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Systemic Lupus Erythematosus

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

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Systemic Lupus Erythematosus

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

  9. Epidemiology: Race, Gender and Age More prevalent in African Americans, Caribbean populations, Hispanics and Asians Female > Male Most common between 20-40 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 6. 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. ANA Interpretation 1:40 1:80 1:160 1:320 1:640 1:1280 1:2560

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

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

  20. 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

  21. 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

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

  23. 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

  24. 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.

  25. 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

  26. Resources for Patients 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/

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