1. Case Study #87: Systemic Lupus Erythematosus (SLE)
By Albert Doyle

2.
Listen to his words and accent

3. Pathophysiology of Lupus
Chronic, autoimmune disease that can affect literally any body system
Rarely presents the same in any two lupus patients
Disruption in apoptosis
Immune system generates auto-antibodies
Antibodies clump together and can latch on to any body system. Attacking cells DNA surrounding the antibody.

4. No know treatment to cure the disease
No known cause
No know treatment to cure the disease
Treatment is aimed at reducing symptoms and balancing the body’s immune system to a functional level
Genetic, epigenetic, hormonal, and other environmental factors associated with SLE

5. Epidemiology 250,000 Americans diagnosed with definite SLE (NADWG).
90% in women of childbearing age
Incidence of SLE in black women 4x higher than that in white women

6. Scenario
D.W. Is a 25-year-old married woman with three children under 5 years old. She came to her physician 7 months ago with vague complaints of intermittent fatigue, joint pain, low-grade fever, and unintentional weight loss. Her physician noted small, patchy areas of vitiligo and scaly rash across her nose, cheeks, back, and chest.

7. D.W.’s laboratory studies revealed:
Positive antinuclear antibody(ANA) titer
Positive dsDNA(positive lupus erythematosus)
Positive anti-Sm(anti-smooth muscle antibody)
Elevated C-reactive protein(CRP)
Elevated erythrocyte sedimentation rate (ESR)
Decreased C3 and C4 serum complement

8. Joint x-ray films demonstrated joint swelling without joint erosion. D
Joint x-ray films demonstrated joint swelling without joint erosion. D.W was subsequently diagnosed with systemic lupus erythematosus (SLE).
She was initially treated with hydroxychloroquine (Plaquenil) 400mg and Deltason (Prednisone) 20mg PO qdaily, bed rest, and ice packs.
D.W responded well to treatment, the steroid was tapered and discontinued, and she was told she could report for follow-up every 6months, unless her symptoms became acute. D.W. Resumed her job in environmental services at a large geriatric facility.

9. 1)What is the significance of each of D.W.’s laboratory findings?
Positive antinuclear antibody (ANA) titer:
Auto-antibodies are in the immune system
Positive dsDNA (positive lupus erythematosus):
Auto-antibodies that target DNA
Highly specific
Positive anti-SM (smooth muscle antibody):
Presence of antibodies against smooth muscle

10. Decreased C3 and C4 serum complement
Elevated C-reactive protein (CRP) and erythrocyte sedimentation rate (ESR)
Measure of inflammation in the body
Decreased C3 and C4 serum complement
Shows impending or current “flare” of lupus symptoms

11. 2)Given that most tests are nonspecific, how is SLE diagnosed?
11 criteria for SLE
Established by the American Rheumatism Association
If individual display 4 or more of these criteria, SLE diagnosis is highly suggested.

12. Malar- purplish/mildly scaly rash over the cheeks and nose
Known as a “butterfly” rash
D.W. displays scaly rash over cheeks, nose, back and chest.

13. Two or more swollen/tender joints
Discoid skin rash
Photosensitivity
Two or more swollen/tender joints
Shown both subjectively and objectively
Brain irritation
Seizures or psychosis (lupus cerebritis)
Mucous membrane ulcers
Pleuritis or pericarditis

14. Low blood counts Kidney abnormalities Tests: Proteinuria
dsDNA, anti-Sm, ANA
ESR and CRP

15. 3) What priority problems were addressed in D. W
3) What priority problems were addressed in D.W.’s care plan at that time of diagnosis?
Coping and understanding of dx
Monitor pain and temp
Medication administration and understanding
Ways to live a normal and safe life dx. w/ SLE

16. Progress:
Eighteen months after diagnosis, D.W. Seeks out her physician because of puffy hands and feet and increased fatigue. D.W. Reports that she has been working longer hours because of the absence of two of her fellow workers.

17. Laboratory Tests at this time:
Laboratory Test Results (8 months after dx):
Sodium
129mmol/L
Norm =
Potassium
4.2mmol/L
Norm=
Chloride
119mmol/L
Norm=96-106
Total CO2
21mmol/L
Norm=20-29
BUN
34mg/dL
Norm=8-20
Creatinine
2.6mg/dL
Norm=
Glucose
123mg/dL
<140
Urinalysis
2+ protein
1+ RBCs

18. 4) Which laboratory findings concern you, and why?
Elevated BUN and Creatinine
Proteinuria and hematuria
Slightly elevated sodium and chloride

19. 5) How will D.W.'s treatment and care plan likely change?
Stabilize labs
Decrease swelling and fatigue
Medication adjustment
Promote kidney function
Decrease risk of infection

20. 6) The physician orders cyclophosphamide (Cytoxan) 100mg/m2/day orally in two divided doses. D.W. Weighs 140 lbs and is 5ft, 4in tall. How much will she receive with each dose?

21. So what was that med? cyclophosphamide (Cytoxan)
Aklylating agent used for immunosuppressant therapy
Used for D.W.’s worsening symptoms of SLE

22. Progress:
D.W. Is seen in the immunology clinic twice monthly during the next 3 months. Although her condition does not worsen, her BUN and creatinine remain elevated. While at work one afternoon, D.W. Beings to feel dizzy and develops a severe headache. She reports to her supervisor, who has her lie down. When D.W. Starts to become disoriented, her supervisor calls 911, and D.W. Is taken to the hospital. D.W. Is admitted for probable lupus cerebritis related to acute exacerbation of her disease.

23. 7) What other findings indicative of central nervous system (CNS) involvement from SLE should D.W. Be assessed for?
Seizures
Decreased LOC
Slurred speech
PERRLA
A&O
Muscle tone
Symmetry

24. 8) What preventive measures need to be instituted to protect D. W
8) What preventive measures need to be instituted to protect D.W. At this time?
Seizure precautions
Infection control
Standard precautions
IV antibiotics
Close monitoring
Neurological assessments

25. 9) While caring for D.W., which of these care activities can be safely delegated to the NAP? (select all that apply):
a: Measuring D.W.'s BP every 2hrs
b: Assisting D.W. With personal hygiene measures
c: Counseling D.W. On seizure safety precautions
d: Assessing D.W.'s neurological status every 2hrs
e: Emptying the urine collection device and measuring the output
f: Monitoring D.W.'s BUN and creatinine levels
A,b,e

26. Progress:
The physician orders pulse therapy with methylprednisolone (Solu-Medrol) 125mg IV every 6 hours and plasmapheresis once daily.

27. 10) What major complications associated with immunosuppression therapy will D.W. Have to be monitored for?
Increased risk of infection
Change in VS
N/V, bone marrow suppression, hair loss, lethargy

28. 11) D.W. Asks about what plasmapheresis does and why might it help her feel better. Describe how you would respond.
Blood is taken out, plasma is filtered out, replaced with other fluids, and replaced back into the body.
Plasma contains inflammatory antibodies and other immunologically active substances

29. 12) D. W. Returns to the floor following plasmapheresis
12) D.W. Returns to the floor following plasmapheresis. The NAP reports to you D.W.'s vital signs.
BP=86/43, HR=108, RR=18, Temp=97.2(36.2)
You go in to assess D.W. And find that she is complaining of a headache and some dizziness.

30. What do you immediately suspect is occurring?
BP=86/43, HR=108, RR=18, Temp=97.2(36.2)
Hypotensive- from plasmapheresis
Dehydrated

31. 13) What do you anticipate your care of D. W
13) What do you anticipate your care of D.W. Will include over the next 2-3hrs?
Stabilizing BP
Hydrating
Decrease dizziness and headache
Stabilize temp
Assessing neurological signs and VS
Keeping doctor aware of any complications

32. 14) What outcome criteria would support that D. W
14) What outcome criteria would support that D.W.'s condition is stabilizing?
Stable VS
A&O x4
Decreased report of pain and swelling extremities
Decrease in fatigue
Decrease in inflammation
Fight off infection

33. Self-care with SLE and kidney complications
15) Identify at least five topics that D.W. must be taught before she is discharged that might help her lead as normal a life as possible.
Medication regimen
Coping
Proper hygiene
Self-care with SLE and kidney complications
When to notify health care provider

34. 16) You note that D. W. 's husband is visiting her this afternoon
16) You note that D.W.'s husband is visiting her this afternoon. You enter the room to as whether they have any questions. D.W.'s husband states, “I have tried to tell her that she cannot go back to work. Sure, we need the money, but the kids and I need her more. I’m afraid that this lupus has weakened her whole body and it will kill her if she goes back to work. Is that right?” How should you respond to his concerns?

35. Further Research: Main cause of SLE
Treatments aimed at curing the disease rather than managing it
grasp further understanding for health care workers to take the best care possible for SLE patients

36. Citations:
Ramachandran, R. R., Sakhuja, V. V., Jha, V. V., Kohli, H. S., & Rathi, M. M. (2012). Plasmapheresis in systemic lupus erythematosus with thrombotic microangiopathy. Internal Medicine Journal, 42(6), 734. doi: /j x
Robinson, M., Sheets Cook, ,., & Currie, L. M. (2011). Systemic lupus erythematosus: A genetic review for advanced practice nurses. Journal Of The American Academy Of Nurse Practitioners, 23(12), doi: /j x
Ferenkeh-Koroma, A. (2012). Systemic lupus erythematosus: nurse and patient education. Nursing Standard, 26(39),
Poole, J. L., Hare, K., Turner-Montez, S., Mendelson, C., & Skipper, B. (2014). Mothers With Chronic Disease: A Comparison of Parenting in Mothers With Systemic Sclerosis and Systemic Lupus Erythematosus. OTJR: Occupation, Participation & Health, 34(1), doi: /
Christian Pagnoux, C. P. (2007). Plasma exchange for systemic lupus erythematosus. Transfusion and Apheresis Science , Retrieved from
Kang, I., & Park, S. H. (2003). Infectious complications in SLE after immunosuppressive t herapies. Current opinion in rheumatology, 5, 528–534.
Hari, P., & Srivastava, R. N. Pulse corticosteroid therapy with methylprednisolone or dexamethasone. Indian journal of pediatrics, 4, 557–560.