Transcript Systemic Lupus Erythematous

Systemic Lupus
Erythematosus
Iris Zink, CRNP
Objectives
Discuss pathophysiology of SLE and its
various presentations
 Discuss impact of SLE on patient’s
health
 Discuss the criteria for diagnosis of
SLE, and interpretation of lab tests
 Discuss the interventions and standard
therapies for treatment of SLE

The Great Imitator
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)
Shoua
Judy
Nakia
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)
Epidemiology: Race, Gender
and Age

More prevalent in African Americans,
Caribbean populations, Hispanics and
Asians

Female > Male

Most common between 20-40 year olds
How is SLE diagnosed?
 Labs
 Symptomatology
 Patients
must meet 4/11 criteria
1.Malar Rash
2.Discoid Rash
3. Photosensitivity
4.Mucocutaneous Ulcers
5. Arthritis
6. Serositis
7. Renal Disorder
8. Neurologic Disorder
Neuropsychiatric
Systemic Lupus Erythematosus (NPSLE)
(95 vs 80%)
CNS (diffuse & central)

Acute confusional state
 Psychosis
 Anxiety
 Depressive disorders
 Cognitive dysfunction *
 Seizures
 CVA
 Chorea
 Myelopathy
 Demyelinating syndrome
 Headaches *
PNS
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Brunner, H & Klein-Gitelman, M; Rheumatologist Vol 3:3, March 2009
Neuropathies
Acute inflammatory
demyelination
9. Hematologic 10. Immunologic
Leukopenia
Thrombocytopenia
Lymphopenia
Anti- DNA
ANA Interpretation
1:40
1:80
1:160
1:320
1:640
1:1280
1:2560
Lab Tests

ANA
 ENA Ro SSA
La SSB
 DNA
 Sm
 RNP
 Jo-1
Histone
 Scl-70
 Antiphospholipid
antibody
 Lupus Anticoagulant
 Complement

Symptoms
•
Fever and fatigue 42%
•
Alopecia 18%
•
Lupus Nephritis 40-60%
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 MI’s 50
times that of age matched controls
Quality of Life

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
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
30%
25%
20%
15%
10%
5%
0%
Jolly, J Rheumatol 2005
AA
Hispanic
Caucasian
Treatment
Only FDA approved medications
• Plaquenil (hydroxychloroquine)
• Aspirin
• Steroids (prednisone)
Nephritis
Nephritis
• Medications for Lupus Nepritis
• Cyclophosphamide (Cytoxan)
• Mycophenolate Mofetil (CellCept)
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
References
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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, 23502357.
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.
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
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www.clinicaltrials.gov
http://www.lupus.org/newsite/index.html
http://www.lupus.org/webmodules/webarticlesnet/
templates/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/