AUTHOR INFORMATION

Section 1 of 11

Authored by David W Lamont, DO, Clinical Assistant Professor, Department of Emergency Medicine, Chicago College of Osteopathic Medicine, St Francis Hospital

Coauthored by Mai Kim Lai, MD, Staff Physician, Department of Emergency Medicine, Sparrow Hospital, Michigan State University College of Human Medicine; Steven H Silber, DO, FACEP, Clinical Assistant Professor, Department of Emergency Medicine, Weill Medical College of Cornell University; Vice Chair, Department of Emergency Medicine, New York Methodist Hospital

David W Lamont, DO, is a member of the following medical societies: American College of Emergency Physicians, American Osteopathic Association, and Society for Academic Emergency Medicine

Edited by Richard S Krause, MD, Clinical Assistant Professor, Residency Program Director, Department of Emergency Medicine, State University of New York at Buffalo School of Medicine; Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine; Gino A Farina, MD, Program Director, Associate Professor of Clinical Emergency Medicine, Department of Emergency Medicine, Long Island Jewish Medical Center, Albert Einstein College of Medicine; John Halamka, MD, Chief Information Officer, CareGroup Healthcare System, Assistant Professor of Medicine, Department of Emergency Medicine, Beth Israel Deaconess Medical Center; Assistant Professor of Medicine, Harvard Medical School; and Charles V Pollack, Jr, MD, MA, FACEP, Professor, Department of Emergency Medicine, University of Pennsylvania College of Medicine; Chairman, Department of Emergency Medicine, Pennsylvania Hospital

Author's Email:	David W Lamont, DO
Editor's Email:	Richard S Krause, MD

eMedicine Journal, January 17 2006, VOLUME 7, Number 1

INTRODUCTION

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Background: Systemic lupus erythematosus (SLE) is an autoimmune disease involving multiple organ systems that is defined clinically and associated with antibodies directed against cell nuclei. Its multisystem manifestations and attendant complications from use of immunosuppressive agents make the diagnosis and management of this entity challenging.

Pathophysiology: Autoantibodies, circulating immune complexes, and T lymphocytes all contribute to the expression of disease. Organ systems affected include dermatologic, renal, central nervous system (CNS), hematologic, musculoskeletal, cardiovascular, pulmonary, the vascular endothelium, and gastrointestinal. The revised criteria for SLE must include 4 of the following at any time during a patient’s history (specificity 95% and sensitivity 75%):

• Malar rash

• Discoid rash

• Photosensitivity

• Oral ulcers

• Arthritis

• Serositis

• Renal disorder

• Neurologic disorder

• Hematologic disorder

• Immunologic disorder

• Antinuclear antibody

Frequency:

• In the US: Because of variable reporting, the overall prevalence ranges from 14.6-50.8 cases per 100,000. Incidence varies from 1.8-7.6 cases per 100,000 per year.

• Internationally: Incidence varies worldwide. In Northern Europe, it has been reported to be 40 cases per 100,000.

Mortality/Morbidity: Recent studies in Europe and Canada have shown a reduction in mortality in patients with lupus with 20-year survival rates close to 70% and 10-year survival rates ranging from 75-85%, with more than 90% of patients surviving more than 5 years.

• Early deaths usually are caused by active disease. Atherosclerosis is a leading cause in late deaths. Infection and nephritis are major causes of mortality in all stages of SLE.

• After dialysis or transplantation, a reduction in disease activity and flares has been reported.

• Thrombosis, often secondary to antiphospholipid syndrome, carditis, pneumonitis, pulmonary hypertension, stroke, myocardial infarction, and cerebritis cause severe morbidity and mortality.

Race: SLE is more common in blacks (1:250) than in whites (1:1000). However, all ethnic groups are susceptible.

Sex: Ninety percent of cases are in women. Also, women who are exposed to estrogen-containing oral contraceptives or hormone replacement have an increased risk of developing SLE. The sex distribution is more equal in those who develop SLE during childhood or when older than 50 years.

Age: Most (80%) cases have been reported to occur in women in their childbearing years.

CLINICAL

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History: Manifestations are protean, and the mean length of time between onset of symptoms and diagnosis is 5 years. The disease is characterized by exacerbations and remissions. Many women relate flares of their lupus to the postovulatory phase of the menstrual cycle, with resolution of symptoms at the time of menses.

• Systemic symptoms include a low-grade fever, fatigue, malaise, anorexia, nausea, and weight loss. Initial presentation may involve one or more organ systems.

• Arthralgias (53-95%) are the initial complaint in many patients. Often, the pain is out of proportion to physical findings.

• Malar, butterfly rash over the cheeks and bridge of the nose (55-90%) with photosensitivity to ultraviolet (UV) light has been reported (mostly in whites). It also often involves the chin and ears.

• Painful or painless ulcers in the nose and mouth are frequent complaints.

• CNS symptoms may range from mild cognitive dysfunction to a history of seizures (12-59%). Any region of the brain, meninges, spinal cord, and cranial and peripheral nerves can be involved. CNS events often occur when SLE is active in other organ systems. Intractable headaches and difficulties with memory and reasoning are the most common features of neurologic disease in patients with lupus.

• Psychiatric symptoms (high-dose steroids also can cause psychosis [5-37%]) - If the psychosis gets worse after stopping the steroid, it is most likely related to the disease process.

• Pleuritic pain (31-57%), dyspnea, cough, fever, and chest pain are important cardiopulmonary complaints.

• Patients may present with abdominal pain, diarrhea, and vomiting. Intestinal perforation and vasculitis are important diagnoses to exclude.

• A number of other symptoms can be elicited by history which can help identify other pathology, including the following:

• Stroke

• Pulmonary embolus

• Deep venous thrombosis (DVT)

• Acute ischemia

• Retinal vasculitis

Physical:

• Fever is a challenging problem in SLE. It can be a manifestation of active lupus or a representation of infection, malignancy, or a drug reaction. Temperature higher than 102°F should prompt a search for infection and may be a lower-grade temperature in patients on immunosuppressive agents.

• Malar rash is a fixed erythema sparing the nasolabial folds. It is a butterfly rash that can be flat or raised over the cheeks and bridge of the nose. It also often involves the chin and ears.

• Discoid rash occurs in 20% of patients with SLE and can be disfiguring secondary to scarring. It presents as erythematous patches with keratotic scaling over sun-exposed areas of the skin and may occur in the absence of any systemic manifestations.

• All patients experience painless or painful oral or vaginal ulcers at some time in their illness, which are helpful in making the diagnosis.

• Gastrointestinal findings include vague abdominal discomfort, nausea, and diarrhea. Acute crampy abdominal pain, vomiting, and diarrhea may signify vasculitis of the intestine.

• Joint findings

• Tenderness, edema, and effusions accompany polyarthritis that is symmetric, nonerosive, and usually nondeforming. It frequently involves the proximal interphalangeal (PIP) and metacarpophalangeal (MCP) joints of the hands, as well as the wrists and knees.

• Consider avascular necrosis, which is common in patients receiving glucocorticoids.

• Also consider septic arthritis when one joint is inflamed out of proportion to all other joints.

• Central nervous system findings

• All types of seizures have been reported, with grand mal being the most common. Sensory or sensorimotor neuropathies are also common.

• Incidence of stroke is high in the first 5 years of disease. Patients with antiphospholipid antibodies are at higher risk for such events.

• Funduscopic examination is important in patients with visual complaints. Retinal vasculitis can lead to blindness and are demonstrated by sheathed narrow retinal arterioles with white exudates adjacent to the vessels.

• Renal system - Specific signs and symptoms of renal disease may not be apparent until advanced nephrotic syndrome or renal failure is present; therefore, obtaining a urine analysis and serum BUN and creatinine levels on a regular basis is important.

• Cardiovascular system findings

• Atherosclerosis occurs prematurely in patients with SLE and is an independent risk factor for cardiovascular disease.

• Pulmonary hypertension, vasculitis with digital infarcts, and splinter hemorrhages may be observed.

• Systolic murmurs are reported in up to 70% of cases. They may be secondary to fever, hypoxia, anemia, or Libman-Sacks endocarditis (associated with antiphospholipid antibodies).

• Pericarditis has an incidence of 20-30% and is the most common presentation of heart involvement. It is usually associated with small effusions, but it may involve larger effusions when uremia is concomitant. Myocarditis can cause heart failure, arrhythmias, and sudden death.

• Pulmonary findings

• Tachypnea, cough, and fever are common manifestations of lupus pneumonitis.

• Hemoptysis may signify pulmonary hemorrhage. However, infection is the most common cause of infiltrates seen on radiographs.

Causes:

• Many of the clinical manifestations of SLE are caused by the effects of circulating immune complexes on various tissues or to the direct effects of antibodies to cell surface components.

• Whether polyclonal B-cell activation or a response to specific antigens exists is unclear.

• A lack of immune tolerance is observed in animal models.

• A genetic predisposition to the development of SLE exists. The concordance rate in monozygotic twins is approximately 25-70%. Each patient manifests his or her disease differently.

• If a mother has SLE, her daughter's risk of developing the disease is 1:40, and her son's risk is 1:250.

• Photosensitivity is clearly a precipitant of skin disease. The presence of antiphospholipid antibodies in patients dictates a constellation of signs caused by thrombosis.
  

DIFFERENTIALS

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Other Problems to be Considered:

Metastatic malignancy
Fever of unknown origin (FUO)
Mixed connective tissue disease
Psychogenic rheumatism
Scleroderma
Discoid lupus
Hemoptysis

Drug-induced lupus

Before making a diagnosis of SLE, ruling out drugs as the cause of the condition is important. Table 1 shows the many pharmacologic agents associated with a lupuslike syndrome. Procainamide, hydralazine, and isoniazid have been studied the best. Many patients receiving these medications have positive antinuclear antibody test results and other serologic findings. Only a few have the clinical manifestations.

Drug-induced lupus differs from SLE by the following features:

• Sex ratios are nearly equal.
  
  
• Nephritis and central nervous system features are not commonly present.
  
  
• No antibodies to native DNA or hypocomplementemia are present.
  
  
• When the drug is discontinued, the patient has resolution of clinical manifestations and reverting of abnormal laboratory values to normal.

WORKUP

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Lab Studies:

• Simple laboratory tests may be helpful in making the diagnosis or in evaluating a flare. However, the diagnosis is mostly dependent on the historical and physical findings.

• Rarely should autoantibody tests be ordered in the ED, unless that is done for the assistance of a secured rheumatology follow-up.

• Complete blood count (CBC)

• Leukopenia, which generally is a good index for disease activity

• Lymphopenia

• Anemia of chronic disease (60-80%)

• Evidence of a hemolytic anemia (10%)

• Cytotoxic therapy (can cause anemia or leukopenia)

• Thrombocytopenia (30-50% of cases), which may be profound secondary to antiplatelet antibodies or to antiphospholipid antibodies

• The partial thromboplastin time (PTT) may be elevated secondary to lupus anticoagulant (antiphospholipid antibody), which is associated with thrombosis.

• Urinalysis

• Pyuria

• Hematuria

• Granular casts

• Proteinuria

• Blood urea nitrogen (BUN) and creatinine

• Usually not elevated at the onset of disease

• Can be useful for the determination of any progression of renal disease

Imaging Studies:

• Chest radiographs

• Effusion

• Infiltrates

• Cardiomegaly

• An echocardiogram may be indicated to evaluate any effusion causing pericardial pain or any valvular pathology and to confirm any signs of pulmonary hypertension.

• Magnetic resonance imaging (MRI) is most useful for assessing brain pathology.

• Computed tomography (CT) is useful to rule out bleeding or mass lesions.

Other Tests:

• Cerebrospinal fluid (CSF) analysis is recommended when the diagnosis of CSF lupus is in question or infection is a possible cause of symptoms.

• High protein levels in 50% of patients and pleocytosis may be found.

• May indicate cerebritis but is not specific for it.

• Order serologies in the ED only with a secured rheumatologic consultation and follow-up.

• Antinuclear antibody (ANA), an antibody to nucleosomal DNA-histone complexes, is more than 95% sensitive but not very specific.

• Anti–double-stranded DNA is more specific for lupus. This test may correlate with the degree of activity of lupus, in general, and with the level of nephritis.

• Antiphospholipid antibody

• Present in 30% of patients with SLE

• Associated with thromboembolic complications

• Associated with mild-to-moderate PTT elevations

TREATMENT

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Emergency Department Care:

• Attend to the management of individual emergencies that may be complications of SLE in the individual patient.

• These can include strokes, acute myocardial infarctions, hemoptysis, respiratory distress, and pulmonary emboli.

• In patients presenting with fever, treating for an infection empirically may be necessary until culture results have been received.

• Individual treatment plans are beyond the scope of this article and are discussed in other articles under the headings related to the particular complication (see Differentials).

Consultations:

• When available, consult a rheumatologist for both patients presenting with symptoms suggestive of SLE and for patients with known disease.

• Complications, such as pericardial tamponade, pulmonary hemorrhage, renal failure, or cerebritis, mandate the appropriate subspecialty consultation.

MEDICATION

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Conservative management with nonsteroidal anti-inflammatory drugs including salicylates is recommended for arthritis, arthralgias, and myalgias not requiring immunosuppression. Only initiate high-dose glucocorticoids and cytotoxic agents by, or in consultation with, a rheumatologist. Patients with thrombosis require anticoagulation with warfarin for a target international normalized ratio (INR) of 3-3.5. Antibiotics may be appropriate in the treatment of ordinary and opportunistic infections.

Drug Category: Nonacetylated salicylates -- These agents are indicated to manage the inflammatory process.

Drug Name	Choline magnesium trisalicylate (Trilisate) -- An excellent initial management drug, which has less GI symptoms and less impairment of platelets and renal function than acetylated agents. Salicylates have analgesic, antipyretic, anti-inflammatory and antirheumatic effects. The pharmacologic effects of these agents are qualitatively similar. Their anti-inflammatory and analgesic activity may be mediated through the inhibition of the prostaglandin synthetase enzyme complex.
Adult Dose	500 mg to 1.5 g PO bid/tid Maintenance dose: 1-4.5 g/d PO
Pediatric Dose	<37 kg: 50 mg/kg/d PO divided bid >37 kg: Administer as in adults
Contraindications	Documented hypersensitivity; bleeding disorders
Interactions	Salicylate intoxication may occur with coadministration of carbonic anhydrase inhibitors; corticosteroids decrease salicylate serum levels; may have additive hypoprothrombinemic effect and may increase bleeding time
Pregnancy	C - Safety for use during pregnancy has not been established.
Precautions	Associated with Reye's syndrome in children or teenagers with influenza or chickenpox; discontinue if dizziness, ringing in ears or impaired hearing occurs (may represent toxicity); caution in liver damage, preexisting hypoprothrombinemia, and vitamin K deficiency

Drug Category: Nonsteroidal anti-inflammatory drugs (NSAIDs) -- These agents are most commonly used for the relief of mild-to-moderate pain. Although the effects of NSAIDs in the treatment of pain tend to be patient specific, ibuprofen is usually the DOC for initial therapy. Other options include fenoprofen, flurbiprofen, mefenamic acid, ketoprofen, indomethacin, and piroxicam.

Drug Name	Ibuprofen (Motrin, Nuprin, Ibuprin, Advil) -- Usually the DOC for treatment of mild-to-moderate pain, if there are no contraindications. Inhibits inflammatory reactions and pain probably by decreasing activity of the enzyme cyclo-oxygenase, which results in inhibition of prostaglandin synthesis.
Adult Dose	400 mg PO q4-6h, 600 mg PO q6h, or 800 mg PO q8h; while symptoms persist; not to exceed 3.2 g/d
Pediatric Dose	6 months to 12 years: 10-70 mg/kg/d PO divided tid/qid; start at the lower end of the dosing range and titrate upward to a maximum of 2.4 g/d >12 years: Administer as in adults
Contraindications	Documented hypersensitivity; peptic ulcer disease; recent GI bleeding or perforation; renal insufficiency; high risk of bleeding
Interactions	Coadministration with aspirin increases risk of inducing serious NSAID-related side effects; probenecid may increase concentrations and, possibly, toxicity of NSAIDs; may decrease effect of hydralazine, captopril, and beta-blockers; may decrease diuretic effects of furosemide and thiazides; monitor PT closely (instruct patients to watch for signs of bleeding); may increase risk of methotrexate toxicity; phenytoin levels may be increased when administered concurrently
Pregnancy	B - Usually safe but benefits must outweigh the risks.
Precautions	Category D in third trimester of pregnancy; caution in congestive heart failure, hypertension, and decreased renal and hepatic function; caution in anticoagulation abnormalities or during anticoagulant therapy

Drug Category: Antimalarials -- These agents are an alternative conservative therapy with both sunblocking and anti-inflammatory effects. Initiation of antimalarial therapy usually does not take place in the ED setting.

Drug Name	Hydroxychloroquine (Plaquenil) -- Inhibits chemotaxis of eosinophils, locomotion of neutrophils, and impairs complement-dependent antigen-antibody reactions. Hydroxychloroquine sulfate 200 mg is equivalent to 155 mg hydroxychloroquine base and 250 mg chloroquine phosphate. Used for the treatment of discoid and systemic lupus erythematosus and rheumatoid arthritis.
Adult Dose	200-400 mg PO qd for several wk depending on response; 200 mg/d for prolonged maintenance therapy
Pediatric Dose	3-5 mg base/kg/d PO qd or divided bid; not to exceed 7 mg/kg/d
Contraindications	Documented hypersensitivity; psoriasis; retinal and visual field changes attributable to 4-aminoquinolones
Interactions	Serum levels increase with cimetidine; magnesium trisilicate may decrease absorption
Pregnancy	C - Safety for use during pregnancy has not been established.
Precautions	Caution in hepatic disease, G-6-PD deficiency, psoriasis, and porphyria; not recommended for long term in children; perform periodic (6 mo) ophthalmologic examinations; test periodically for muscle weakness

Drug Category: Glucocorticoids -- Have both anti-inflammatory properties and cause profound and varied metabolic effects. In addition, these agents modify the body's immune response to diverse stimuli. High-dose glucocorticoids are used for severe SLE complications, such as hematologic or CNS disease, serositis, vasculitis, or glomerulonephritis.

Drug Name	Prednisone (Deltasone) -- Used as an immunosuppressant in the treatment of autoimmune disorders. By reversing increased capillary permeability and suppressing PMN activity, it may decrease inflammation.
Adult Dose	5-60 mg/d qd or divided bid/qid; taper over 2 wk as symptoms resolve
Pediatric Dose	4-5 mg/m2/d; alternatively, 1-2 mg/kg PO qd; taper over 2 wk as symptoms resolve
Contraindications	Documented hypersensitivity; viral infection, peptic ulcer disease, hepatic dysfunction, connective tissue infections, and fungal or tubercular skin infections; GI disease
Interactions	Coadministration with estrogens may decrease prednisone clearance; concurrent use with digoxin, may cause digitalis toxicity secondary to hypokalemia; phenobarbital, phenytoin, and rifampin may increase metabolism of glucocorticoids (consider increasing maintenance dose); monitor for hypokalemia with coadministration of diuretics
Pregnancy	B - Usually safe but benefits must outweigh the risks.
Precautions	Abrupt discontinuation of glucocorticoids may cause adrenal crisis; hyperglycemia, edema, osteonecrosis, myopathy, peptic ulcer disease, hypokalemia, osteoporosis, euphoria, psychosis, myasthenia gravis, growth suppression, and infections may occur with glucocorticoid use

Drug Name	Methylprednisolone (Solu-Medrol, Depo-Medrol, Adlone) -- Useful in the treatment of inflammatory and autoimmune reactions. By reversing increased capillary permeability and suppressing PMN activity, it may decrease inflammation.
Adult Dose	Loading dose: 125-250 mg IV Maintenance dose: 0.5-1 mg/kg/dose q6h for up to 5 d
Pediatric Dose	Loading dose: 2 mg/kg IV Maintenance dose: 0.5-1 mg/kg/dose q6h for up to 5 d
Contraindications	Documented hypersensitivity; viral, fungal or tubercular skin infections
Interactions	Coadministration with digoxin, may increase digitalis toxicity secondary to hypokalemia; estrogens may increase levels of methylprednisolone; phenobarbital, phenytoin and rifampin may decrease levels of methylprednisolone (adjust dose); monitor patients for hypokalemia when taking medication concurrently with diuretics
Pregnancy	C - Safety for use during pregnancy has not been established.
Precautions	Hyperglycemia, edema, osteonecrosis, peptic ulcer disease, hypokalemia, osteoporosis, euphoria, psychosis, growth suppression, myopathy, and infections are possible complications of glucocorticoid use

Drug Category: Immunosuppressives/cytotoxic agents -- These agents are used in active SLE cases resistant to corticosteroids. Patients should be monitored closely because of the adverse effects, and these agents should be administered by experienced specialist physicians. These drugs are more beneficial when used in conjunction with glucocorticoids. Treatment with glucocorticoids plus cyclophosphamide is more beneficial, although more toxic, than treatment with glucocorticoids plus azathioprine.

Drug Name	Cyclophosphamide (Cytoxan, Neosar) -- Chemically related to nitrogen mustards. As an alkylating agent, the mechanism of action of the active metabolites may involve cross-linking of DNA, which may interfere with growth of normal and neoplastic cells. Pediatric dosing is controversial and not first line.
Adult Dose	10-20 mg/kg IV q3-4wk or 1.5-2.5 mg/kg PO qd
Pediatric Dose	Not established
Contraindications	Documented hypersensitivity; severely depressed bone marrow function
Interactions	Allopurinol may increase risk of bleeding or infection and enhance myelosuppressive effects; may potentiate doxorubicin-induced cardiotoxicity; may reduce digoxin serum levels and antimicrobial effects of quinolones Chloramphenicol may increase half-life while decreasing metabolite concentrations; may increase effect of anticoagulants; coadministration with high doses of phenobarbital may increase rate of metabolism and leukopenic activity; thiazide diuretics may prolong cyclophosphamide-induced leukopenia and neuromuscular blockade by inhibiting
Pregnancy	D - Unsafe in pregnancy
Precautions	Regularly examine hematologic profile (particularly neutrophils and platelets) to monitor for hematopoietic suppression; regularly examine urine for RBCs, which may precede hemorrhagic cystitis

Drug Name	Azathioprine (Imuran) -- Antagonizes purine metabolism and inhibits synthesis of DNA, RNA, and proteins. May decrease proliferation of immune cells, which results in lower autoimmune activity. Pediatric dosing is controversial and not first line.
Adult Dose	1.5-2.5 mg/kg PO qd
Pediatric Dose	Not established
Contraindications	Documented hypersensitivity; low levels of serum thiopurine methyl transferase (TPMT)
Interactions	Toxicity increases with allopurinol; concurrent use with ACE inhibitors may induce severe leukopenia; may increase levels of methotrexate metabolites and decrease effects of anticoagulants, neuromuscular blockers, and cyclosporine
Pregnancy	D - Unsafe in pregnancy
Precautions	Increases risk of neoplasia; caution with liver disease and renal impairment; hematologic toxicities may occur; check TPMT level before therapy and follow liver, renal, and hematologic function; pancreatitis rarely associated

FOLLOW-UP

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Further Inpatient Care:

• Patients may require admission for treatment of severe complications, such as lupus nephritis (for pulse steroids, cytotoxic agents) or other entities responsive to high-dose steroids.

• Admit patients with neuropsychiatric presentation or stroke syndromes.

• Admit patient to appropriate service (rheumatology if available) with subspecialty consultations as needed.

Further Outpatient Care:

• Conservative management is indicated for the symptomatic relief of arthralgias, arthritis, or myalgias with nonacetylated salicylates, NSAIDs, or low-dose glucocorticoids.

In/Out Patient Meds:

• Nonacetylated salicylates or NSAIDs

Deterrence/Prevention:

• Advise patients to use a sunscreen. Exposure to ultraviolet light causes SLE to flare in approximately 70% of patients.

Complications:

• Vasculitis and its various complications (eg, intestinal perforations)

• Pericarditis

• Myocarditis

• Lupus pneumonitis

• Pulmonary hemorrhage, pulmonary hypertension

• Proliferative glomerulonephritis

• Hemolytic anemia, thrombocytopenia

• Intravascular thrombosis (eg, stroke and myocardial infarctions)

• Complications of high dose glucocorticoid therapy

• Complications of cytotoxic agents

Prognosis:

• Prognosis has improved over the last few years.

• Mortality typically is due to renal failure or infection.

Patient Education:

• Protection from the sun

• Compliance with medications and follow-up appointments

• For excellent patient education resources, visit eMedicine's Immune System Center. Also, see eMedicine's patient education article Lupus (Systemic Lupus Erythematosus).

MISCELLANEOUS

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Medical/Legal Pitfalls:

• Failure to evaluate for ordinary or opportunistic infections

• Failure to involve rheumatologist or primary physician in the management and disposition of a case

Special Concerns:

• Whether flares of SLE are more frequent during pregnancy is controversial. The flares do not seem to be exceedingly more serious compared to those in nonpregnant patients. Pre-eclampsia, fetal wastage, prematurity, and intrauterine growth retardation are more frequent. Predictors for fetal loss include active nephritis at conception and the presence of antiphospholipid (aPL) antibodies.

• High-dose aspirin and NSAIDs should be avoided in the last few weeks of pregnancy. Hydroxychloroquine has not been shown to induce congenital malformations. Furthermore, unnecessary discontinuation of hydroxychloroquine during pregnancy may result in lupus flares. Prednisone, prednisolone, and methylprednisolone are the corticosteroids of choice, if necessary, during pregnancy because of their minimal placental transfer.

TEST QUESTIONS

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CME Question 1: A 30-year-old woman with known history of systemic lupus erythematosus (SLE) presents to the ED with chief complaint of amenorrhea. She currently is taking hydroxychloroquine and Motrin for her SLE. Pregnancy test results were found to be positive with a quantitative beta–human chorionic gonadotropin level consistent with 8 weeks' gestation. The patient denies any other complaints. Which of the following statements is incorrect?

A: Incidence of preeclampsia is increased in patients with SLE.
B: A predictor for fetal loss includes active nephritis at the time of conception.
C: Nonsteroidal anti-inflammatory drugs (NSAIDS) and aspirin are safe to use throughout the course of pregnancy.
D: Patients taking hydroxychloroquine should have ophthalmologic examinations every 6 months.
E: Incidence of premature delivery is increased in SLE pregnancies.

The correct answer is C: High-dose aspirin and NSAIDs should be avoided in the last few weeks of pregnancy because of their possible effects on uterine contraction, platelet function, and physiological changes that take place around birth, such as closure of the ductus arteriosus.

CME Question 2: A 29-year-old woman presents with fatigue, rash, and Raynaud phenomenon. Which statement regarding the diagnosis of systemic lupus erythematosus (SLE) is correct?

A: The mean duration between the onset of the first symptom and the diagnosis is 3 weeks.
B: A temperature higher than 102°F is common.
C: The presence of oral ulcers constitutes one of the diagnostic criteria requirements.
D: Pericarditis must be accompanied by a significant fluid collection.
E: Neurologic manifestations, which include seizures, focal deficits, sensorimotor neuropathy, and psychiatric disturbances, are rare.

The correct answer is C: Revised criteria for SLE require 4 of the following: malar rash, discoid rash, photosensitivity, oral ulcers, serositis, renal disorder, neurologic disorder, hematologic disorder, immunologic disorder, or antinuclear antibody. Look for painless oral ulcers on examination. The mean time to diagnosis is reported to be 5 years. Temperature higher than 102°F should prompt a search for infection. Pericarditis infrequently is accompanied by tamponade. It can occur with small amounts of pericardial fluid. Neurologic symptoms are protean. Psychiatric disturbances are common.

Pearl Question 1 (T/F): A disease flare is a potential cause for a febrile illness in a patient with lupus.

The correct answer is True: A disease flare or a routine or opportunistic infection secondary to immunosuppressive agents is a potential cause for a febrile illness in a patient with lupus.

Pearl Question 2 (T/F): The most common patient profile for systemic lupus erythematosus (SLE) is a 35-year-old man.

The correct answer is False: A women in her childbearing years is the most common patient profile for SLE.

Pearl Question 3 (T/F): The recommended management for lupus nephritis is outpatient oral steroids.

The correct answer is False: Admission and high-dose steroids are recommended.

Pearl Question 4 (T/F): Systemic lupus erythematosus (SLE) is an independent risk factor for cardiovascular disease.

The correct answer is True: Atherosclerosis occurs prematurely in patients with SLE and is independent of traditional risk factors for cardiovascular disease.

BIBLIOGRAPHY

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eMedicine Journals > Emergency Medicine > Rheumatology > Systemic Lupus Erythematosus

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Author Information | Introduction | Clinical | Differentials | Workup | Treatment | Medication | Follow-up | Miscellaneous | Test Questions | Bibliography