Systemic Lupus Erythematosus in Emergency Medicine 

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

Background

Systemic lupus erythematosus (SLE) is a multiorgan system autoimmune disease with numerous immunological and clinical manifestations. It is characterized by an autoantibody response to nuclear and cytoplasmic antigens. The disease mainly involves the skin, joints, kidneys, blood cells, and nervous system. Diagnosing and managing SLE in the emergency department can be very challenging if it is not considered in one's differential diagnosis. Also, the laboratory testing of SLE may be unavailable on an emergent basis.

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Pathophysiology

Systemic lupus erythematosus (SLE) is a multifactorial disease involving genetic, environmental, and hormonal factors. Its precise pathogenesis is unclear. There is growing evidence in favor of a clearance deficiency of apoptotic cells as the core mechanism in the pathogenesis of SLE.[1] Defective clearance of apoptotic cells causes secondary necrosis with release of intracellular content and inflammatory mediators. Macrophages respond and present self-antigens to T and B cells.[1]

Pathogenic autoantibodies are the primary cause of tissue damage in patients with lupus. The production of these antibodies arises by means of complex mechanisms involving every key facet of the immune system.[2] The abnormal cellular and humoral response to the formation of these autoantibodies is modulated by genetic, environmental, and hormonal factors:

  • Genetic factors
    • Genes of the MHC HLA-A1, B8, and DR3 have been linked to lupus.
    • Genetic deficiency of complement factors C1q, C2, or C4
  • Environmental factors[3]
    • Occupational exposure - Silica, pesticides, mercury
    • Drugs - Many drugs have been implicated in drug-induced lupus.
    • Sunlight
  • Epstein-Barr virus (EBV) has also been identified as a possible factor in the development of lupus.[3]
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Epidemiology

Frequency

United States

In the United States, the annual incidence of systemic lupus erythematosus (SLE) averages 5.1 per 100,000 per year. The incidence of SLE in black women is approximately 4 times higher than in white women. SLE is more frequent in Asian women than in white women.[4]

The reported prevalence of SLE in the population is 52 cases per 100,000.[5]

The frequency of SLE could be increasing due to mild forms of the disease that are being recognized.

International

The highest prevalences of systemic lupus erythematosus (SLE) were reported in Italy, Spain, Martinique, and the UK Afro-Caribbean population.[5] SLE is more common in women with West African ancestry who have emigrated from their home area, suggesting that there may be an environmental trigger as well as a genetic basis for their disease.

Mortality/Morbidity

The life expectancy of such patients has improved from an approximate 4-year survival rate of 50% in the 1950s to a 15-year survival rate of 80% today.[6] A patient in whom SLE is diagnosed by age 20 years still has a 1 in 6 chance of dying within 15 years, most often from lupus or infection.[7] After 35, the patient is more likely to die from myocardial infarction or stroke.[7]

Infectious diseases have emerged as one of the leading causes of morbidity and mortality, accounting for 29% of all deaths in these patients.[8]

Women with SLE between 35 and 44 years of age had a 52-fold higher risk of having a myocardial infarction than matched women from the Framingham cohort.[9]

Ten-year survival rates in other countries in Asia and Africa are significantly lower, ranging from 60-70%.[10, 11, 12]

Race

Black women have a higher rate of SLE than any other race, followed by Asians, then white women.[5]

In the United States, black women are 4 times more likely to have SLE than white women.[5]

Sex

For all ages, the female-to-male ratio is 7:1 and 11:1 during the childbearing years.[13]

Age

Onset of systemic lupus erythematosus (SLE) is usually after puberty, typically in the 20s and 30s.

Twenty percent of all cases of lupus are diagnosed during the first 2 decades of life.[14]

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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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