2. What is SLE?
Systemic lupus erythematosus (SLE) is an autoimmune disease in which
organs and cells undergo damage initially mediated by tissue-binding
autoantibodies and immune complexes.
SLE can affect any part of the body, but most often harms the heart, joints,
skin , lungs , blood vessels , liver, kidneys, and nervous system.
Characterized by remissions and exacerbations
3. Prevalence
90% of cases are of women
Prevalence rate is between 15 and 65 years of age
Symptoms occur between 15 and 25 years
Prevalence in general population is 1 in 1000
People of all genders, ages, and ethnic groups are susceptible.
Highest prevalence is in African-American and Afro Caribbean women, and
lowest prevalence is in white men.
5. Environmental
Exposure to ultraviolet light causes flares of SLE in approximately 70% of patients, possibly
by increasing apoptosis in skin cells or by altering DNA and intracellular proteins to make
them antigenic.
Some infections induce normal immune responses that involve certain T and B cells that
recognize self-antigens; such cells are not appropriately regulated, and autoantibody
production occurs. (Epstein Barr virus)
Current tobacco smoking increases risk for SLE.
Prolonged occupational exposure to silica.
Certain chemicals(hydrazine) or drugs.
6. Hormonal
Estrogen-containing oral contraceptives or hormone replacement have an
increased risk of developing SLE.
Estradiol binds to receptors on T and B lymphocytes, increasing activation
and survival of those cells, thus favoring prolonged immune response.
Genes on the X chromosome that influence SLE, such as TREX-l, may play
a role in gender predisposition.
7. Genetic
Approximately 45 predisposing genes have been identified to predispose the occurrence
of SLE.
HLA molecules are most commonly found, in multiple ethnic groups as well as multiple
genes across the MHC.
Other genetic factors in whites include;
1. innate immunity pathway gene polymorphisms (associated with IFN α)
2. genes in lymphocyte signaling pathways
3. genes that affect clearance of apoptotic cells or immune complexes
4. genes that influence neutrophil adherence
5. and genes that influence DNA repair
8. Pathophysiology
Interactions between susceptibility genes and environmental factors result in
abnormal immune responses. These responses are:
1. Activation of innate immunity(dendritic cells, monocyte/macrophages)
2. Lowered activation thresholds and abnormal activation pathways in adaptive immunity
cells (mature T and B lymphocytes)
3. Ineffective regulatory CD4+ and CD8+ T cells, B cells, and myeloid-derived suppressor
cells.
4. Reduced clearance of immune complexes and apoptotic cells
10. Clinical Manifestations
Achy joints / arthralgia
Fever of more than 38 ° C
Arthritis / swollen joints
Prolonged or extreme fatigue
Skin Rashes
Anaemia
Kidney Involvement
Pain in the chest on deep breathing / pleurisy
Butterfly-shaped rash across the cheeks and nose
Sun or light sensitivity / photosensitivity
Hair loss / Alopecia
Abnormal blood clotting problems
Fingers turning white and/or blue in the cold
Mouth or nose ulcers
16. Treatment and Management
Treatment goals
Use of drugs with:
least side effects
lowest dosage to control disease
long term damage control
Mild case : avoid use of steroids
Severe case : aggressive treatment
17. Treatment and Management
Non Steroidal Anti Inflammatory Drugs (NSAIDS) :
NSAIDS have analgesic, antipyretic, and anti-inflammatory properties.
Drugs include:
• Ibuprofen
• naproxen
Antimalarial
Hydroxychloroquine (400mg) used as an adjunct to corticosteroid therapy.
Plaquenil can be used alone or with other drugs.
Corticosteroids suppress the immune system and reduce inflammation caused
by lupus.
• Prednisone (20mg)
18. Treatment and Management
Immunosuppressive drugs for patients whose kidneys and CNS is
affected by lupus.
• Cytoxan (25mg) – restrain the overactive immune system by blocking
the production of immune cells.
Other therapies include:
• Plasma exchange
• Intravenous immunoglobin
• Stem cell transplantation
• Immune therapy