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

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)

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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) 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

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

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

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 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

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

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

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/