A Family Doctor's Tale - SLE

DOC I HAVE Systemic Lupus Erythematosis

Systemic Lupus Erythematosis or SLE  is a rare autoimmune disease which attacks more women than men. 
The butterfly rash on the cheeks gives rise to the name Lupus or wolf appearance of the face. Besides the skin reactions, the disease attacks the whole of the body including the joints, eyes, lungs, blood, etc. Unfortunately the condition is not curable but can be controlled with medications.

Systemic Lupus Erythematosis is a chronic multisystem autoimmune inflammatory disease which attacks the whole body.


It typically has a butterfly erythrematous rash on the face.


It is not contagious.


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.


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


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.


Muscoloskeletal 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:
-----------

1.Retinal hemorrhages and exudates


2.Cytoid bodies


Lungs and Pleura:
-----------------------

1.Recurrent pleurisy


2.Pleural effusions - small


3.Pulmonary infiltrates


Cardiovascular
-----------------

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 wire loop changes and epithelial crescents in kidneys resulting in proteinuria, hematuria and renal failure.


Central nervous system:
------------------------------

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:
-----------------

1.Thrombotic thrombocytopenic purpura


2.Sjogren's syndrome


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, neutropenia, thrombocytopenia, Coombs' test(usually positive)


4.Renal or skin biopsy


5.Xrays of the chest, abdomen and brain


6.MRI of brain, kidney,

Some Complications are:
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


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:
---------------
1.short course of anti inflammatory medications such as NSAID or corticosteroids.


2.Rest and freedom from stress


Severe cases:
--------------------
Patients with serious illness which has affected their organ may require stronger medications:
1.high doses of corticosteroids initially 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 vascularitis.
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.


The prognosis depends on the severity of the disease


Severe cases with renal disease has poorer prognosis.

1.Adequate rest


2. Avoid stress


3. Avoid the sun and use sunscreen.


4.Healthy lifestyle with exercise prevents arthritis