1. Systemic Lupus Erythematosus
Iris Zink, CRNP

2. 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

3. The Great Imitator

4. Average time of two years from onset of symptoms to diagnosis
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)

5. Shoua

6. Judy

7. Nakia

8. 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)

9. Epidemiology: Race, Gender and Age
More prevalent in African Americans, Caribbean populations, Hispanics and
Asians
Female > Male
Most common between year olds

10. How is SLE diagnosed? Labs Symptomatology
Patients must meet 4/11 criteria

11. 1.Malar Rash 2.Discoid Rash

12. 3. Photosensitivity 4.Mucocutaneous Ulcers

13. 5. Arthritis Serositis

14. 7. Renal Disorder 8. Neurologic Disorder

15. 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 3:3, March 2009

16. 9. Hematologic 10. Immunologic
Leukopenia
Thrombocytopenia
Lymphopenia
Anti- DNA

17. Antinuclear Antibodies ANA
1: 40
1: 80
1:160
1: 320
1: 640
1:1280
1:2560
1:5120

18. ANA Interpretation
1:40
1:80
1:160
1:320
1:640
1:1280
1:2560

19. Lab Tests ANA ENA Ro SSA La SSB DNA Sm RNP Jo-1 Histone Scl-70
Antiphospholipid antibody
Lupus Anticoagulant
Complement

20. Symptoms
Fever and fatigue 42%
Alopecia 18%
Lupus Nephritis 40-60%

21. 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 have MI’s 50 times that of age matched controls

22. 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

23. Treatment Only FDA approved medications Plaquenil (hydroxychloroquine)
Aspirin
Steroids (prednisone)
Medications for Lupus Nepritis
Cyclophosphamide (Cytoxan)
Mycophenolate Mofetil (CellCept)
Nephritis
Nephritis 23

24. 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

25. References
Cervera, R., Khamashta, M. A., Font, J. (2003). Morbidity and mortality in SLE during a 10 yr period. Medicine (Baltimore), 82,
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,
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,
Petri, M. (2004). SLE. In Imboden, J., Hellman, D. & Stone, J. (Eds.) Current Rheumatology. Chapter 19. Retrieved 9/29/06 from
Petri, M. Systemic Lupus Erythematosus 2006 update. (2006). J Clin Rheum 2006, 12, Tan, E. M., Cohen, A. S., Fries, J. F., et al. (1992). The 1992 revised criteria for the classification of SLE. Arthritis Rheum, 25,
Schur, P. H. (2006). Epidemiology and pathogenesis of SLE. Up to date v Retrieved 09/27/06 from
Schur, P. H. (2006). Hematologic manifestations of SLE in adults. Up to Date v Retrieved 10/23/06 from 25

26. 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

27. Resources for Patients
LFA, Michigan & Northwest Ohio - Findlay, OH , Toll-free (within OH and MI only)