Systemic Lupus Systemic Lupus ErythematosusErythematosus

Iris Zink, CRNP

ObjectivesObjectives

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 ImitatorThe 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)

ShouaShoua

JudyJudy

NakiaNakia

SLE Prevalence 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 Epidemiology: Race, Gender and Ageand Age

More prevalent in African Americans, Caribbean populations, Hispanics and

Asians

Female > Male

Most common between 20-40 year olds

How is SLE diagnosed?How is SLE diagnosed?

Labs

Symptomatology

Patients must meet 4/11 criteria

1.Malar Rash 2.Discoid Rash1.Malar Rash 2.Discoid Rash

3. Photosensitivity 3. Photosensitivity 4.Mucocutaneous Ulcers4.Mucocutaneous Ulcers

5. Arthritis 6. Serositis5. Arthritis 6. Serositis

7. Renal Disorder 7. Renal Disorder 8. Neurologic Disorder8. Neurologic Disorder

Neuropsychiatric Neuropsychiatric Systemic Lupus Erythematosus (NPSLE) Systemic Lupus Erythematosus (NPSLE)

(95 vs 80%)(95 vs 80%)CNS (diffuse & central)

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

PNS

Neuropathies Acute inflammatory

demyelination

Brunner, H & Klein-Gitelman, M; Rheumatologist Vol 3:3, March 2009

9. Hematologic 10. Immunologic9. Hematologic 10. Immunologic

Anti- DNALeukopenia

ThrombocytopeniaLymphopenia

ANA Interpretation

1:40 1:160 1:320 1:640 1:1280 1:25601:80

Lab TestsLab Tests

ANAENA Ro SSA

La SSBDNASmRNPJo-1

HistoneScl-70Antiphospholipid

antibodyLupus AnticoagulantComplement

SymptomsSymptoms

• Fever and fatigue 42%

• Alopecia 18%

• Lupus Nephritis 40-60%

Causes of DeathCauses 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 LifeQuality 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

0%

5%

10%

15%

20%

25%

30%

AA Hispanic CaucasianJolly, J Rheumatol 2005

TreatmentTreatment

NephritisNephritis

DAMP AS RHINODAMP 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

ReferencesReferences 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.

SummarySummary

Due to variety of symptoms, must know entire history

Great imposterRefer to Rheumatology if SLE suspectedIn SLE watch closely as things change

fast and CVD is a real concernMonitor for QOL issues

Resources for PatientsResources 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/