Systemic Lupus Erythematosus BY DR SHARMEEN TALPUR Definition
Systemic Lupus Erythematosus BY DR SHARMEEN TALPUR
Definition • is an autoimmune disease of the body's connective tissues. • Autoimmune means that the immune system attacks the tissues of the body. • attacks parts of the cell nucleus. • between the ages of 20 and 30, although it can show up at any age.
Pathogenesis • A healthy immune system produces proteins called antibodies and specific cells called lymphocytes. • that help fight and destroy viruses, bacteria, and other foreign substances that invade the body.
• In lupus, the immune system produces antibodies against the body's healthy cells and tissues. • These antibodies, contribute to the inflammation of various parts of the body and can cause damage to organs and tissues. • Lupus can affect -joints, skin, kidneys, heart, lungs, blood vessels, and brain.
Causes The exact causes is not known. • Inherited genes, viruses, ultraviolet light, and drugs may all play some role. • Genetic factors increase the tendency of developing autoimmune diseases
• • female hormones play an important role in the expression of SLE. a key enzyme's failure to dispose of dying cells may contribute the development of SLE. • a genetic mutation • Drug-induced lupus. ( hydralazine, betablokers, isoniazid).
There are several kinds of lupus: Discoid lupus erythematosus is a chronic skin disorder red, raised rash appears on the face, scalp, or elsewhere The rash may last for days or years.
Sub acute cutaneous lupus erythematosus refers to skin lesions that appear on parts of the body exposed to sun. The lesions do not cause scarring. Drug-induced lupus is a form of lupus caused by medications. Many different drugs can cause druginduced lupus Neonatal lupus is a rare disease that can occur in newborn babies of women with SLE
• Sjögren's syndrome is an autoimmune disorder in which immune cells attack and destroy the excroin glands that produce tears and saliva. • Sjögren's syndrome is also associated with rheumatic disorders such as rheumatoid arthritis
Secondary sjogren’s syndrome • Dry eyes • Dry mouth
Clinical manifestations • The symptoms of SLE come on in waves, called flares or flare-ups. • general discomfort, extreme fatigue, mild lymphadenopathy, fever, and weight loss at some point.
“Common Symptoms of Lupus • • • Painful or swollen joints and muscle pain Unexplained fever Red rashes, most commonly on the face Chest pain upon deep breathing Unusual loss of hair Pale or purple fingers or toes from cold or stress.
• • Sensitivity to the sun Swelling (edema) in legs or around eyes Swollen glands Extreme fatigue
• Malar rash: • This is a "butterfly-shaped" red rash over the cheeks below the eyes and across the bridge of the nose. • It may be a flat or a raised rash. • The rashes are made worse by sun exposure.
• Raynaud’s phenomenon • is commonly found in lupus. • reaction of distal digits to cold or emotion, in which the skin colour changes from white to blue to red)
• Maculopapular eruption
• Vasculitic skin lesion
• Alopecia
• Oral ulcer: • Painless sores in the nose or mouth need to be observed and documented by a doctor.
Different symptoms in different body systems : • Kidneys Inflammation of the kidneys (nephritis) The typical renal lesion is a proliferative glomerulonephritis characterised by heavy hematuria, proteinurea and casts on microscopy.
• lungs: • Most common menifast as pleurisy or pleural effusion. other features include pneumonitis, atelactasis, reduced lung vol and pulmonary fibrosis leading to breathlessness.
• Blood vessels: • Blood vessels may become inflamed (vasculitis) • Muscles and Bones: • joint pain or inflammation. • The pain can come and go, or it can be long lasting.
• Blood: • Hemolytic anemia, leucopenia (a decreased number of white blood cells), or thrombocytopenia (a decrease in the number of platelets in the blood, which assist in clotting). • Heart: • T he most common manifestation is pericarditis. myocarditis and libman-sacks endocarditis can also occur.
• Digestive System: • Mouth ulcers • , Mesenteric vasculitis is a serious complication which can be present as abdominal pain, bowel infarction or perforation. • Neurological effects • fatigue, headache and poor concentration are common. • Pregnancy. • However, the chances of miscarriage,
Diagnostic studies – Complete blood count (CBC) – Erythrocyte sedimentation rate (ESR) – Urinalysis – Blood chemistries – Antinuclear antibody test (ANA) – Other autoantibody tests (anti-DNA) • Skin biopsy • Kidney biopsy
Diagnostic studies – The diagnosis is based on a combination of clinical features and laboratory abnormalities. – Patients with active SLE almost always test positive for ANA, but ANA –negative SLE can very rarely occur in the presence of antibodies to the Ro antigen, – Anti –ds. DNA antibodies are charecteristic of severs active SLE but occur in only around 30% of cases. – A raised ESR , LEUCOPENIA, AND LYMPHOPENIA are typical of active SLE, along with hemolytic anemia and
Treatment • A conservative regimen of physical and emotional rest is required • protection from direct sunlight • a healthful diet • prompt treatment of infections • pregnancy must be planned for times when the disease is under control, and the patient is on allowable medications.
Medications 1 -NSAIDs: • Nonsteroidal anti-inflammatory drugs (NSAIDs) such as ibuprofen and naproxen. 2 - Antimalarials: • (Plaquenil). It may be used alone or in combination with other drugs 3 - Corticosteroids: short courses of oral steroids may require for rash, synovoitis, pleurisy and pericarditis.
Life threatning diseases affecting the kidneys , CNS, CVS require high doses steroides and Immunosuppressives Commonly used regimen is methylpridnisolone coupled with cyclophosphamide (Cytoxan)
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