Systemic Lupus Erythematosus

Safaa EID

Medical surgical and critical care department

Definition

• is an autoimmune disease of the body's connective tissues.

• Autoimmune means that the immune system attacks the tissues of the body.

• attacks parts of the cell nucleus.

• between the ages of 15 and 40, although it can show up at any age.

Pathogenesis

• A healthy immune system produces proteins called antibodies and specific cells called lymphocytes.

• that help fight and destroy viruses, bacteria, and other foreign substances that invade the body.

• In lupus, the immune system produces antibodies against the body's healthy cells and tissues.

• These antibodies, contribute to the inflammation of various parts of the body and can cause damage to organs and tissues.

• Lupus can affect

-joints, skin, kidneys, heart, lungs, blood vessels, and brain.

Causes

The exact causes is not known.

• Inherited genes, viruses, ultraviolet light, and drugs may all play some role.

• Genetic factors increase the tendency of developing autoimmune diseases

• female hormones play an important role in the expression of SLE.

• a key enzyme's failure to dispose of dying cells may contribute the development of SLE.

• a genetic mutation

• Drug-induced lupus. ( hydralazine, beta-blokers, isoniazid) .

There are several kinds of lupus:

Discoid lupus erythematosus is

a chronic skin disorder red, raised rash appears

on the face, scalp, or elsewhere

The rash may last for days or years .

Sub acute cutaneous lupus erythematosus refers to skin lesions that appear on parts of the body

exposed to sun. The lesions do not cause scarring.

Drug-induced lupus is a form of lupus caused by medications. Many different drugs can cause drug-

induced lupus

Neonatal lupus is a rare disease that can occur in newborn babies of women with SLE

• Sjögren's syndrome is an autoimmune

disorder in which immune cells attack and destroy the excroin glands that produce

tears and saliva.

• Sjögren's syndrome is also associated with rheumatic disorders such as rheumatoid

arthritis

Secondary sjogren’s syndrome

• Dry eyes

• Dry mouth

Clinical manifestations

• The symptoms of SLE come on in waves, called flares or flare-ups. In between flares, patients may have almost no symptoms.

• general discomfort, extreme fatigue, fever, and weight loss at some point.

“Common Symptoms of Lupus

• Painful or swollen joints and muscle pain

• Unexplained fever

• Red rashes, most commonly on the face

• Chest pain upon deep breathing

• Unusual loss of hair

• Pale or purple fingers or toes from cold or stress .

• Sensitivity to the sun

• Swelling (edema) in legs or around eyes

• Swollen glands

• Extreme fatigue

• Malar rash:

• This is a "butterfly-shaped" red rash over the cheeks below the eyes and across the bridge of the nose.

• It may be a flat or a raised rash.

• The rashes are made worse by sun exposure.

• Raynaud’s phenomenon

• is commonly found in lupus.

• reaction of distal digits to cold or emotion, in which the skin colour changes from white to blue to red)

• Maculopapular eruption

• Vasculitic skin lesion

• Alopecia

• Oral ulcer:

• Painless sores in the nose or mouth need to be observed and documented by a doctor.

Different symptoms in different body systems :

• Kidneys

Inflammation of the kidneys (nephritis)

Lungs

Central nervous system:

• Blood vessels:

• Blood vessels may become inflamed (vasculitis)

• Muscles and Bones:

• joint pain or inflammation.

• The pain can come and go, or it can be long lasting.

• Blood:

• anemia, leucopenia (a decreased number of white blood cells), or thrombocytopenia (a decrease in the number of platelets in the blood, which assist in clotting).

• Heart:

• (myocarditis and endocarditis) or the membrane that surrounds it (pericarditis).

• Digestive System:

• Symptoms include abdominal pain, loss of appetite, nausea, and sometimes vomiting.

• psychological and emotional effects

• such as grief, depression, and anger, depression

• Pregnancy.

• However, the chances of miscarriage, premature birth, and death of the baby in the uterus are high.

Diagnostic studies– Complete blood count (CBC)

– Erythrocyte sedimentation rate (ESR) – Urinalysis – Blood chemistries – Antinuclear antibody test (ANA) – Other autoantibody tests (anti-DNA)

• Skin biopsy • Kidney biopsy

Treatment• A conservative regimen of physical and

emotional rest is required

• protection from direct sunlight

• a healthful diet

• prompt treatment of infections

• pregnancy must be planned for times when the disease is under control, and the patient is on allowable medications.

1-NSAIDs: • Nonsteroidal anti-inflammatory drugs (NSAIDs) such

as ibuprofen and naproxen.

2- Antimalarials: • (Plaquenil). It may be used alone or in combination

with other drugs

3- Corticosteroids:

Medications

4- Immunosuppressives: For some patients whose kidneys or central nervous system are affected by lupus, such as cyclophosphamide

(Cytoxan)

- restrain the overactive immune system by blocking the production of immune cells.

Other therapy

• Plasma exchange

• Intravenous Immunoglobulin

• Stem cell transplantation

• Immune therapy ( anti-IL10, anti-CD20, and immune tolerance therapy)

• Minimize appearance of lesions.• Alleviate discomfort

• Minimize fatigue. • Maintain weight at optimal range

• Teach the patient to recognize fever and signs and symptoms of infection.

• Maintain joint function and increase muscle strength.

Nursing care

– Recognize anemia and develop a plan of care

– Minimize episodes of bleeding.

– Minimize incidence of infection.

– Educate the patient about immunizations – Educate patient nutritional status.

Thanks