By the end of the session the student will be enabled to:

Discuss with understanding the etiologyand pathophysiology of Systematic Lupus Erthematosus (SLE)

Demonstrate theoretical knowledge applied to nursing and collaborative care management of patients with SLE.

• Chronic, progressive multisystem

inflammatory autoimmune disease periods

of exacerbations & remission

• Multifactorial origins

• Genetic

• Hormonal

• Environmental

• Immunologic

• Women are ten times more likely to

develop this than men

Women more likely to develop this than men

Menarche - oral contraceptives

Typically affects skin joints and serous membranes

Genetic influence is suspect

Hormonal influence Higher risk amongst non

white population Genes from HLA complex

show strong associations with SLE

Hormones known to play a role in the development of SLE

Environment• Sun exposure and burns

• ? Infectious agents Anti seizure drugs Procainamide (Pronestyl) Heart -decreases the speed of

electrical conduction through the heart muscle, prolongs the electrical phase during which the heart's muscle cells can be electrically stimulated, and prolongs the recovery period during which the heart muscle cells cannot be stimulated.

Hydralazine (apresoline) is a direct-acting smooth muscle relaxant used to treat hypertension by acting as a vasodilator primarily in arteries and arterioles.

Hydralazine (Apresoline)Anti Hypertensive

Production of a large variety of auto

antibodies, erythrocytes, coagulation

proteins, lymphocytes, platelets and

many other self proteins

Autoimmune reactions directed against

the constituents of the cell nucleus.

Over aggressive autoimmune response

related to the actions of the B and T cells.

Ranges in severity mild affecting all

body systems

Any organ can be affected by the

circulating immune complexes.

Most commonly affected are the skin ,

muscle, serous membranes, heart ,lung

linings, kidneys and nervous tissue

Weight loss

Fever

Arthralgia (joint stiffness and pain)

Excessive fatigue

Palmar erythema Butterfly rash

Severe rashes in those who are

photosensitive in sun exposed areas

Classic butterfly rash 50% of people

Persistent lesions round coin shaped

Ulcers of the oral and/or nasal

membranes

Alopecia

Dry, scaly and atrophied scalp

Photosensitivity

Polyarthralgia, morning stiffness

Arthritis – may cause swan neck deformities, ulnar deviation andsubluxation.

Increased risk of bone loss and fracture

Tachypnea and cough Restrictive lung disease Possible pleurisy Cardiac dysrhythmias from fibrosis of sino arterial and atrio

ventricular nodes Pericarditis may occur Hypertension Hypercholesterolemia Secondary anti phospholipid syndrome Antiphospholipid syndrome is a disorder in which your

immune system mistakenly produces antibodies against certain normal proteins in your blood. Antiphospholipidsyndrome can cause blood clots to form within your arteries or veins as well as pregnancy complications, such as miscarriages and stillbirths.

Hypercholesterolemia (also spelled hypercholesterolaemia) is the presence of high levels of cholesterol in the blood. It is a form of "hyperlipidemia.

Lupus Nephritis (LN)

Mild proteinuria Glomerulonephritis

Primary goal: Slow the progression by

giving corticosteroids and immuno

suppressants

Generalized or focal seizures

Peripheral neuropathy

Cognitive dysfunction – deposition of

immune complexes within the brain

tissue.

Presents as mood disorders, psychiatric

problems

Potential for stroke /aseptic meningitis

Headaches

Anemia

Mild leukopenia

Thrombocytopenia

Either excessive bleeding or

blood clot development -Blood

clot development treated with

WARFARIN (coumadin)

Increased susceptibility to infection

Defects in the ability to phagocytize

invading bacteria

Pneumonia most common infection

Fever serious indictor life

threatening problems

Avoid live vaccination (e.g. varicella)

although other vaccines are safe

No specific test

H & P (Table 65-14)

Labs

+ANA (antinuclear antibodies)

Antibodies (e.g. anti-DNA)

Specific for SLE

Anti-double-stranded DNA

Anti-Smith

Early diagnosis better prognosisManage exacerbation periodPrevent complications of treatmentSurvival depends on:

• Age

• Race

• Gender

• Socioeconomic status

• Co-morbidity

• Severity of disease

NSAIDs - Polyarthralgias/PolyarthritisAntimalarial drugs (hydroxychloroquine

Plaquenil) → Fatigue/Skin & joint problemsNB: Funduscopic & visual field exams q6- 12 months → Retinopathy

Alternative: Antileprosy drug (e.g. Dapsone)

Anticoagulants (e.g. Coumadin/Heparin) for Clotting

Corticosteroids (Methylprednisolone) → Polyarthritis & Cutaneous SLE

Steroid-sparing drugs (Methotrexate) with Folic Acid Immunosuppressive drugs (azathioprine Imuran &

cyclophosphamide Cytoxan to reduce long-term use of corticosteroids & organ-system

disease NSAIDs continue to be an important intervention, especially for

patients with mild polyarthralgias or polyarthritis. Antimalarial agents such as hydroxychloroquine (Plaquenil) often

are used to treat fatigue and moderate skin and joint problems. Steroid-sparing immunosuppressants such as methotrexate can

serve as an alternate treatment and are prescribed in combination with folic acid to decrease minor side effects of corticosteroids.

Immunosuppressive drugs such as azathioprine (Imuran) and cyclophosphamide (Cytoxan) may be prescribed to reduce the need for long-term corticosteroid therapy.

Assess patient’s physical, psychologic, &

sociocultural problems with long-term

management of SLE

Subjective & Objective data (Table 65-16)

Specific considerations

Pain

Fatigue

Fatigue

Acute pain

Impaired skin integrity

Overall goals

Pain relief

Adhere to therapeutic regimen

Demonstrate awareness of & avoid

activities that cause disease exacerbation

Maintain optimal role function & a

positive self-image

Accurate recording of severity of

symptoms & response to therapy

Assess• Fever pattern

• Joint inflammation

• Limitation of motion

• Location & degree of discomfort

• Fatigability

36-year-old woman was admitted 8 years ago with polyarthritis, facial & palmar erythema, and general malaise.

She was diagnosed w/ probable systemic lupus erythematosus (SLE)

She was started on prednisone 100 mg/every other day

Within a few weeks of taking prednisone, she developed cushing’s syndrome

She has also had intermittent tonic - clonic(grand mal) seizures that are treated w/ Dilantin

During the past year, her lab studies indicate early renal failure

She has had occasional UTIs that have responded to treatment

1. What common clinical manifestations of SLE does she have? Polyarthritis, facial and palmar erythema, and general malaise.

2. What psychosocial issues should you discuss w/ her? Concerns over her long-term prognosis, family planning, consultation about managing rash, and stress management.

3. What patient teaching should you do w/ her? Discuss the avoidance of triggers (e.g., sun exposure, stress)