Systemic lupus erythematous
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Transcript of Systemic lupus erythematous
SYSTEMIC LUPUS ERYTHEMATOUSBy Rodnishwar PrasadGroup 23
WHAT IS LUPUS? Systemic Lupus Erythematous (SLE) is a complex autoimmune disease
The immune system attacks the body’s cells and tissue, resulting in inflammation and tissue damage.
SLE can affect any part of the body, but most often harms the heart, joints, skin , lungs , blood vessels , liver, kidneys, and nervous system.
Over 40 different genes predispose to SLE
Characterized by remissions and exacerbations
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
ETIOLOGYThe cause of SLE is currently unknown but there may be environmental
and genetic factors to it.
Environmental Factors:UV lights, certain chemicals(hydrazine), drugs, infections and smoking.
B Cell Activation:Results in autoantibody(IgG) production to a variety of antigen.
Impaired T cell regulation of immune system
SIGNS & SYMPTOMSAchy 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
PATHOPHYSIOLOGYThe plasma cells are producing antibodies that are specific for self proteins, ds-DNA
Overactive B-cells which are stimulated by estrogen.
Suppressed regulatory function in T-cells.
IL-10, also a B-cell stimulator is in high concentration in lupus patient serum.
High concentration linked to cell damage caused by inflammation
Increased levels of Ca2+ leads to spontaneous apoptosis.
PATHOPHYSIOLOGYRBCs lack CR1 receptor which decreases the affective removal of
complexes
IgG is the most “pathogenic” because it forms intermediate complexes that can get to the small places and block them.
DNA is the main antigen for which antibodies are formed.
Extracellular DNA has an affinity for basement membrane where it is bound by auto-antibodies.
Classical thickening of the basement membrane
DIAGNOSISComplete blood count(CBC)
Erythrocyte sedimentation rate
Creatine phosphokinase – usually elevated in SLE myositis.
Renal biopsy – indicates of lupus nephritis.
Serology – antinuclear antibody (ANA) test. Positive confirms SLE
TREATMENTTreatment goals
Use of drugs with:-least side effects-lowest dosage to control disease-long term damage control
Mild case : avoid use of steroidsSevere case : aggressive treatment
DRUGS TO USENon Steroidal Anti Inflammatory Drugs (NSAIDS) :
• NSAIDS have analgesic, antipyretic, and anti-inflammatory properties.• Drugs include:
-ibuprofen-naproxen
Antimalarial :• Hydroxychloroquine 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
DRUGS TO USEImmunosuppressive drugs for patients whose kidneys and CNS is affected
by lupus.• Cytoxan – restrain the overactive immune system by blocking the production
of immune cells.
Other therapies include:• Plasma exchange• Intravenous immunoglobin• Stem cell transplantation• Immune therapy
PROGNOSISUsually chronic, relapsing, and unpredictable. Remissions may last for
years.
If initial acute phase is controlled, even if very severe ( with cerebral thrombosis or severe nephritis), the long-term prognosis is usually good.
The 10-yr survival in most developed countries is 95%. Improved prognosis is in part due to earlier diagnosis and more effective therapies.
More severe disease requires more toxic therapies, which increase risk of mortality.
PATIENT EDUCATIONMinimize appearance of lesions. Alleviate discomfort Minimize fatigue. Maintain weight at optimal rangeTeach the patient to recognize
fever and signs and symptoms of infection.
Maintain joint function and increase muscle strength.
Recognize anaemia and develop a plan of care
Minimize episodes of bleeding.Minimize incidence of infection.Educate the patient about
immunizationsEducate patient nutritional status.
REFERENCE1. Kim, JM. (2014). Simultaneous presentation of acute disseminated
encephalomyelitis (ADEM) and systemic lupus erythematosus (SLE) after enteroviral infection: can ADEM present as the first manifestation of SLE. Available: http://www.ncbi.nlm.nih.gov/pubmed/25488421. Last accessed 16th Dec 2014.
2. Klinik für Kinder und Jugendmedizin. (2014). Pathogenesis and new therapeutic approaches for systemic lupus erythematosus. Available: http://www.ncbi.nlm.nih.gov/pubmed/25479933. Last accessed 16th Dec 2014.
3. Leal, GN. (2014). Subclinical right ventricle systolic dysfunction in childhood-onset systemic lupus erythematosus: insights from two-dimensional speckle-tracking echocardiography. Available: http://www.ncbi.nlm.nih.gov/pubmed/25492941. Last accessed 16th Dec 2014.
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