Systemic Lupus Erythematosus

Question 0

Pathophysiology of SLE

Answer 0

1. Autoantibodies agains normal body components, results from hyperactivity of B cells (humoral immune response) and because of disordered T cell function (cellular immune response).
2. Autoantibodies react with an antigen to form immune complexes, which are then deposited in the connective tissue of blood vessels, lymphatic vessels, & other tissues -> inflammatory response-> inflammation-> local tissue damage.
   Kidneys, musculoskeletal system, brain, heart, spleen, lungs, GI tract, skin, and peritoneum.

Question 1

Systemic Lupus Erythematosus

Answer 1

1. Chronic, inflammatory, connective tissue disease of unknown origin that affects almost all body systems.
2. Characterized by remissions and exacerbations
3. Majority of cases diagnosed during teenage and early adult years.
4. Most characteristic autoantibodies produced against Nucleic acids (DNA), histones etc.

Question 2

Etiology of SLE:

Answer 2

1. Genetic- certain human leukocyte antigen genes
2. environmental- viruses, bacterial agents, chemical drugs, UV light
3. hormonal factors- sex hormones, women have reduced levels of androgens. Estrogens have been shown to enhance antibody responses & have an adverse effect in clients with SLE

Question 3

Risk Factors for SLE

Answer 3

1. Women 9:1 with men
2. Women of childbearing age
3. More common in AA, Hispanics, Asians than in Caucasians
4. Drugs Induced- Procainamide, hydralazine, isoniazid- usually resolve when med is discontinued.

Question 4

3 major classifications of SLE

Answer 4

1. Systemic lupus
2. Drug induced lupus
3. discoid lupus

Question 5

Systemic Lupus

Answer 5

1. Involves one or more of the following systems: Cardiovascular, central nervous, Hematological, kidneys, lungs, and musculoskeletal.
2. Renal manifestations- proteinuria, cellular casts, nephrotic syndrome.
3. hematologic- anemia, leukopenia, thrombocytopenia
4. Cardiovascular- pericarditis, vasculitis, Raynaud’s phenomenon
5. CNS- transient nervous system involvement- decline in intellect, memory loss, disorientation, psychosis, seizures, depression, and stroke, conjunctivitis, photophobia, and transient blindness- renal vasculitis
6. GI- anorexia, nausea, abd pain, diarrhea
7. Liver may be enlarged.

Question 6

Drug Induced Lupus

Answer 6

1. associated with antineoplastic drugs, isoniazid (INH), hydralazine (Apresoline), and others.
2. Symptoms generally subside after drugs are discontinued.

Question 7

Discoid Lupus

Answer 7

1. Limited to the skin- characteristic red butterfly rash across the cheeks and bridge of the nose.
2. Photosensitive- diffuse, maculopapular rash on skin exposed to the sun
3. discoid lesions- raised, scaly, circular lesions w/erythematous rim
4. hives, Alopecia- hair usually grows back

Question 8

Leading cause of Death in SLE

Answer 8

1. with active disease there is an increased risk for infections which are often opportunistic and severe
2. Pneumonia and Septicemia are the leading causes of death, followed by effects of renal or CNS involvement

Question 9

Manifestations of SLE similar to RA

Answer 9

1. Early s/s mimic those of RA- fever, loss of appetite, malaise, and weight loss, and musculoskeletal manifestations- multiple arthralgias and symmetric polyarthritis.
2. 90% have joint symptoms, synovitis may be present, but is RARELY deforming.

Question 10

Common manifestations of SLE

Answer 10

1. Painful or swollen joints and muscle pain
2. Unexplained fever
3. Red rash, especially on the face (Late Stage)
4. Unusual loss of hair
5. Pale, cyanotic fingers and toes
6. Sensitivity to the sun
7. edema in legs and around eyes
8. Ulcers in the mouth
9. Enlarged glands
10. Extreme fatigue

Question 11

Raynaud’s Phenomenon

Answer 11

Raynaud phenomenon manifests as recurrent vasospasm of the fingers and toes and usually occurs in response to stress or cold exposure. Resulting in discoloration of the fingers, toes, and occasionally other areas.
Cold hands, bluish pigmentation..

Question 12

Conception and SLE

Answer 12

1. Women who conceieve while in remission appear to have little risk for adverse outcomes.
2. Increased risk for spontaneous abortion, still birth, prematurity, and intrauterine growth retardation. Infants may have characteristic skin rash which usually disappears by 12 months.
3. Increased risk for congenital heart block- no treatment exists

Question 13

Diagnostic Tests:

Answer 13

1. Anti-DNA antibody testing- rarely found in any other disorder
2. Erythrocyte sedimentation rate
3. Serum complement levels- decreased if used up by development of antigen-antibody complexes
4. CBC
5. Urinalysis- proteinuria, hematuria, blood cell casts
6. Kidney biopsy

Question 14

Pharmacologic Therapies:

Answer 14

1. Aspirin & NSAIDs - anti platelet helps prevent thrombosis, but may cause liver toxicity and hepatitis
2. Antimalarial drugs- skin & arthritic manifestations
3. corticosteroid- severe life threatening

Question 15

Hydroxychloroquine (Plaquenil)

Answer 15

1. Antimalarial drug
2. effective in reducing frequency of acute episodes of SLE
3. Renal toxicity and severe blindness (irreversible)
4. Opthalmologic examination every 6 months

Question 16

Prednisone

Answer 16

1. Corticosteroid
2. 40-60 mg perday
3. may require long term
4. increased risk for cushingoid effects, weight gain, hypertension, infection, accelerated osteoporosis, and hypokalemia.

Question 17

Immunosuppressant Therapy

Answer 17

1. Azathiprine (Imuran), Cyclophosphamide (Cytoxan), Cyclosporine (Sandimmune)
2. decrease proliferation of cells within immune system
3. usually administered w/ corticosteroid
4. Risk for infection, malignancy, bone marrow depression, and toxic effects
5. Monitor CBC, WBC, BUN, creat, liver enzyme levels
6. have entire family receive flu shot*

Question 18

Immunosuppressant Patient Education:

Answer 18

1. Avoid large crowds- infections
2. Report S/S of infection
3. Use contraceptive- birth defects
4. Avoid aspirin or ibuprofen- bleeding risk
5. Menstruation may stop
6. Report difficult breathing or cough

Question 19

Pt. education on photosensitivity:

Answer 19

1. caution to avoid sun exposure 100%!
2. use sunscreesn w/ SPF 15 or higher apply 30 min. before going outdoors.
3. Avoid being out doors during hours of greates sun intensity (10am-3pm)
4. Reapply sunscreen after swimming, exercising, or bathing
5. Wear loose clothing and long sleeves and whide-brimmed hats when outdoors
   Avoid use of oral contraceptives- estrogen can trigger acute episode.

Question 20

Complementary Therapies:

Answer 20

1. Exacerbations of SLE linked to stress

2. Stress reducing techniques- guided imagery, yoga, massage, aroma therapy.

Question 21

Nursing Diagnosis:

Answer 21

1. Risk for ineffective management of therapeutic regimen RT complexity of therapeutic regimen
2. Risk for ineffective tissue perfusion (renal) RT interrupted blood flow to kidneys
3. Risk for impaired skin integrity RT immunologic deficit
4. Chronic pain RT joint inflammation and injury
5. Disturbed body image RT changes from disease & medication
6. Risk for activity intolerance RT chronic disease

Question 22

1 Nursing Diagnosis

Answer 22

1. Impaired Skin Integrity