Systemic Lupus Erythematosus

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Systemic Lupus Erythematosus

• Iris Zink, CRNP

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Objectives

• Discuss pathophysiology of SLE and its various
  presentations
• Discuss impact of SLE on patients health
• Discuss the criteria for diagnosis of SLE, and
  interpretation of lab tests
• Discuss the interventions and standard therapies
  for treatment of SLE

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The Great Imitator
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• SLE Definition
• Lupus is a systemic autoimmune disease in which
  the body loses
  tolerance to self
• Can affect virtually any organ in the body and
  initial symptoms are often nonspecific, making it
  very difficult to diagnose
• Most commonly seen in women of childbearing age
  but 10 of patients are men
• Average time of two years from onset of symptoms
  to diagnosis
• (Cevera, Medicine, 1993 Font, Semin Arthritis
  Rheum, 2004)

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Shoua
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Judy
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Nakia
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SLE Prevalence

• Prevalence 40-50 cases per 100,000 people
• Approximately 750,000 cases in U.S. today
• Much more common in developed countries and in
  urban areas
• 15-20 diagnosed during childhood

(Schur, Epidemology and pathogenesis of SLE. Up
to date v. 14.2 Petri. SLE. Current
Rheumatology, Chapter 19)
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Epidemiology Race, Gender and Age

• More prevalent in African Americans, Caribbean
  populations, Hispanics and
• Asians
• Female gt Male
• Most common between 20-40 year olds

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How is SLE diagnosed?

• Labs
• Symptomatology
• Patients must meet 4/11 criteria

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1.Malar Rash 2.Discoid Rash
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3. Photosensitivity 4.Mucocutaneous Ulcers
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5. Arthritis 6. Serositis
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7. Renal Disorder 8. Neurologic Disorder
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Neuropsychiatric Systemic Lupus Erythematosus
(NPSLE) (95 vs 80)

• CNS (diffuse central)

• PNS

• Acute confusional state
• Psychosis
• Anxiety
• Depressive disorders
• Cognitive dysfunction
• Seizures
• CVA
• Chorea
• Myelopathy
• Demyelinating syndrome
• Headaches

• Neuropathies
• Acute inflammatory demyelination

Brunner, H Klein-Gitelman, M Rheumatologist
Vol 33, March 2009
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9. Hematologic 10. Immunologic
Leukopenia Thrombocytopenia Lymphopenia
Anti- DNA
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Antinuclear Antibodies ANA
1 40 1 80 1160 1 320 1 640 11280 12560 151
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ANA Interpretation
140
1160
1320
1640
11280
12560
180
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Lab Tests

• ANA
• ENA Ro SSA
• La SSB
• DNA
• Sm
• RNP
• Jo-1

• Histone
• Scl-70
• Antiphospholipid antibody
• Lupus Anticoagulant
• Complement

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Symptoms

• Fever and fatigue 42
• Alopecia 18
• Lupus Nephritis 40-60

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Causes of Death

• Death from renal disease is most common in first
  3-5 years
• Patients who survive the first 5 years of disease
  die from CVD at a much younger age than disease
  free individuals
• Women with SLE ages 35-44 have MIs 50 times
  that of age matched controls

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Quality of Life

• 90 patients SLE
• Significantly worse QOL vs.
• age matched controls
• HTN, diabetes, or MI
• Lupus lower than patients with CHF
• re physical function, bodily pain, general
  health

Jolly, J Rheumatol 2005
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Treatment

• Only FDA approved medications
• Plaquenil (hydroxychloroquine)
• Aspirin
• Steroids (prednisone)
• Medications for Lupus Nepritis
• Cyclophosphamide (Cytoxan)
• Mycophenolate Mofetil (CellCept)

Nephritis
Nephritis
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DAMP AS RHINO

• Discoid rash
• ANA
• Malar rash
• Photosensitivity
• Arthritis
• Serositis (pleural, pericardial)
• Renal involvement
• Hematologic abnormality
• Immunologic abnormality
• Neurologic abnormality (seizures, psychosis)
• Oral/ nasal ulcer

www.medicalnemonics.com
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References

• Cervera, R., Khamashta, M. A., Font, J. (2003).
  Morbidity and mortality in SLE during a 10 yr
  period. Medicine (Baltimore), 82, 299-308.
• Ho, A., Barr, S. G., Magder, L. S. (2001). A
  decrease in complement is associated with
  increased renal and hematologic activity in
  patients with SLE. Arthritis Rheum, 44,
  2350-2357.
• Hochberg, M .C. (1997). Updating the ACR revised
  criteria for the classification of SLE letter.
  Arthritis Rheum, 40, 1725.
• Jolly, M. (2005). How does quality of life of
  patients with SLE compare with that of other
  common chronic illnesses? J Rheumatol, 32,
  1706-8.
• Petri, M. (2004). SLE. In Imboden, J., Hellman,
  D. Stone, J. (Eds.) Current Rheumatology.
  Chapter 19. Retrieved 9/29/06 from
  www.accessmedicine.com.
• Petri, M. Systemic Lupus Erythematosus 2006
  update. (2006). J Clin Rheum 2006, 12,
  37-40.Tan, E. M., Cohen, A. S., Fries, J. F., et
  al. (1992). The 1992 revised criteria for the
  classification of SLE. Arthritis Rheum, 25,
  1271-7.
• Schur, P. H. (2006). Epidemiology and
  pathogenesis of SLE. Up to date v.14.2. Retrieved
  09/27/06 from www.uptodateonline.com.
• Schur, P. H. (2006). Hematologic manifestations
  of SLE in adults. Up to Date v.14.2. Retrieved
  10/23/06 from www.uptodateonline.com.

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Summary

• Due to variety of symptoms, must know entire
  history
• Great imposter
• Refer to Rheumatology if SLE suspected
• In SLE watch closely as things change fast and
  CVD is a real concern
• Monitor for QOL issues

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Resources for Patients

• www.clinicaltrials.gov
• http//www.lupus.org/newsite/index.html
• http//www.lupus.org/webmodules/webarticlesnet/tem
  plates/nwohio_home.aspx
• LFA, Michigan Northwest Ohio - Findlay, OH
  419-423-9313, Toll-free 888-335-8787 (within OH
  and MI only)
• http//www.arthritis.org/
• http//www.mayoclinic.com/