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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. 1 nursece4less.com nursece4less.com nursece4less.com nursece4less.com 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. 2 nursece4less.com nursece4less.com nursece4less.com nursece4less.com 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. 3 nursece4less.com nursece4less.com nursece4less.com nursece4less.com 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 4 nursece4less.com nursece4less.com nursece4less.com nursece4less.com 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 5 nursece4less.com nursece4less.com nursece4less.com nursece4less.com 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 6 nursece4less.com nursece4less.com nursece4less.com nursece4less.com 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 7 nursece4less.com nursece4less.com nursece4less.com nursece4less.com 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 8 nursece4less.com nursece4less.com nursece4less.com nursece4less.com 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 9 nursece4less.com nursece4less.com nursece4less.com nursece4less.com 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 10 nursece4less.com nursece4less.com nursece4less.com nursece4less.com 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 11 nursece4less.com nursece4less.com nursece4less.com nursece4less.com 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 12 nursece4less.com nursece4less.com nursece4less.com nursece4less.com 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 13 nursece4less.com nursece4less.com nursece4less.com nursece4less.com 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. 14 nursece4less.com nursece4less.com nursece4less.com nursece4less.com 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 15 nursece4less.com nursece4less.com nursece4less.com nursece4less.com 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, 16 nursece4less.com nursece4less.com nursece4less.com nursece4less.com 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 17 nursece4less.com nursece4less.com nursece4less.com nursece4less.com 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 18 nursece4less.com nursece4less.com nursece4less.com nursece4less.com 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.” 19 nursece4less.com nursece4less.com nursece4less.com nursece4less.com 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. 20 nursece4less.com nursece4less.com nursece4less.com nursece4less.com 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. 21 nursece4less.com nursece4less.com nursece4less.com nursece4less.com 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 22 nursece4less.com nursece4less.com nursece4less.com nursece4less.com 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). 23 nursece4less.com nursece4less.com nursece4less.com nursece4less.com 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 24 nursece4less.com nursece4less.com nursece4less.com nursece4less.com 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, 25 nursece4less.com nursece4less.com nursece4less.com nursece4less.com 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 26 nursece4less.com nursece4less.com nursece4less.com nursece4less.com 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 27 nursece4less.com nursece4less.com nursece4less.com nursece4less.com 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 28 nursece4less.com nursece4less.com nursece4less.com nursece4less.com 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 29 nursece4less.com nursece4less.com nursece4less.com nursece4less.com 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. 30 nursece4less.com nursece4less.com nursece4less.com nursece4less.com 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. Please take time to help NurseCe4Less.com course planners evaluate the nursing knowledge needs met by completing the self-assessment of Knowledge Questions after reading the article, and providing feedback in the online course evaluation. Completing the study questions is optional and is NOT a course requirement. 31 nursece4less.com nursece4less.com nursece4less.com nursece4less.com 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 32 nursece4less.com nursece4less.com nursece4less.com nursece4less.com 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 33 nursece4less.com nursece4less.com nursece4less.com nursece4less.com 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. 34 nursece4less.com nursece4less.com nursece4less.com nursece4less.com 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. 35 nursece4less.com nursece4less.com nursece4less.com nursece4less.com 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. 36 nursece4less.com nursece4less.com nursece4less.com nursece4less.com 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 37 nursece4less.com nursece4less.com nursece4less.com nursece4less.com 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 38 nursece4less.com nursece4less.com nursece4less.com nursece4less.com 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.” 39 nursece4less.com nursece4less.com nursece4less.com nursece4less.com 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 40 nursece4less.com nursece4less.com nursece4less.com nursece4less.com 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.” 41 nursece4less.com nursece4less.com nursece4less.com nursece4less.com 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 42 nursece4less.com nursece4less.com nursece4less.com nursece4less.com 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 43 nursece4less.com nursece4less.com nursece4less.com nursece4less.com 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 44 nursece4less.com nursece4less.com nursece4less.com nursece4less.com 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. 45 nursece4less.com nursece4less.com nursece4less.com nursece4less.com 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 published works that are intended as helpful material for your further reading. 1. 2. 3. 4. Hahns BH. Systemic lupus erythematosus. In: Kasper D, Fauci A, Hauser S, Long D, Jameson JL, Loscalzo J, eds. Harrison’s Principles of Internal Medicine, 19th ed. New York, NY: McGrawHill;2015. Online edition, accessed November 3, 2016 from www.UCHC.edu. Ortona E, Pierdominici M, Maselli A, Veroni C, Aloisi F, Shoenfeld Y. Sex-based differences in autoimmune diseases. Ann Ist Super Sanita. 2016;52(2):205-212. Somers EC, Marder W, Cagnoli P, et al. Population-based incidence and prevalence of systemic lupus erythematosus: The Michigan Lupus Epidemiology and surveillance program. Arthritis Rheumatol. 2014;66(2):369-378. Jarukitsopa S, Hoganson DD, Crowson CS, et al. Epidemiology of systemic lupus erythematosus and cutaneous lupus erythematosus in a predominantly white population in the United States. Arthritis Care Res (Hoboken). 2015;67(6):817-828. 46 nursece4less.com nursece4less.com nursece4less.com nursece4less.com 5. 6. 7. 8. 9. 10. 11. 12. 13. 14. 15. 16. 17. Ungprasert P, Sagar V, Crowson CS, Incidence of systemic lupus erythematosus in a population-based cohort using revised 1997 American College of Rheumatology and the 2012 Systemic Lupus International Collaborating Clinics classification criteria. Lupus. 2016 Jun 30. pii: 0961203316657434. [Epub ahead of print] Long H, Yin H, Wang L, Gershwin ME, Lu Q. The critical role of epigenetics in systemic lupus erythematosus and autoimmunity. J Autoimmun. 2016;74:118-138. Gianfrancesco MA, Balzer L, Taylor KE, et al. Genetic risk and longitudinal disease activity in systemic lupus erythematosus using targeted maximum likelihood estimation. Genes Immun. 2016;17(6):358-362. Teruel M, Alarcón-Riquelme ME. The genetic basis of systemic lupus erythematosus: What are the risk factors and what have we learned. J Autoimmun. 2016;74:161-175. Barbhaiya M, Costenbader KH. Environmental exposures and the development of systemic lupus erythematosus. Curr Opin Rheumatol. 2016;28(5):497-505. Jiang F, Li S, Jia C, et al. Smoking and the risk of systemic lupus erythematosus: an updated systematic review and cumulative meta-analysis. Clin Rheumatol. 2015;34(11):1885-1892. Binder SR, Litwin CM. Anti-phospholipid antibodies and smoking: An overview. Clin Rev Allergy Immunol. 2016 Jul 5. [Epub ahead of print] Ghaussy NO, Sibbitt W Jr, Bankhurst AD, Qualls CR. Cigarette smoking and disease activity in systemic lupus erythematosus. J Rheumatol. 2003; 30(6):1215-1221. Moulton VR, Tsokos GC. Why do women get lupus? Clin Immunol. 2012;144(1):53-56. Tsokos GC. Systemic lupus erythematosus. N Engl J Med. 2011; 365(22):2110-2121 Rojas-Villarraga A, Torres-Gonzalez JV, Ruiz-Sternberg ÁM. Safety of hormonal replacement therapy and oral contraceptives in systemic lupus erythematosus: a systematic review and metaanalysis. PLoS One. 2014 Aug 19;9(8):e104303. doi: 10.1371/journal.pone.0104303. eCollection 2014. Marder W, Littlejohn EA, Somers EC. Pregnancy and autoimmune connective tissue diseases. Best Pract Res Clin Rheumatol. 2016;30(1):63-80. Pollard KM. Silica, silicosis, and autoimmunity. Front Immunol. 2016 Mar 11;7:97. doi: 10.3389/fimmu.2016.00097. eCollection 2016. 47 nursece4less.com nursece4less.com nursece4less.com nursece4less.com 18. 19. 20. 21. 22. 23. 24. 25. 26. 27. 28. Lucas CD, Amft N, Reid PT. Systemic lupus erythematosus complicating simple silicosis. Occup Med (Lond). 2014;64(5):387-390. Lima GL, Paupitz J, Aikawa NE, et al. 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[Epub ahead of print] Wallace DJ. Overview of the management and prognosis of systemic lupus erythematosus in adults. UpToDate. December 18, 2015. https://www.uptodate.com/contents/overview-of-themanagement-and-prognosis-of-systemic-lupus-erythematosusin-adults. Accessed November 6, 2016. Wilhelm TR, Magder LS, Petri M. Remission in systemic lupus erythematosus: durable remission is rare. Ann Rheum Dis. 2016 Aug 24. pii: annrheumdis-2016-209489. doi: 10.1136/annrheumdis-2016-209489. [Epub ahead of print] Steiman AJ, Urowitz MB, Ibañez D, Papneja A, Gladman DD. Prolonged clinical remission in patients with systemic lupus erythematosus. J Rheumatol. 2014;41(9):1808-1816. Gladman DD. Overview of the clinical manifestations of systemic lupus erythematosus in adults. UpToDate. September 20, 2015. http://www.uptodate.com/contents/overview-of-theclinicalmanifestations-of-systemic-lupus-erythematosus-inadults. Accessed November 4, 2016. Boodhoo KD, Liu S, Zuo X. 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A pilot study to determine if vitamin D repletion improves endothelial function in lupus patients. Am J Med Sci. 2015;350(4):302-307. 100. Andreoli L, Dall'Ara F, Piantoni S, et al. A 24-month prospective study on the efficacy and safety of two different monthly regimens of vitamin D supplementation in pre-menopausal women with systemic lupus erythematosus. Lupus. 2015;24(45):499-506. 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. 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