Systemic Lupus Erythematosus in Emergency Medicine Follow-up

  • Author: Mark J Leber, MD, MPH, FACEP; Chief Editor: Rick Kulkarni, MD   more...
 
Updated: Jan 4, 2011
 

Further Inpatient Care

Patients with systemic lupus erythematosus (SLE) 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 patients to appropriate service (rheumatology if available) with subspecialty consultations as needed.

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Further Outpatient Care

Conservative management is indicated for the symptomatic relief of arthralgia, arthritis, or myalgia with nonacetylated salicylates, nonsteroidal anti-inflammatory drugs (NSAIDs), or low-dose glucocorticoids.

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Inpatient & Outpatient Medications

Nonacetylated salicylates or NSAIDs may be indicated.

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Deterrence/Prevention

Patients with systemic lupus erythematosus (SLE) should be encouraged to take steps to prevent sunburn by limiting sun exposure, wearing protective clothing, and using sunscreen that protects against both UV-A and UV-B. Exposure to UV light causes SLE to flare in approximately 70% of patients. Sunscreen should have an SPF of at least 15 and should be applied frequently (every 2-3 h) and in the quantity recommended by the manufacturer.

Photosensitive systemic lupus erythematosus (SLE) Photosensitive systemic lupus erythematosus (SLE) rashes typically occur on the face or extremities, which are sun-exposed regions. Photo courtesy of Dr. Erik Stratman, Marshfield Clinic.

Patients should have yearly vaccinations for influenza. Pneumococcus vaccine should be updated every 3-6 years.[20] Hepatitis B vaccines are recommended. Patients on high-dose immunotherapy should NOT receive live vaccines.

Although organ involvement requires specific drug therapy, a number of general issues are applicable to every patient with SLE.

Patients should avoid exposure to direct or reflected sunlight and other sources of UV light. Use sunscreens that block both UV-A and UV-B with sun protection factor (SPF) of 30.

A conservative approach is to recommend a balanced diet consisting of carbohydrates, proteins, and fats. However, the diet should be modified based on disease activity and response to therapy. Patients with active inflammatory disease and fever may require an increase in caloric intake.

Glucocorticoids enhance appetite, resulting in potentially significant weight gain. Hunger can be somewhat lessened by the ingestion of water, antacids, proton pump inhibitors, and/or histamine H2 blockers. A low calorie diet should be instituted if significant weight gain occurs.

Most patients with SLE have low serum levels of 25-hydroxyvitamin D (calcidiol), probably due at least in part to avoidance of sun exposure. Patients with low vitamin D levels should be treated with supplemental vitamin D.

Patients on long-term glucocorticoids and postmenopausal women should ingest 800 units of vitamin D plus 1500 mg of calcium per day and/or a bisphosphonate to minimize the degree of bone loss.

Cigarette smoking may increase the risk of developing SLE, and smokers in general have more active disease. Patients should be counseled not to smoke or to quit smoking and be provided with help to do so.

Inactivity produced by acute illness causes a rapid loss of muscle mass and stamina resulting in a sense of fatigue. This can usually be treated with graded exercise. In selected refractory cases, relief can be obtained with antimalarial drugs.

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Complications

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Prognosis

Mortality of those with systemic lupus erythematosus (SLE) has decreased over the past 20 years.[25]

Five-year survival rate in Western countries ranges between 93-95%.[25]

Mortality typically in the first few years of illness is active disease (eg, CNS, renal, or cardiovascular disease) or infection due to immunosuppression, whereas late deaths are due to atherosclerosis.[25, 9]

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Patient Education

Patients should protect their skin from the sun.

Compliance with medications and follow-up appointments is important.

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

For more information, see Medscape’s Lupus Resource Center.

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Contributor Information and Disclosures
Author

Mark J Leber, MD, MPH, FACEP  Attending Physician and Faculty, Department of Emergency Medicine and Residency Program, Lincoln Medical and Mental Health Center

Mark J Leber, MD, MPH, FACEP is a member of the following medical societies: American College of Emergency Physicians and American College of Physicians

Disclosure: Nothing to disclose.

Coauthor(s)

Viraj S Lakdawala, MD  Chief Resident, Department of Emergency Medicine, Lincoln Medical and Mental Health Center, Bronx, NY

Viraj S Lakdawala, MD is a member of the following medical societies: American Academy of Emergency Medicine and American College of Emergency Physicians

Disclosure: Nothing to disclose.

Specialty Editor Board

Richard S Krause, MD  Senior Clinical Faculty/Clinical Assistant Professor, Department of Emergency Medicine, University of Buffalo State University of New York School of Medicine and Biomedical Sciences

Richard S Krause, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Emergency Medicine, American College of Emergency Physicians, and Society for Academic Emergency Medicine

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD  Senior Pharmacy Editor, eMedicine

Disclosure: eMedicine Salary Employment

Gino A Farina, MD, FACEP, FAAEM  Associate Professor of Clinical Emergency Medicine, Albert Einstein College of Medicine; Program Director, Department of Emergency Medicine, Long Island Jewish Medical Center

Gino A Farina, MD, FACEP, FAAEM is a member of the following medical societies: American Academy of Emergency Medicine, American College of Emergency Physicians, and Society for Academic Emergency Medicine

Disclosure: Nothing to disclose.

John D Halamka, MD, MS  Associate Professor of Medicine, Harvard Medical School, Beth Israel Deaconess Medical Center; Chief Information Officer, CareGroup Healthcare System and Harvard Medical School; Attending Physician, Division of Emergency Medicine, Beth Israel Deaconess Medical Center

John D Halamka, MD, MS is a member of the following medical societies: American College of Emergency Physicians, American Medical Informatics Association, Phi Beta Kappa, and Society for Academic Emergency Medicine

Disclosure: Nothing to disclose.

Chief Editor

Rick Kulkarni, MD 

Rick Kulkarni, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Emergency Medicine, American College of Emergency Physicians, American Medical Association, American Medical Informatics Association, Phi Beta Kappa, and Society for Academic Emergency Medicine

Disclosure: WebMD Salary Employment

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The classic malar rash, also known as a butterfly rash, with distribution over the cheeks and nasal bridge. Note that the fixed erythema, sometimes with mild induration as seen here, characteristically spares the nasolabial folds.
Photosensitive systemic lupus erythematosus (SLE) rashes typically occur on the face or extremities, which are sun-exposed regions. Photo courtesy of Dr. Erik Stratman, Marshfield Clinic.
This axial, T2-weighted brain MRI demonstrates an area of ischemia in the right periventricular white matter of a 41-year-old woman with long-standing systemic lupus erythematosus (SLE). She presented with headache and subtle cognitive impairments but no motor deficits. Faintly increased signal intensity was also seen on T1-weighted images, with a trace of enhancement following gadolinium that is too subtle to show on reproduced images. Distribution of the abnormality is consistent with occlusion of deep penetrating branches, such as may result from local vasculopathy, with no clinical or laboratory evidence of lupus anticoagulant or anticardiolipin antibody. Cardiac embolus from covert Libman-Sacks endocarditis remains less likely due to distribution.
Table 1. American College of Rheumatology Diagnostic Criteria
CriterionDefinition
1. Malar rashFixed erythema, flat or raised, over the malar eminences, tending to spare the nasolabial folds
2. Discoid rashErythematous raised patches with adherent keratotic scaling and follicular plugging (Atrophic scarring may occur in older lesions)
3. PhotosensitivitySkin rash as a result of unusual reaction to sunlight, by patient history or physician observation
4. Oral ulcersOral or nasopharyngeal ulceration, usually painless, observed by a physician
5. ArthritisNonerosive arthritis involving ≥2 peripheral joints, characterized by tenderness, swelling, or effusion
6. Serositis(A) Pleuritis: Convincing history of pleuritic pain or rub heard by a physician or evidence of pleural effusion



or



(B) Pericarditis: Documented by ECG or rub or evidence of pericardial effusion
7. Renal disorder(A) Persistent proteinuria >0.5 g/d or >3+ if quantitation not performed



or



(B) Cellular casts: May be red blood cell, hemoglobin, granular, tubular, or mixed
8. Neurologic disorder(A) Seizures: In the absence of offending drugs or known metabolic derangements (eg, uremia, ketoacidosis, electrolyte imbalance)



or



(B) Psychosis: In the absence of offending drugs or known metabolic derangements (eg, uremia, ketoacidosis, electrolyte imbalance)
9. Hematologic disorder(A) Hemolytic anemia: With reticulocytosis



or



(B) Leukopenia: < 4000/mm3 total on ≥2 occasions



or



(C) Lymphopenia: < 1500/mm3 on ≥2 occasions



or



(D) Thrombocytopenia: < 100,000/mm3 in the absence of offending drugs
10. Immunologic disorder(A) Anti-DNA: Antibody to native DNA in abnormal titer



or



(B) Anti-Sm: Presence of antibody to Sm nuclear antigen



or



(C) Positive finding of antiphospholipid antibodies based on (1) an abnormal serum level of IgG or IgM anticardiolipin antibodies, (2) a positive test result for lupus anticoagulant using a standard method, or (3) a false-positive serologic test for syphilis known to be positive for at least 6 months and confirmed by Treponema pallidum immobilization or fluorescent treponemal antibody absorption tests
11. Antinuclear antibodyAn abnormal titer of antinuclear antibody by immunofluorescence or an equivalent assay at any point in time and in the absence of drugs known to be associated with drug-induced lupus syndrome
A person can be diagnosed with SLE if any 4 or more of the 11 criteria are present, serially or simultaneously, during any interval of observation.
Table 2. Drugs Associated With Lupus Erythematosus
Definite Association
ChlorpromazineMethyldopa
HydralazineProcainamide
IsoniazidQuinidine
Possible Association
Beta-blockersMethimazole
CaptoprilNitrofurantoin
CarbamazepinePenicillamine
CimetidinePhenytoin
EthosuximidePropylthiouracil
HydrazinesSulfasalazine
LevodopaSulfonamides
LithiumTrimethadione
Unlikely Association
AllopurinolPenicillin
ChlorthalidonePhenylbutazone
Gold



salts



Reserpine
GriseofulvinStreptomycin
MethysergideTetracyclines
Oral contraceptives
*Data from Tierney et al.[23]
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