A Simple Guide to Systemic Lupus Erythematosis

A Simple Guide to Systemic Lupus Erythematosis
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What is Systemic Lupus Erythematosis?
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Systemic Lupus Erythematosis is a chronic multisystem autoimmune inflammatory disease which attacks the whole body.

It typically has a butterfly erythematous rash on the face.

It is not contagious.

Who is affected by Systemic Lupus Erythematosis?
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Systemic Lupus Erythematosis is more common in women than in men(9:1 ratio)

Most cases begin at the age range of 15-45 years and occurs less frequently between the age of 45-70 years.

It occurs more frequently in blacks than in white people.


What is the Cause of Systemic Lupus Erythematosis?
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The exact cause of Systemic Lupus Erythematosis is not known.

1.It has been suggested that an autoimmune disease is the main cause of Systemic Lupus Erythematosis disease.

The antibodies produced by the body to fight germs starts to attack the body's own healthy tissue.

2.Psychological problems like stress and anxiety is not a cause of Systemic Lupus Erythematosis but has been known to trigger off the disease.

3.Some cases are induced by medications such as :
hydralazine
procainamide
penicillamine
isoniazid

What are the Symptoms and signs of Systemic Lupus Erythematosis?
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The disease may be mild to severe.

There are also periods of remissions and activity.

During the active stage:
Systemic involvement:
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1.fever

2.anorexia

3.weakness

4.weight loss

5.Lymphadenopathy

Skin Mucosa Involvement:
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1.Typical butterfly erythrematous rash on the face

2.pleomorphic erythrematous and maculopapular lesions on the face, neck and extremeties.

3.Vasculitic nail bed and digital pulp lesions

4.Alopecia, petechiae, bullae, and mucous membrane lesions.

Musculoskeletal System:
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1.Polyarthritis especially small joints of fingers

2.Arthritis milder and non erosive compared to rheumatoid arthritis

3.morning stiffness

4.muscle atrophy and weakness may occur

Eyes:
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1.Retinal hemorrhages and exudates

2.Cytoid bodies

Lungs and Pleura:
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1.Recurrent pleurisy

2.Pleural effusions - small

3.Pulmonary infiltrates

Cardiovascular
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1.Pericarditis

2.Endocarditis with vegetation on the mitral valve especially

Gastrointestinal
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1.Non specific nausea

2.Gastrointestinal ulcers, hemorrhage, necrosis

Renal
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1.Focal membranous nephritis

2.Proliferative nephritis - deposit of autoimmune complexes on capillary walls give rise to typical wireloop changes and epithelial crescents in kidneys resulting in proteinuria, hematuria,and renal failure.

Central nervous system:
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1.Lesions in the brain can cause mental disorders( dementia, psychosis,epilepsy), chores, cerebrovascular accidents,cranial nerve lesions.

2.Peripheral neuropathy of various types

Others:
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1.Thrombotic thrombocytopenic purpura

2.Sjogren's syndrome


How do you make the Diagnosis of Systemic Lupus Erythematosis?
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1.A history of facial butterfly rash, systemic symptoms

2.Blood for ESR, positive ANA(antnuclear Antibodies), LE cells

3.Full blood count for anemia, hemolytic anemia, neutopenia, thrombocytopenia, Coombs' test(usually positive)

4.Renal or skin biopsy

5.X- rays of the chest, abdomen and brain

6.MRI of brain, kidney,

What are the complications of Systemic Lupus Erythematosis?
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1.Severe Dermatitis with discoid lesions (maculopapular in nature)

2.Polyathritis with swelling of joints

3.Anemia

4.Pleural effusions

5.hemorrhage

6.Dementia

7.Renal failure

What is the treatment of Systemic Lupus Erythematosis?
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There is no cure for SLE at the present moment.

Treatment is to relieve symptoms and prevent organs from deteriorating.

Patients with mild symptoms may not need any treatment.

Mild Cases:
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1.short course of antiinflammatory medications such as NSAID or corticosteroids.

2.Rest and freedom from stress

Severe cases:
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Patients with serious illness which has affected their organ may require stronger medications:
1.high doses of corticosteroids intially intravenously, followed by oral medication whose dosage is reduced slowly to the minimum possible.

Corticosteroids are particularly helpful if internal organs are involved.

Side effects of corticosteroids include gastric problems, thinning of the bones, infection, facial puffiness, cataracts.

2.NSAIDs can reduce inflammation and pain especially in the musculoskeletal system.

Examples of NSAIDs include aspirin, ibuprofen, naproxen.

The side effects are stomach upset, abdominal pain, ulcers or GIT bleeding.

To reduce the side effects, NSAIDs are usually taken with food or H2 antagonist like cimetidine, ranitidine.

3.Antimalarial medication such as Hydroxychloroquine has been particularly effective for SLE patients with fatigue, skin, and joint disease.

Side effects include diarrhea, upset stomach, and eye pigment changes.

Eye pigment changes are rare, but require monitoring by an ophthalmologist

Other antimalarial drugs, such as chloroquine or quinacrine, are considered

4.Treatment for severe skin disease skin disease include dapsone and retinoic acid (Retin-A).

5.immunosuppressive medications are used for treating patients with more severe manifestations of SLE with damage to internal organ(s).

Examples of immunosuppressive medications include methotrexate, azathioprine (Imuran), cyclophosphamide, and cyclosporine .

Side effects are depression of blood cell counts and increased risks of infection and bleeding.

6. mycophenolate mofetil (Cellcept) is very effective in lupus with kidney disease.

It has been able to reverse active lupus kidney disease and maintain remission.

It's lower side effects made it more useful than immunosuppresive drugs.

7.plasmapheresis is used to remove antibodies and other immune substances from the blood to suppress immunity.
It has also help to to remove proteins (cryoglobulins) that can lead to vasculitis.
SLE patients with low platelet levels can have severe bleeding.

7.rituximab is an intravenously infused antibody that suppresses a particular white blood cell, the B cell, by reducing the number in the blood stream.
B cells play a central role in lupus activity, and when suppressed, the disease tends toward remission.

8.omega-3 fish oils could help patients with lupus by decreasing disease activity and possibly decreasing heart disease risk.

9.rest during periods of active disease
Poor sleep quality can cause fatigue in patients with SLE.
Sleep quality and the effect of underlying depression, lack of exercise can have an adverse effect on the health of SLE patients.
It is important to maintain muscle tone and range of motion in the joints.

10.Endstage kidney damage from SLE requires dialysis and/or a kidney transplant.


What is the prognosis of Systemic Lupus Erythematosis?
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The prognosis depends on the severity of the disease

Severe cases with renal disease has poorer prognosis.


How is SLE prevented?
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1.Adequate rest

2. Avoid stress

3. Avoid the sun and use sunscreen.

4.Healthy lifestyle with exercise prevents arthritis