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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