Systemic Lupus Erythematosus

Systemic Lupus Erythematosus (SLE)

Systemic Lupus Erythematosus

Systemic Lupus Erythematosus is a chronic autoimmune disease in which the immune system attacks its own tissues, causing widespread inflammation and tissue damage in the affected organs.

 It can affect the joints, skin, brain, lungs, kidneys, and blood vessels.

Causes of Systemic Lupus Erythematosus

Idiopathic, but believed to result from a combination of genetics, hormones and the environment. It appears that people with a genetic predisposition to the condition may develop it when they come in contact with potential triggers in the environment, these triggers may include;

  • sunlight
  • infections
  • medications
  • physical or emotional stress
  • female hormone may play a role in causing the condition, this explains why the condition is 8 times more common in women, especially of child-bearing age.

Pathophysiology of Systemic Lupus Erythematosus

The pathophysiology of SLE may be related to the cell death process, which is called apoptosis.

Apoptosis may not be occurring as it is supposed to be, which leads to an immune system attack, and the creation of antinuclear antibodies. These antinuclear antibodies are formed because the immune system sees the proteins found in the nucleus of the cells as nuclear antigens. Normally, when a cell gets sick or old it’s time to die will have approached, and the normal process that it follows to die and get cleared out of the body is called apoptosis, also known as Programmed Cell Death.

In patients with lupus, cells begin to die when the patient is exposed to SLE risk factors such as UV Light. These risk factors cause damage to the body cell, badly damaging the cell DNA material leading to programmed cell death ( apoptosis). After cell death, it produces tiny apoptotic bodies that expose the inside of the dead cell, especially the nucleus, its DNA, histone and proteins too the rest of the body.

So, the immune system of the patient ( susceptibility genes) have an effect on this patients immune system that they take the apoptotic bodies to be nuclear foreign bodies or nuclear antigen (since they are from the nucleus of the apoptotic cell), hence the body starts an attack and also the susceptibity genes reduce the bodies ability to clear the apoptotic particles leading to an increase of these nuclear antigens.

The body’s B-Cells start the production of Anti-bodies(Antinuclear Antibodies) against these nuclear antigens, and so the antinuclear antibodies bind to the nuclear antigens forming Antigen-Antibody Complexes.

These Complexes get access to the blood stream and stick to blood vessel walls, and all other body organs and tissues like the kidney, the skin, joints and the heart. These deposited complexes initiate a local inflammatory reaction which causes damage to the complement system, which after a cascade of activation leave cells with channels that let fluid and molecules in and out of the cell, causing the cell to burst and die. This damage should normally affect foreign or infected cell, but in SLE, it affects normal cells leading tissue damage.

This tissue damage due to immune complexes it is referred to as a TYPE 3 HYPERSENSITIVE REACTION.

If the patient develops antibodies targeting other cells like Red and White blood cells, and phospholipid molecules, which can mark them for Phagocytosis and Destruction, this, then, is a TYPE 2 HYPERSENSITIVITY REACTION.

Systemic Lupus Erythematosus signs and symptoms unmeb

Clinical Feature of Systemic Lupus Erythematosus

 Often there are periods of illnesses called flares and periods of remission where there are only a few symptoms.

Symptoms may be mild or severe, may come on slowly or develop suddenly, and may be temporary or permanent. The symptom experienced will depend on the body system affected by the disease.

The most common symptoms include;

  • fever
  • fatigue
  • shortness of breath
  • chest pain
  • dry eyes
  • a facial rash that covers the bridge of the nose and the cheeks which is called a butterfly rash
  • headaches
  • confusion and memory loss
  • fingers and toes that turn white or blue with cold exposure or during stressful periods known as Raynaud’s phenomenon
  • hair loss, Diffuse thinning or hair fragility with visible broken hairs (in the absence of other causes)
  • anemia
  • joint swelling and joint pain
  • unusual sensitivity to sunlight.

Diagnosis and Investigations

No single test can establish a diagnosis, the combination of blood and urine tests imaging tests biopsy and SLE criteria established by the American rheumatism association may help improve the accuracy of the diagnosis.

  • blood and urine tests :  may include complete blood count, urinalysis, erythrocyte sedimentation rate, antinuclear antibody or a ANA test.
  • imaging tests such as chest X-ray, echocardiogram

Systemic Lupus International Collaboration Criteria (SLICC) for the diagnosis of SLE

With the criteria set by the American Rheumatism Association a person has the disease If any four(4) out of the eleven(11) symptoms are present simultaneously or on two separate conditions.

  1.  malar rash: over the cheeks of the face
  2.  discoid skin rash or red patches on the skin that can cause scarring 
  3. photosensitivity: skin rash as a result of unusual reaction to sunlight.
  4. oral ulcers sores or ulcers of the lining of the mouth or throat 
  5. arthritis 
  6. pleuritis or inflammation of the membrane around the lungs or pericarditis inflammation of the lining tissue around the heart 
  7. kidney abnormalities more than 0.5 grams per day amounts of protein in urine or clumps cellular elements called Casts seen in urine under a microscope
  8. neurologic disorder manifested by seizures or psychosis
  9. antinuclear antibody or positive ANA antibody testing
  10. immunologic disorder or abnormal immune tests including positive anti Smith anti dsDNA or false positive serological test for syphilis
  11. blood hematologic disorder such as low red blood count or white blood count treatment

Management of Systemic Lupus Erythematosus.

Aims

  • Ensure long-term survival
  • Achieve the lowest possible disease activity
  • Prevent organ damage
  • Minimize drug toxicity
  • Improve quality of life
Nurse’s Role in the management Systemic Lupus Erythematosus

L.U.P.U.S

L stands for Labs to help diagnose and monitor flares

Antibody Labs:

  • Positive ANA (anti-nuclear antibodies)
  • Anti-dsDNA (anti-double stranded DNA anti-body)
  • Anti-Sm antibody (Anti-Smith antibody)
  • Elevated ESR (erythrocyte sedimentation rate) and CRP (c-reactive protein)
  • CBC, metabolic panel etc.

U stands for Use Medications: to decrease occurrence of flares, protect organs/tissues/joints from damage, and improve quality of live.

Steroids: Prednisone, Prednisolone etc.

  • Decreases inflammation quickly, but causes side effects
  • Used when patient is not experiencing relief from other medications (severe cases)

NSAIDs (non-steroidal anti-inflammatory drugs): Ibuprofen, Naproxen etc.

  • Decreases inflammation (helpful with fever, joint pain)

Antimalarial: Hydroxychloroquine (very common medication for patients with severe lupus)

  • Decreases antibodies attacking the body, helps control the disease long term (preventing flares)

Immunosuppressant: Azathioprine or Mycophenolate Mofetil etc.

  • Suppresses immune system (increases risk for infection and certain cancers)
  • For severe cases of lupus and sometimes you may hear them referred to as “steroid-sparing” meaning the use of them helps lower the amount of steroids the patient may have to take. 
  • Educate about preventing infection and monitoring self for infection because the medication regime for lupus (example: taking steroids as well) can prevent the signs and symptoms of infection appearing (example: fever).
  • Vaccines up-to-date but no live vaccines (shingles, MMR, intranasal flu, smallpox, rotavirus)

Biologics: Belimumab

  • Binds with a protein that supports the activity of B-cells to decrease the activity of B-cell ,result: decreases antibody attacks and decreases inflammation
    • No LIVE vaccines

P for Pregnancy (many patients are childbearing age and need education):

Women who want to become pregnant need to make sure their lupus has been in control for at least 6 months before conceiving. Pregnancy and the post-partum period can cause flares.

U for Understanding Flares

Triggers: sunlight, stress, sickness, not taking medications correctly or needing an adjustment

Prevention: “LESS” Flares

  • Lower stress (avoid overworking, emotional, illness, and use techniques to help prevent stress)
  • Exercise (helps joints and manages weight)
  • Sleep (need more than 8 hours to prevent the body from getting too exhausted)
  • Sun Protection (sunscreen and large-brimmed hats…sunlight can activate a flare)

S for Signs of a flare of lupus: educate patient to keep diary of symptoms to monitor for flares

  • Fatigue
  • Low grade fever
  • Achy joints
  • Rash
  • Edema of the legs and hands
Medical Management

The treatment choice for systemic lupus erythematosus (SLE) is varied based on the severity of the disease and symptoms:

  • Mild cases are defined as disease pattern with one or two organ involvement.
  • Moderate cases are defined as more than 2 organ involvement during disease flares with low grade of involvement and complications or one or two organ involvement with more extensive involvements.
  • Severe cases are defined as presentation of the disease with life threatening complications and multiple (more than 2) organ involvements.
  • Hydroxychloroquine: 200 to 400 mg daily as a single daily dose or in 2 divided doses. Generally, all patients with any type of SLE manifestation should be treated with hydroxychloroquine regardless of the severity of the disease.

In most cases,

  •  Hydroxychloroquine PO 200 to 400 mg daily as a single daily dose or in 2 divided doses AND methylprednisolone as intravenous  0.5 to 1 g/day for three days in acutely ill patients, or 1 to 2 mg/kg/day in more stable patients
  • Alternatively; Hydroxychloroquine PO 200 to 400 mg daily as a single daily dose or in 2 divided doses AND prednisone oral; 40-60 mg/day.

Fever management

  •  Celecoxib PO 100 to 200 mg twice daily
  • Alternatively; Acetaminophen 1000 mg every 6 hours; maximum daily dose: 3000 mg daily 

Raynaud’s phenomenon treatment

  • Calcium channel blocker (nifedipine) 10 to 30 mg 3 times daily

Chronic pain management

  • Moderate pain should be treated with mild prescription opiates such as:
    • Co-codamol (Acetaminophene+opioid): Acetaminophen (300 to 1,000 mg/dose)/codeine (15 to 60 mg/dose) every 4 hours as needed; adjust dose according to severity of pain and response of patient (maximum: acetaminophen 4,000 mg/codeine 360 mg per 24 hours)
  • Moderate to severe chronic pain should be treated with stronger opioids such as:
    • Hydrocodone: Single doses >40 mg or >60 mg with a total daily dose ≥80 mg

Cutaneous lupus erythematosus

  • High potency topical steroid twice daily for patients with CLE 
    • Hydrocortisone 1% or 2.5% for facial involvement.

Lupus nephritis treatment

  • Aggressive antihypertensive therapy with blood pressure goal of 130/85
  • In patients with proteinuria, antiproteinuric therapy with blockade of the renin-angiotensin system include ACEIs and ARBs:
    • ACE inhibitors; captopril PO 25 mg 3 times daily
    • ARBs; losartan PO initial: 50 mg once daily; can be increased to 100 mg once daily based on blood pressure response.

Other considerations.

    • Vitamin D and calcium supplements for preventing osteoporosis in patients using corticosteroids
    • Antihypertensive drugs and statins are also recommended in patients using corticosteroids
Nursing Interventions and Rationales

1. Encourage adequate nutrition and hydration to promote healthy skin and healing in the presence of wounds.

2. Instruct the client to clean, dry, and moisturize intact skin; use warm (not hot) water, especially over bony prominences; use unscented lotion. Use mild shampoo. Scented lotions may contain alcohol, which dries the skin. Prescribed solutions reduce dryness of the scalp and maintain skin integrity.

3. Instruct the client to avoid contact with harsh chemicals and to wear appropriate protective gloves, as needed. Avoid hair dye, permanent solution, and curl relaxers. Chemicals aggravate this condition.

4. Recommended prophylactic pressure-relieving devices (e.g., special mattress, elbow pads). Such devices aid in the prevention of skin breakdown.

For skin rash:

  • Wear protective eyewear.
  • Wear a wide-brimmed hat and carry an umbrella.
  • Avoid ultraviolet rays.

The sun can exacerbate a skin rash or precipitate a disease flare. Special lotions, glasses, and other items may be required to protect the skin from sunlight exposure.

5. Inform the client of the availability of special makeup to cover rashes, especially facial rashes.
These preparations are specially formulated to completely cover rashes, birthmarks, and darkly pigmented areas. This will help the client who is having problems adjusting to body image changes.

6. Introduce or reinforce information about the use of hydroxychloroquine.
This antimalarial drug is a slow-acting medicine used to relieve or reduce inflammation and rash. It may take 8 to 12 weeks to effect. A potential side effect is a retinal toxicity. The client must follow up with an ophthalmologist every 6 months. Topical cortisone medication may likewise be used.

For oral ulcers:

  • Instruct the client to avoid spicy or citrusy foods.
    These foods might irritate fissures or ulcers in the mucous membranes.
  • Instruct the client to rinse the mouth with half-strength hydrogen peroxide three times per day.
    Hydrogen peroxide helps keep oral ulcers clean.
  • Instruct the client to keep ulcerated skin clean and dry. Apply dressings as needed.
    Skin is necessary to prevent infection and promote healing.
  • Instruct the client to apply topical ointments as prescribed.
    Vitamins A and E may be useful in maintaining skin health.

For hair loss:

  • Instruct the client that scalp hair loss occurs during the exacerbation of disease activity.
    Scalp hair loss may be the first sign of impending disease exacerbation. Scalp hair loss may not be permanent. As disease activity subsides, scalp hair begins to regrow.
  • Instruct the client that scalp hair loss may be caused by high-dose corticosteroids (prednisone) and immunosuppressant drugs.
    Hair will regrow as the dose decreases.
  • Encourage the client to investigate ways (e.g., scarves, hats, wigs) to conceal hair loss.
    Hair loss may interfere with lifestyle and self-image.
Complications.
  • Skin scarring
  • Joint deformities
  • Kidney failure
  • Stroke
  • Heart attack
  • Pregnancy complications
  • Hip destruction (also called avascular necrosis)
  • Cataracts
  • Bone fractures
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