation has also been reported as different
between women and men.28
No two cases of SLE 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 SLE have mild disease
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characterized by episodic flares, when signs and symptoms worsen for
a while, and that 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.
Systemic lupus erythematosus 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 SLE patient illustrates the way the disease is not
always recognized immediately:30
“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 antidepressants and I kept refusing even
though I was beginning to think that maybe I could use some.
So, I stayed away from the doctor for quite some time.”
The common signs and symptoms and estimations of their frequency
in patients who have SLE are listed in Table 1.1
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Table 1: Signs and Symptoms of SLE
Anorexia, fatigue, fever, malaise, weight loss: 95%
Cutaneous, i.e., Malar rash, photosensitivity: 80%
Hematologic, i.e., anemia, leukopenia: 85%
Musculoskeletal, i.e., arthralgias and myalgias: 95%
Neurologic: 60%
Systemic lupus erythematosus essentially affects every organ system.
A brief review of organ system effects is provided below.
Cardiac Disease
Cardiac disease is common in patients who have systemic lupus
erythematous, and the conducting system, the coronary arteries, the
myocardium, the pericardium, and the valves may be affected.27,31,32
Pericarditis is the most common cardiac abnormality caused by
SLE,1,27,32 and conduction defects and valvular disease may occur as
well. Cardiovascular disease is a major cause of morbidity in SLE
patients.33 SLE increases the risk of coronary artery disease and
myocardial infarction;32,34,35 and, cardiovascular disease is the most
common cause of mortality in patients who have SLE.36
Cutaneous Lesions
Systemic lupus erythematosus almost always affects skin and mucous
membranes. A full discussion of cutaneous lesions that may be caused
by SLE would be quite lengthy, and is not included here. Some of the
common cutaneous signs of SLE are highlighted, which include:
1) Alopecia, 2) Malar rash (commonly called a butterfly rash) seen as a
red area over the cheeks and the nose, 3) Oral and nasal ulcers, and
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4) Photosensitivity.1,27 Cutaneous lesions are less common in late-onset
systemic lupus erythematosus.37
Photosensitivity is defined by the American College of Rheumatology
as a “Skin rash as a result of unusual reaction to sunlight, by patient
history or physician observation.”38 It occurs in approximately 40-50%
of patients who have SLE,39 and patients develop a diffuse
erythematous or macular rash on the areas that have been exposed to
sunlight.40
Gastrointestinal
Gastrointestinal (GI) symptoms such as abdominal discomfort or pain,
diarrhea, anorexia, and nausea are quite common in patients who
have systemic lupus erythematous and most of these are caused by
adverse medication reactions, or bacterial or viral infections.41
However, there are GI complications such as intestinal pseudoobstruction, pancreatitis, peritonitis, and vasculitis that are
complications of SLE and these are associated with high rates of
morbidity and mortality.
Hematologic
Anemia, leukopenia, and thrombocytopenia are common complications
of SLE.27 These can be mild to severe,42 but severe hematologic
complications such as aplastic anemia, hemolytic anemia, and severe
thrombocytopenia are rare.27,43,44
Anemia (hemoglobin < 12 g/dL in women and < 13.5 g/dL in men)
affects approximately 50% of patients who have SLE,42 and it can be
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caused solely by the disease, blood loss as a side effect of medications
used to treat SLE, autoimmune hemolysis, or renal damage.45 Systemic
lupus erythematous can possibly affect iron hemostasis and
erythropoietin activity, both of which would explain why SLE alone
may cause anemia.42
Musculoskeletal
Arthralgias and myalgias affect over 90% of patients who have SLE,27,46
and acute arthritis is also very common in SLE patients.1,46 Arthralgias,
arthritis, and myalgias typically affect the hands, knees, and wrists
and cause pain, swelling and tenderness, and the symptoms are often
migratory and symmetrical. In most patients, these musculoskeletal
problems are intermittent and temporary; chronic arthritis has been
estimated to affect < 5% of patients who have SLE.46
Neurologic/Psychiatric
The American College of Rheumatology (ACR) has identified 19 specific
ways in which SLE can affect the nervous system, and collectively
these are called neuropsychiatric lupus syndrome (NPSLE).38,47
Neuropsychiatric lupus syndrome can result in a wide variety of signs,
symptoms, and complications. Patients who have NPSLE may develop
anxiety, delirium, depression, headaches, psychosis, cognitive
dysfunction, peripheral neuropathies, or seizures,1,27 and these can be
caused by the disease, treatments, or complications of SLE. Several of
these are discussed below.
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Cognitive Dysfunction
Cognitive dysfunction is a common feature of systemic lupus
erythematous,1,27 and it has been reported to affect 80-90% of patients
who have the disease.48 There is no typical pattern of SLE-associated
cognitive dysfunction, but many patients have impaired attention,
executive function, memory, and visuospatial ability48 and these may
not be evident until the patient is tested.48
Depression
Depression has been reported to affect almost 69% of patients who
have SLE and depression is positively associated with disease
activity.49
Seizures
Seizures are one of the ACR diagnostic criteria for systemic lupus
erythematous.38,50 Seizures have been reported to occur in 7-40% of
patients who have SLE;51 they may be evident at the time SLE is
diagnosed or much later, and there may be a single seizure or
recurrent seizures.52 Seizures are especially common in patients who
have an SLE-induced encephalopathy.53
Ocular
Systemic lupus erythematosus can affect any part of the eye. Common
ocular complications of SLE are keratoconjunctivitis sicca, mild
retinopathy, and renal artery or vein occlusion.54
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Pulmonary
A wide range of pulmonary complications can be caused by SLE,
including (but not limited to) interstitial lung disease, lupus
pneumonitis, pleuritis with or without pleural effusion, pulmonary
arterial hypertension, pulmonary embolism, and hemorrhage.1,27,55
Pleuritis is the most common of these,56,57 and it is the first
presentation in 5-10% of all patients.56 Pleuritis in SLE is more
common in men,56 and small pleural effusions can accompany
pleuritis.56,57
Renal
Lupus nephritis is an inflammatory disease of the kidneys that affects
approximately 60% of patients who have systemic lupus
erythematous.27,58,59 Lupus nephritis has six clinically recognized
presentations,58 it is a major contributor to the morbidity and mortality
of SLE and in approximately 5-10% of patients it can progress to endstage renal disease.58 When the onset is soon after the diagnosis of
SLE, the prognosis is poor. Remissions of lupus nephritis do occur but
the relapse rate is very high.
Mortality Rate of SLE
The five-year survival rate for SLE has improved over the past several
decades and it is now estimated to be > 90%.24 However, patients who
have SLE have an increased rate of mortality60,61 estimated to be two to
five times that of the general population.24 Late onset of the disease,
lower socioeconomic status, male gender, African American and
Hispanic ethnicity, high disease activity, poor disease control, presence
of antiphospholipid antibodies, antiphospholipid antibody syndrome,
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and prolonged use of corticosteroids all increase the risk of dying from
SLE,24,60,61 and the primary causes of death from SLE are cardiovascular
disease and infections.60,61
Systemic Lupus Erythematosus Disease Progression
For many patients who have SLE the disease is typified by periods of
remission and exacerbation. These exacerbations are called flares and
they are a characteristic feature of SLE; between 65-70% of all
patients who have SLE experience flares.62 A flare can be caused by
medication changes, pregnancy, exposure to sunlight, medications, or
stress, and the signs and symptoms can involve any organ system.
The duration and severity of a flare cannot be predicted;24,29 there are
no clinical or laboratory factors that can consistently predict when and
to whom a flare may occur.63,64 Patients with early onset disease, lupus
nephritis, or who are on immunosuppressive therapy may have a
greater risk of flares.63
There are no universally used definitions of an SLE flare,24 but
clinicians often categorize them based on clinical and laboratory
findings. Examples are provided below.24
Mild SLE
Mild SLE includes arthralgias, fatigue, low-grade fever, and a malar
rash. Treatment or an adjustment to current therapy may be needed
or a watchful waiting approach can be used. For mild to moderate
pain, a non-steroidal anti-inflammatory such as ibuprofen may
effective. If the flare causes more intense pain, a more potent opioid
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analgesic such as hydrocodone, oxycodone, or fentanyl may be
necessary.
Moderate SLE
The symptoms are more intense and extensive, i.e., the patient may
have pleuritic chest pain or a pleural effusion. Treatment and/or
adjustment to the current therapy is needed.
Severe SLE
Severe SLE includes lupus nephritis or infection of the kidneys.
SLE and Pregnancy
Systemic lupus erythematosus can have adverse effects on pregnant
women and the fetus, and pregnancy can have a negative impact on
SLE activity. Pregnancy and SLE increases the risk of miscarriage,
stillbirth, pre-term delivery, and preeclampsia, and infants born to a
mother who has SLE may develop neonatal lupus syndrome.65-67
Pregnancy complications are more likely if the mother has
hypertension, lupus nephritis, or secondary antiphospholipid syndrome
(a condition in which antibodies are produced that induce a
hypercoaguable state where the cause is unknown (primary) or due to
an existing condition (secondary)). Disease activity before and during
pregnancy also increases the risk for complications.66
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Diagnosis Of SLE
The diagnosis of SLE is difficult. In
the early stages the symptoms are
non-specific. There is no definitive
test for SLE; the clinical presentation
is very heterogeneous, and SLE can
be easily mistaken for an infectious,
malignant, or other autoimmune
disease.68,69 These issues are
especially important because a delay
in diagnosis has been associated
with a worse prognosis69 and delays are common.
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.70 This quote from a SLE
patient illustrates the way the disease is not always recognized
immediately.30
“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 antidepressants and I kept refusing even
though I was beginning to think that maybe I could use some.
So, I stayed away from the doctor for quite some time.”
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Most sources list the ACR classification criteria as the tool to be used
for diagnosing SLE. There are 11 criteria and a patient must have four
to be considered as having SLE. However, the criteria were developed
as a way of classifying SLE, not diagnosing the disease. The criteria
are very sensitive and specific71 and they are widely used. While
patients may not meet the criteria they may still have SLE68 and the
ACR criteria do not reflect all the signs and symptoms of SLE.
The Systemic Lupus International Collaborating Clinics (SLICC) has
developed classification criteria that some researchers have found to
be useful for diagnosing SLE, and the ACR and SLICC criteria are often
used together.39,72
Table 2: ACR Classification Criteria for SLE
1. Malar rash: Fixed erythema, flat or raised, over the malar
eminences, tending to spare the nasolabial folds.
2. Discoid rash: Erythematous raised patches with adherent keratotic
scaling and follicular plugging; atrophic scarring may occur in older
lesions.
3. Photosensitivity: Skin rash as a result of unusual reaction to
sunlight, by patient history or physician observation.
4. Oral ulcers: Oral or nasopharyngeal ulceration, usually painless,
observed by a physician.
5. Arthritis: Nonerosive arthritis involving 2 or more peripheral joints,
characterized by tenderness, swelling, or effusion.
6. Serositis: Pleuritis - convincing history of pleuritic pain or rub heard
by a physician or evidence of pleural effusion OR pericarditis documented by ECG or rub or evidence of pericardial effusion.
7. Renal disorder: Persistent proteinuria greater than 0.5 grams per
day or greater than 3+ if quantitation not performed OR cellular
casts - may be red cell, hemoglobin, granular, tubular, or mixed.
8. Neurologic disorder: Seizures - in the absence of offending drugs or
known metabolic derangements; i.e., uremia, ketoacidosis, or
electrolyte imbalance OR psychosis - in the absence of offending
drugs or known metabolic derangements, i.e., uremia, ketoacidosis,
or electrolyte imbalance.
9. Hematologic disorder: Hemolytic anemia - with reticulocytosis, OR
leukopenia-less than 4,000/mm3 total on 2 or more occasions, OR
lymphopenia - less than 1,500/mm3 on 2 or more occasions, OR
thrombocytopenia - less than 100,000/mm3 in the absence of
offending drugs.
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10.Immunologic: Anti-DNA: Antibody to native DNA in abnormal titer,
OR Anti-Sm: Presence of antibody to Sm nuclear antigen, OR
Positive finding of antiphospholipid antibodies on;
11.An abnormal level of serum antibodies on IGG or IGM on
anticardiolipin antibodies.
12.A positive test for lupus anticoagulant using a standard method, or
A false positive serologic test for least 6 months and confirmed by
Treponema pallidium immobilization or fluorescent treponemal
antibody absorption test.
13.Positive antinuclear antibody: An abnormal titer of antinuclear
antibody by immunofluoresence or an equivalent assay at any point
in time and in the absence of drugs.
Tests that may be used to aid in diagnosing SLE would include a
complete blood count (CBC) and measurement of antinuclear antibody
measurement, anti-double-stranded deoxyribonucleic acid (dsDNA),
complement levels (C3 and C4), renal biopsy, Chest CT scan,
C-reactive protein (CRP), erythrocyte sedimentation rate (ESR), blood
urea nitrogen (BUN) and creatinine measurements, urinalysis, urine
protein and creatinine, estimated glomerular filtration rate (GFR),
12-lead ECG, renal ultrasound, joint X-rays, and a comprehensive
metabolic panel (CMP).
Pharmacological Treatment Of SLE
There is no cure for SLE but pharmacological treatment decreases
disease activity, relieves symptoms, and can prevent organ damage.
Drug therapy for SLE is challenging. The disease affects multiple organ
systems and each patient has a unique presentation. There are periods
of exacerbation and remission, and the disease may progress over
time.
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Therapy decisions are driven by clinical and laboratory factors, and an
example of what drugs to use and when to use them is provided by
Wallace and paraphrased here.24
Mild SLE
Patients have joint, muscle, and skin issues only. Use an antimalarial,
with or without a non-steroidal anti-inflammatory, and/or a shortterm, low-dose course of a glucocorticoid.
Moderate SLE
These patients have organ involvement but no life-threatening signs or
symptoms. Use an antimalarial drug, with or without a non-steroidal
anti-inflammatory and a short-term, low-dose course of a
glucocorticoid. Another immunosuppressive such as azathioprine or
methotrexate is often needed.
Severe SLE
Use a short-term course of high doses of a glucocorticoid, given
intravenously. The dose is relatively high but the duration is
comparatively short and this approach is often called pulse therapy.
Another immunosuppressive can be used, as well. Antimalarial drugs
should be continued.
Antimalarials
The antimalarials chloroquine and hydroxychloroquine have antiinflammatory and immunologic properties. They are the mainstay of
drug therapy for patients who have SLE and all patients who have SLE
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should be receiving one of these medications.24,73 The antimalarials
have been shown to be effective for relief of the dermal,
musculoskeletal, and generalized symptoms of SLE;24 and there is
evidence that suggests they reduce the risk of developing
cardiovascular disease, diabetes, and thrombotic events,24,74,75 prevent
flares, and protect against organ damage and mortality.24,76
Hydroxychloroquine is the preferred drug but if it is not tolerated then
chloroquine can be used.72 The antimalarials are effective but the
optimal benefit may take several months to achieve.72
Chloroquine and hydroxychloroquine are usually well tolerated.
Gastrointestinal and dermatological side effects (i.e., abdominal
cramping, diarrhea, nausea, pruritus, rashes, and skin dryness) are
common and should stop once the drug has been discontinued.75
Serious side effects such as cardiomyopathy, deafness, seizures, and
toxic epidermal necrolysis have been reported but occur rarely.
Perhaps the most feared adverse effect of these drugs is retinopathy.
Retinopathy caused by chloroquine or hydroxychloroquine is
irreversible and it can cause vision loss.75 The onset can occur long
after the medications have been discontinued and it can progress after
discontinuation of therapy.75 The risk of retinopathy is directly related
to the total dose and the duration of use,75,77 and the risk has been
estimated to be < 1% with five years of therapy, < 2% with 10 years
of therapy; and, almost 20% after 20 years of therapy.77 The
maximum daily dose of hydroxychloroquine is ≤ 5.0 mg/kg (real
weight) and for chloroquine, ≤2.3 mg/kg (real weight).
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The American Academy of Ophthalmology recommends that patients
being treated with these medications should have a baseline fundus
examination to rule out pre-existing maculopathy and annual
screening should be done every five years for patients who are taking
acceptable doses and do not have risk factors of high dose, long
duration of therapy, renal disease, or concomitant use of tamoxifen.77
Glucocorticoids
The glucocorticoids may be used in place of the antimalarials or with
the anti-malarial medication. The degree of disease activity and the
patient’s clinical condition will determine how and when these drugs
are used, at what dose and for how long.24
Example: The antimalarials have a delayed onset and a low dose of
prednisone (≤ 7.5 mg a day), and can be used until the full effect of
chloroquine or hydroxychloroquine has been reached. For example, if a
patient has severe symptoms and major organs are affected then high
doses of glucocorticoids given intravenously for several days, along
with other immunosuppressive drugs, may be used.24
Prescribing glucocorticoids for patients who have systemic lupus
erythematous has been standard care for decades but these drugs,
although commonly used and relatively benign in most cases, should
not be considered routine.
First, the use of these glucocorticoids for this patient population has
been described as more of an art than science as the specifics of
glucocorticoid dosing for SLE patients are not evidence-based.73,78,79
Second, long-term use (i.e., five years or longer) of glucocorticoids
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can cause serious harm like avascular osteonecrosis, cataracts,
diabetes mellitus, hypertension, and osteoporotic fractures,78-80 and a
significant amount of the damage associated with SLE is caused by
chronic use of glucocorticoids.81 This can be avoided by using doses of
prednisone or an equivalent drug of < 7.5 mg/day, using IV pulse
therapy instead of high oral doses when possible, and the early use of
other immunosuppressive drugs, and, making sure the patient is
prescribed an antimalarial.79
Immunosuppressive Drugs
If a patient has not responded well to chloroquine or
hydroxychloroquine, or if he/she needs high doses and/or prolonged
treatment with glucocorticoids, another immunosuppressive drug such
as azathioprine, cyclophosphamide, methotrexate, or mycophenolate
should be prescribed.72,73 Side effects of these medications are usually
minor. Wallace (2015) advises the following.24
•
Patients with moderate SLE activity often need a steroid-sparing
drug such as azathioprine or methotrexate to control symptoms.
•
Patients who have severe or life-threatening SLE are usually
treated with corticosteroids, alone or in combination with
another immunosuppressive drug such mycophenolate, or
cyclophosphamide.
There is some evidence that these medications can be helpful in
treating nonspecific SLE disease activity and decreasing the need for
steroids,82-84 and their use is recommended by authoritative sources.
However, the application of azathioprine, cyclophosphamide,
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methotrexate, and mycophenolate for patients who have nonspecific
SLE activity is like that of glucocorticoids; they are widely prescribed
but the evidence is slim relative to how and when to use them and the
dose that should be given.84,85
Biologic Agents
Biologic agents such as belimumab, cyclosporine, or rituximab can be
prescribed if a patient has not responded to antimalarials and
glucocorticoids.24 These medications can improve clinical and
laboratory aspects of SLE and improve quality of life but the research
on their use is limited.86-90
Non-Steroidal Anti-Inflammatories
Non-steroidal anti-inflammatories (NSAIDs) can be used to treat mild
systemic lupus erythematosus disease activity. There is very little data
on their effectiveness for this application,73 and the side effects and
risks associated with these drugs, i.e., GI disturbances and renal
damage, would require that they be used cautiously.
Pharmacological Treatment During Pregnancy
The correct timing of a pregnancy, i.e., a period in which there is no
SLE flare, proper counseling, risk assessment, and the right treatment
can ensure a safe pregnancy and delivery for a woman who has
systemic lupus erythematosus. Poor pregnancy outcomes are more
likely if the mother has hypertension, lupus nephritis, or secondary
antiphospholipid syndrome. High disease activity before and during
pregnancy also increases the chances of complications,65 and an
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assessment for these risk factors (and nonspecific ones such diabetes
mellitus and thyroid disease) should be done.91
Pharmacological treatment during a pregnancy is, for the most part,
no different than it is for a non-pregnant woman. The antimalarials
and glucocorticoids in the proper dose should be continued; both have
been shown to be safe during pregnancy and discontinuing the
antimalarials increases the risk for a flare.91 For detailed information
about pharmacological treatment during pregnancy and systemic lupus
erythematosus, the reader is referred to Lazzaroni, et al. (2016).91
Prevention: Self-Care And Lifestyle
Non-pharmacological therapies have been investigated for their
effectiveness to reduce the risk of flares, manage disease activity, and
prevent complications. Unfortunately, this aspect of SLE care has not
been extensively studied and many of the research findings are
contradictory and inconclusive. Despite this fact, many lifestyle
interventions have value in and of themselves and a healthy lifestyle
should be encouraged in patients who have SLE.
Dietary Interventions
Dietary interventions with beta
carotene, omega-3
polyunsaturated acids, vitamin B6,
phosphorus, and other nutrients
and specific diets (i.e., restricted
calorie, low glycemic index) have
not been shown to be consistently
effective for reducing disease
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activity and dependence on medications, but some studies have found
that supplementation can reduce fatigue and decrease the incidence of
cardiovascular disease in SLE patients.92-96
Exercise
Recent reviews of the literature by Rodriguez Huerta, et al. (2016) and
del Pino-Sedeño, et al. (2016) found contradictory and inconsistent
evidence that exercise had specific benefits for patients who had
systemic lupus erythematosus.93,94 However, Yelnik, et al. (2016) did
find that few patients who had SLE were aware that the disease
increased the risk of developing atherosclerosis and cardiovascular
disease (CVD)96 and an exercise program can help reduce CVD risk
factors in this patient population.
Smoking Cessation
Stopping smoking can improve quality of life and reduces disease
activity.93
Sunlight Avoidance
Patients who have systemic lupus erythematosus should avoid direct
exposure to sunlight and use sunblock.24
Vitamin D Supplementation
Many patients who systemic lupus erythematosus are vitamin D
deficient or have a vitamin D insufficiency,97 and there is some
evidence that low levels of vitamin D are harmful to patients who have
SLE.98 Providing vitamin D supplementation for patients who have SLE
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has produced inconsistent results: some researchers have found a
benefit19,20,99 while others have not,95,100 and there is no consensus on
guidelines for when and for whom vitamin D supplementation should
be given.98
Coping And Support For SLE Patients
Individuals with SLE are likely to
have a range of painful feelings
about their condition, from fear to
extreme frustration. The challenges
of living with SLE increase the risk of
depression and related mental
health problems, such as anxiety,
stress and low self-esteem. Ways to
potentially help those with SLE and families cope are further discussed
below.
Education About Lupus
Individuals diagnosed with SLE should be encouraged to write down
questions about their disease to ask at their appointments with a
medical clinician. They should ask clinicians for reputable sources of
further information. The more individuals with lupus know about the
disease and prognosis, as well as recommended treatment, the more
confident they feel in their treatment choices. Organizations that
provide SLE education for clinicians to recommend to SLE patients are
highlighted below.

The Lupus Foundation of America – www.lupus.org
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
Lupus Research Institute – www.lupusresearchinstitute.org

S.L.E. Lupus Foundation – www.lupusny.org

Lupus Research Alliance - http://www.lupusresearch.org/

Lupus International - www.lupusinternational.com

Molly’s Fund – www.mollysfund.org
Family and Friends
When a person has been diagnosed with SLE, it is important they are
encouraged to reach out to friends and family to explain ways support
can be provided during flares. SLE can be frustrating for loved ones
because they usually cannot see it and the person may not appear
sick. They cannot tell if the individual is having a good day or a bad
day unless they communicate how they are feeling. Those with SLE
should be open about what they are feeling so that friends and family
know what to expect.
Peer Support, Networking and Quality of Life
Living with SLE can have profound effects on a person’s mental and
emotional wellbeing. Patients are likely to experience mental and
physical problems such as difficulty sleeping or with their focus and
concentration. 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, work
and activity limitations, pain, fatigue and other physical symptoms,
social isolation, uncertainty about the future, and difficulty with family
relationships.
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Summary
Systemic lupus erythematosus can cause damage to essentially every
organ system, and the pathogenesis of SLE is a complex, and
incompletely understood process involving genetic, environmental, and
immunologic factors. There is no cure for patients with SLE and these
patients have a significantly higher risk for early mortality.
Although there is no cure for SLE, symptomatic therapy for SLE has
been effective and advances in treatment have dramatically increased
survival rates. Pharmacological treatments are used to mitigate
disease activity, relieve symptoms, and prevent organ damage. Nonpharmacological therapies have also been investigated for their
effectiveness to reduce the risk of flares, to manage disease activity,
and to prevent complications. Lifestyle changes such as cessation of
smoking, diet and exercise have been beneficial for some patients.
Research in all these areas involving SLE continues with the goals of
controlling symptoms, improving quality of life and reducing early
mortality.
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1.
Systemic lupus erythematosus (SLE) is a rare autoimmune
disease, chronic in nature, that primarily affects
a.
b.
c.
d.
2.
Native Americans.
women of childbearing age.
elderly Caucasian women and men.
post-menopausal women.
True or False: Systemic lupus erythematous is an
autoimmune disease caused by genetic predisposition and
exposure to environmental factors such as nutrition,
infection, and certain chemicals.
a. True
b. False
3.
Environmental risk factors that definitively cause systemic
lupus erythematous include
a.
b.
c.
d.
4.
A 2014, meta-analysis of risk factors for systemic lupus
erythematosus found an increased risk for SLE associated
with
a.
b.
c.
d.
5.
post-menopausal hormone therapy.
cigarette smoking.
use of oral contraceptives.
None of the above
the use of oral contraceptives.
post-menopausal hormone therapy.
pregnancy.
Answers a., and b., above
Which of the following statements is/are true about
systemic lupus erythematosus?
a.
b.
c.
d.
More than 90% of patients with SLE are women
SLE is less common in African Americans
SLE is more common in Caucasians than Native Americans.
All of the above
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6.
_______________ is the most common cause of mortality
in patients who have systemic lupus erythematosus.
a.
b.
c.
d.
7.
Thrombocytopenia
Kidney disease
Cardiovascular disease
Lupus pneumonitis
True or False: Research has identified susceptibly genes for
systemic lupus erythematosus, and these genetic
variations appear to account for most of the heritable
cases of SLE.
a. True
b. False
8.
__________________, commonly called a butterfly rash,
is a cutaneous sign of SLE that manifests as a red area over
the cheeks and the nose.
a.
b.
c.
d.
9.
Malar rash
Alopecia
Dermatomyositis
Sjögren syndrome
Systemic lupus erythematosus may only cause damage to
which of the following systems or organs?
a.
b.
c.
d.
Every organ system
The skin
Musculoskeletal systems
The kidneys
10. Gastrointestinal (GI) symptoms such as abdominal pain,
diarrhea, anorexia, and nausea are quite common in
patients who have SLE and most of these are caused by
a.
b.
c.
d.
adverse medication reactions.
bacterial infections.
viral infections.
All of the above
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11. True or False: Systemic lupus erythematosus primarily
affects women, but when men are diagnosed with the
disease, the disease presentation in men is the same as
with women.
a. True
b. False
12. In most patients, musculoskeletal problems such as
arthralgias, arthritis, and myalgias, are
a.
b.
c.
d.
chronic.
often static and asymmetrical.
intermittent and temporary.
unremitting.
13. Cognitive dysfunction, such as impaired attention or
impaired memory, associated with SLE has been reported
to affect ________ of patients who have the disease.
a.
b.
c.
d.
10%
half
80-90%
50-60%
14. ________________ is the most common cardiac
abnormality caused by systemic lupus erythematosus.
a.
b.
c.
d.
Conduction defects
Valvular disease
Thrombocytopenia
Pericarditis
15. Seizures are especially common in lupus patients who have
a.
b.
c.
d.
cognitive dysfunction.
an SLE-induced encephalopathy.
neuropsychiatric lupus syndrome (NPSLE).
Sjögren syndrome.
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16. True or False: Systemic lupus erythematous may affect iron
hemostasis and erythropoietin activity, both of which
would explain why SLE alone may cause anemia.
a. True
b. False
17. Lupus nephritis is an inflammatory disease of the
_______________ that affects approximately 60% of
patients who have systemic lupus erythematosus.
a.
b.
c.
d.
heart
lungs
kidneys
central nervous system
18. A lupus patient with pleuritic chest pain or a pleural
effusion is probably experiencing
a.
b.
c.
d.
a mild flare.
a moderate flare.
a severe flare.
lupus nephritis.
19. Which of the following drugs has been implicated as a
cause of drug-induced systemic lupus erythematosus?
a.
b.
c.
d.
Procainamide
Hydrocodone
Fentanyl
Ibuprofen
20. A woman with systemic lupus erythematosus who becomes
pregnant has an increased risk of
a.
b.
c.
d.
neuropsychiatric lupus syndrome (NPSLE).
Sjögren syndrome.
preeclampsia.
antiphospholipid syndrome.
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21. True or False: There is no cure for systemic lupus
erythematous but pharmacological treatment decreases
disease activity, relieves symptoms, and can prevent organ
damage.
a. True
b. False
22. Tests that may be used to aid in diagnosing systemic lupus
erythematosus include a
a.
b.
c.
d.
complete blood count (CBC)
renal biopsy
CT scan of the chest
All of the above
23. ______________ have been shown to be effective relief
for the dermal, musculoskeletal, and generalized
symptoms of SLE.
a.
b.
c.
d.
Anti-epileptics
Statins
Antimalarials
Proton pump inhibitors
24. Prescribing _______________ for systemic lupus
erythematosus patients has been standard care for
decades but these drugs should not be considered routine.
a.
b.
c.
d.
antimalarials
statins
anti-epileptics
glucocorticoids
25. Pharmacological treatment of lupus during a pregnancy is
as follows:
a.
b.
c.
d.
antimalarials should be replaced by glucocorticoids.
glucocorticoids should be discontinued.
antimalarials should be continued.
non-pharmacological therapies must be used.
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26. True or False: Patients who have systemic lupus
erythematosus are encouraged to increase their exposure
to sunlight because Vitamin D is known to mitigate the
symptoms of SLE.
a. True
b. False
27. Dietary interventions with beta carotene, omega-3
polyunsaturated acids, vitamin B6, phosphorus, and other
nutrients and specific diets
a.
b.
c.
d.
have been a consistently effective treatment for SLE.
are a replacement for pharmacological treatment.
can reduce fatigue and decrease cardiovascular disease.
All of the above
28. Patients with systemic lupus erythematosus may reduce
the risk of developing _______________________ by
following an exercise program
a.
b.
c.
d.
hematologic problems
renal disease
Sjögren syndrome
atherosclerosis and cardiovascular disease
29. Providing vitamin D supplementation for patients who have
systemic lupus erythematosus
a.
b.
c.
d.
will mitigate the symptoms.
may or may not help.
will exacerbate the symptoms.
has no effect whatsoever.
30. SLE can be frustrating for loved ones because
a.
b.
c.
d.
the symptoms are always visible.
lupus patients are not open about their disease.
the person may not appear sick.
All of the above
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CORRECT ANSWERS:
1.
Systemic lupus erythematosus (SLE) is a rare autoimmune
disease, chronic in nature, that primarily affects
b. women of childbearing age.
p. 5: “Systemic lupus erythematosus (SLE) is a rare
autoimmune disease, chronic in nature, which primarily
affects women of childbearing age.”
2.
True or False: Systemic lupus erythematous is an
autoimmune disease caused by genetic predisposition and
exposure to environmental factors such as nutrition,
infection, and certain chemicals.
a. True
p. 5: “Systemic lupus erythematosus can cause damage to
essentially every organ system, and the pathogenesis of SLE
is a complex, and incompletely understood process involving
genetic, environmental, and immunologic factors.”
pp. 5-6: “…Long, et al., (2106) wrote: ‘Both genetic
predisposition and environmental factors such as nutrition,
infection, and chemicals are implicated in the pathogenic
process of autoimmunity, however, how much and by what
mechanisms each of these factors contribute to the
development of autoimmunity remain unclear.’”
3.
Environmental risk factors that definitively cause systemic
lupus erythematous include
a.
b.
c.
d.
post-menopausal hormone therapy.
cigarette smoking.
use of oral contraceptives.
None of the above
p. 6: “There are many environmental exposures that may be
risk factors for the development of systemic lupus
erythematous. Not surprisingly, the evidence that supports a
causative role for these environmental risk factors in the
development of SLE is sometimes strong and at other times
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weak and conflicting. Environmental risk factors that are likely
to increase the risk for SLE are cigarette smoking, female
gender, oral contraceptives and postmenopausal hormone
therapy, silica exposure, drug use, and other environmental
factors, which are further discussed below.”
4.
A 2014, meta-analysis of risk factors for systemic lupus
erythematosus found an increased risk for SLE associated
with
b. post-menopausal hormone therapy.
p. 7: “Oral contraceptives have long been thought of a risk
factor for developing SLE. However, the evidence linking oral
contraceptives and SLE is conflicting, and a recent (2014)
meta-analysis did not find an association between the use of
oral contraceptives and an increased risk for SLE. The same
meta-analysis did find an association between postmenopausal hormone therapy and an increased risk for SLE.
Female sex hormones influence immune system function and
this may explain the association between therapeutic use of
hormones, female gender and SLE.”
5.
Which of the following statements is/are true about
systemic lupus erythematosus?
a. More than 90% of patients with SLE are women
p. 5: “More than 90% of patients who have SLE are women.”
6.
_______________ is the most common cause of mortality
in patients who have systemic lupus erythematosus.
c. Cardiovascular disease
p. 12: “Cardiovascular disease is a major cause of morbidity
in SLE patients; SLE increases the risk of coronary artery
disease and myocardial infarction; and, cardiovascular
disease is the most common cause of mortality in patients
who have SLE.”
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7.
True or False: Research has identified susceptibly genes for
systemic lupus erythematosus, and these genetic
variations appear to account for most of the heritable
cases of SLE.
b. False
p. 6: “… however, although research has identified susceptibly
genes for SLE, it is unclear how and for whom these result in
the active disease, and these genetic variations appear to
account for only a small part of the heritability of SLE.”
8.
__________________, commonly called a butterfly rash,
is a cutaneous sign of SLE that manifests as a red area over
the cheeks and the nose.
a. Malar rash
p. 12-13: “Some of the common cutaneous signs of SLE are
highlighted, which include: 1) Alopecia, 2) Malar rash
(commonly called a butterfly rash) seen as a red area on over
the cheeks and the nose, 3) Oral and nasal ulcers; and,
4) Photosensitivity.”
9.
Systemic lupus erythematosus may only cause damage to
which of the following systems or organs?
a. Every organ system
p. 5: “Systemic lupus erythematosus can cause damage to
essentially every organ system, and the pathogenesis of SLE
is a complex, and incompletely understood process involving
genetic, environmental, and immunologic factors.”
10. Gastrointestinal (GI) symptoms such as abdominal pain,
diarrhea, anorexia, and nausea are quite common in
patients who have SLE and most of these are caused by
a.
b.
c.
d.
adverse medication reactions.
bacterial infections.
viral infections.
All of the above
p. 13: “Gastrointestinal (GI) symptoms such as abdominal
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discomfort or pain, diarrhea, anorexia, and nausea are quite
common in patients who have systemic lupus erythematous
and most of these are caused by adverse medication
reactions or bacterial, or viral infections. However, there are
GI complications such as intestinal pseudo-obstruction,
pancreatitis, peritonitis, and vasculitis that are complications
of the disease of SLE and these are associated with high rates
of morbidity and mortality.”
11. True or False: Systemic lupus erythematosus primarily
affects women, but when men are diagnosed with the
disease, the disease presentation in men is the same as
with women.
b. False
p. 10: “The clinical picture of SLE and the severity of the
signs and symptoms are quite variable. Many patients have
dermatologic, mild hematologic, musculoskeletal, and
serological effects. While others have predominantly central
nervous system and renal problems, and the disease is mild
in some cases and severe in others. Disease presentation has
also been reported as different between women and men.”
12. In most patients, musculoskeletal problems such as
arthralgias, arthritis, and myalgias, are
c. intermittent and temporary
p. 14: “Arthralgias and myalgias affect over 90% of patients
who have SLE, and acute arthritis is also very common in SLE
patients…. In most patients, these musculoskeletal problems
are intermittent and temporary; chronic arthritis has been
estimated to affect < 5% of patients who have SLE.”
13. Cognitive dysfunction, such as impaired attention or
impaired memory, associated with SLE has been reported
to affect ________ of patients who have the disease.
c. 80-90%
p. 15: “Cognitive dysfunction is a common feature of systemic
lupus erythematous, and it has been reported to affect 8090% of patients who have the disease.”
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14. ________________ is the most common cardiac
abnormality caused by systemic lupus erythematosus.
d. Pericarditis
p. 12: “Pericarditis is the most common cardiac abnormality
caused by SLE, and conduction defects, valvular disease may
occur as well.”
15. Seizures are especially common in lupus patients who have
b. an SLE-induced encephalopathy.
p. 15: “Seizures are especially common in patients who an
SLE-induced encephalopathy.”
16. True or False: Systemic lupus erythematous may affect iron
hemostasis and erythropoietin activity, both of which
would explain why SLE alone may cause anemia.
a. True
p. 13: “Systemic lupus erythematous can possibly affect iron
hemostasis and erythropoietin activity, both of which would
explain why SLE alone may cause anemia.”
17. Lupus nephritis is an inflammatory disease of the
_______________ that affects approximately 60% of
patients who have Systemic lupus erythematosus (SLE).
c. kidneys
p. 16: “Lupus nephritis is an inflammatory disease of the
kidneys that affects approximately 60% of patients who have
systemic lupus erythematous.”
18. A lupus patient with pleuritic chest pain or a pleural
effusion is probably experiencing
b. a moderate flare.
p. 18: “Moderate SLE: The symptoms are more intense and
extensive, i.e., the patient may have pleuritic chest pain or a
pleural effusion. Treatment and/or adjustment to the current
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therapy is needed.”
19. Which of the following drugs has been implicated as a
cause of drug-induced systemic lupus erythematosus?
a. Procainamide
p. 8: “Commonly used drugs such as anti-epileptics, proton
pump inhibitors, and statins have been implicated as causes
of drug-induced SLE but aside from hydralazine and
procainamide, which have a 5-8% and 20% risk, respectively
of causing SLE, drug-induced SLE is rare.”
20. A woman with systemic lupus erythematosus who becomes
pregnant has an increased risk of
c. preeclampsia.
p. 18: “Pregnancy and SLE increases the risk of miscarriage,
stillbirth, pre-term delivery, and preeclampsia, and infants
born to a mother who has SLE may develop neonatal lupus
syndrome.”
21. True or False: There is no cure for systemic lupus
erythematous but pharmacological treatment decreases
disease activity, relieves symptoms, and can prevent organ
damage.
a. True
p. 21: “There is no cure for SLE but pharmacological
treatment decreases disease activity, relieves symptoms, and
can prevent organ damage.”
22. Tests that may be used to aid in diagnosing systemic lupus
erythematosus include a
a.
b.
c.
d.
complete blood count (CBC)
renal biopsy
CT scan of the chest
All of the above
p. 21: “Tests that may be used to aid in diagnosing SLE would
include a complete blood count (CBC) and measurement of
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antinuclear antibody measurement, anti-double-stranded
deoxyribonucleic acid (dsDNA), complement levels (C3 and
C4), renal biopsy, Chest CT scan, C-reactive protein (CRP),
erythrocyte sedimentation rate (ESR), blood urea nitrogen
(BUN) and creatinine measurements, urinalysis, urine protein
and creatinine, estimated glomerular filtration rate (GFR),
12-lead ECG, renal ultrasound, joint X-rays, and a
comprehensive metabolic panel (CMP).”
23. ______________ have been shown to be effective relief
for the dermal, musculoskeletal, and generalized
symptoms of SLE.
c. Antimalarials
p. 23: “The antimalarials have been shown to be effective
relief for the dermal, musculoskeletal, and generalized
symptoms of SLE and there is evidence that suggests they
reduce the risk of developing cardiovascular disease,
diabetes, and thrombotic events, prevent flares, and have a
protect against organ damage and mortality.”
24. Prescribing _______________ for systemic lupus
erythematosus patients has been standard care for
decades but these drugs should not be considered routine.
d. glucocorticoids
p. 24: “Prescribing glucocorticoids for patients who have
systemic lupus erythematous has been standard care for
decades but these drugs, although commonly used and
relatively benign in most cases, should not be considered
routine.”
25. Pharmacological treatment of lupus during a pregnancy is
as follows:
c. antimalarials should be continued.
p. 27: “Pharmacological treatment during a pregnancy is, for
the most part, no different than it is for a non-pregnant
woman. The antimalarials should be continued and
glucocorticoids in the proper dose should be, as well; both
have been shown to be safe during pregnancy and
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discontinuing the antimalarials increases the risk for a flare.”
26. True or False: Patients who have systemic lupus
erythematosus are encouraged to increase their exposure
to sunlight because Vitamin D is known to mitigate the
symptoms of SLE.
b. False
p. 28: “Patients who have systemic lupus erythematosus
should avoid direct exposure to sunlight and use sunblock.”
27. Dietary interventions with beta carotene, omega-3
polyunsaturated acids, vitamin B6, phosphorus, and other
nutrients and specific diets
c. can reduce fatigue and decrease cardiovascular disease.
p. 27: “Dietary interventions with beta carotene, omega-3
polyunsaturated acids, vitamin B6, phosphorus, and other
nutrients and specific diets (i.e., restricted calorie, low
glycemic index) have not been shown to be consistently
effective for reducing disease activity and dependence on
medications, but some studies have found that
supplementation can reduce fatigue and decrease the
incidence of cardiovascular disease in SLE patients.”
28. Patients with systemic lupus erythematosus may reduce
the risk of developing _______________________ by
following an exercise program
d. atherosclerosis and cardiovascular disease
p. 28: “However, Yelnik, et al., (2016) did find that few
patients who had SLE were aware that the disease increased
the risk of developing atherosclerosis and cardiovascular
disease (CVD) and an exercise program can help reduce CVD
risk factors in this patient population.”
29. Providing vitamin D supplementation for patients who have
systemic lupus erythematosus
b. may or may not help.
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p. 28-29: “Providing vitamin D supplementation for patients
who have SLE has produced inconsistent results: some
researchers have found a benefit while others have not, and
there is no consensus on guidelines for when and for whom
vitamin D supplementation should be given.”
30. SLE can be frustrating for loved ones because
c. the person may not appear sick.
p. 30: “SLE can be frustrating for loved ones because they
usually cannot see it and the person may not appear sick.”
References Section
The References below include published works and in-text citations of
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