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Autoimmune Diseases Dr. Raid Jastania
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Autoimmune Diseases Group of diseases with common pathological process Presence of auto-antibody ?defect in B-cells or T-cells Genetic and Environmental etiological factors Result in failure of self-tolerance The process involve Ag-Ab binding and Ag-Ab complex formation Process may lead in complement activation and inflammation with tissue injury
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Autoantibody Failure to maintain self-tolerance Autoantibody can be formed to: –Nuclear antigen –Cytoplasmic antigen –Cell surface antigen –Proteins and phospholipids
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Anti Nuclear Antibody (ANA) ANA represent diversity of antibodies that bind to several nuclear antigen –Anti-DNA –Anti-Histone –Antibody to Non-Histone –Antibody to nucleolar antigen ANA is usually detected by indirect immunofluorescence
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Anti Nuclear Antibody (ANA) Patterns of ANA staining: –Homogenous: antibody to chromatin, histone or DNA –Rim/Peripheral: antibody to DNA –Speckled: antibody to histone (Sm, ribonucleoprotein RNP, SS-A (Ro), SS-B (La)) –Nucleolar pattern
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Systemic Lupus Erythematosus Autoimmune Disease Multi-system disease Variable behaviour, unpredictable, remitting relapsing, acute and gradual. May involve any organ Common: Skin, kidney, serosal membranes, joints, heart.
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Systemic Lupus Erythematosus ANA (anti-DNA, anti-Sm, anti- phospholipid) Prevalence: 1/2500 person Female: male 9:1 1/700 women More common and severe in blacks (1/245) Onset: 2 nd or 3 rd decade
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SLE: Criteria for Diagnosis Malar Rash Discoid Rash Photosensitivity Oral ulcers Arthritis Serositis Renal disorder Neurologic disorder Hematologic disorder Immunologic disorder ANA
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SLE ANA is sensitive to SLE but not specific ANA is present in 5-15% of normal people More specific to SLE are” –Anti-DNA –Anti-Sm LE bodies (hematoxylin bodies) Antiphospholipid 40-50% of cases Antiphospholipid syndrome (lupus anticoagulants)
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SLE Atiology Genetic factors –25% concordance in monozygotic twins –Increase risk of disease in family members –SLE and HLA-DQ association –Some SLE patients have deficiency in complement components
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SLE Atiology Non-Genetic factors: –Lupus-like syndrome with drug admenistration, procainamide, hydralazine –Association with sex hormone (more in female) –Trigger by exposure to sun light
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SLE Atiology Immunologic Factors: –Polyclonal B-cell activation? –Oligoclonal B-cell activation –CD4+ T helper cell activation
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SLE: mechanism of tissue injury Immune complex disease –Example: deposition of Ag-Ab complex in glomeruli results in kidney disease Type II hypersensitivity: –Hemolysis –Thrombocytopenia
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SLE: common clinical manifestations Hematologic: anemia, leukopenia, thrombocytopenia Arthritis Skin rash Fever Fatigue Weight loss Renal disease CNS abnormality Pleuritis Myalgia Pericarditis GI inflammation Raynaud phenomenon Peripheral neuropathy
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Pathological Findings Ag-Ab complex deposition Common: kidney, hear, vessels, serosal membranes, and skin With the consequences of inflammation and tissue injury
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Pathological Findings Vessels: –Acute necrotizing vasculitis Skin: –Rash, erythema –Cell injury/necrosis of the basal layer of epidermis, edema, inflammation –Deposition of Ig, complement components
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Pathological Findings Joints –Inflammation of synuvium, edema, mononuclear cell infiltrate Spleen: –Enlargement with follicular hyperplasia Serosa: pleura, peritoneum, pericardium –Serous effusion –Fibrinous inflammation –fibrosis
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Pathological Findings Heart: –Pericarditis, myocarditis –Valvular lesion: Libman-Sacks endocarditis –Coronary artery disease Kidney –Deposition of complexes in glomeruli –Lupus nephritis –Cell injury/necrosis, proliferation of mesangium, endothelium, and epithelium
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