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IN THE NAME OF GOD

IN THE NAME OF GOD. SYSTEMIC LUPUS ERYTHEMATOSIS (SLE). DEFINITION. Autoimmune Multisystem disease Autoantibodies and immune complexes. EPIDEMIOLOGY. Women of child-bearing years (90%) Most common age at onset: second and third decade All ages and ethnic groups Both sexes

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IN THE NAME OF GOD

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  1. IN THE NAME OF GOD

  2. SYSTEMIC LUPUS ERYTHEMATOSIS(SLE)

  3. DEFINITION • Autoimmune • Multisystem disease • Autoantibodies and immune complexes

  4. EPIDEMIOLOGY • Women of child-bearing years (90%) • Most common age at onset: second and third decade • All ages and ethnic groups • Both sexes • Prevalence in US 10-400/100,000 • Prevalence in Iran 30/100,000

  5. PATHOGENESIS

  6. PATHOGENESIS Predisposition Susceptibility Genes Induction Autoimmunity Expansion Injury Clinical Disease

  7. GENETIC BASIS • Twins: • Monozygotic 57% • Dizygotic 5% • Familial aggregation: • First degree relative 12% • HLA: DR2, DR3 • C1q, C2, C4

  8. ENVIRONMENTAL • Ultraviolet B light • Sex hormones • Estrogen • Androgen • Infectious agent • Drug

  9. Apoptosis T-cell Macrophages

  10. Apoptosis SM Ro/ss-a DNA

  11. Apoptosis T-cell Macrophages

  12. Apoptosis B cell T-cell Macrophages

  13. PATHOGENESIS • UV Flare of SLE in 70% of patients • Infections: • Induce B and T cells Recognize self Ag Auto Ab • EBV: - More common in SLE patients - Activate B cell - Amino acid sequences Mimic some on DNA

  14. PATHOGENESIS • Female: • Ab responses than male • OCP & HRT: Risk of SLE (1.2-2 fold) • Estradiol T & B cell Activation & Survival Prolonged immune response Bind to

  15. Genetic Complement activity Environmental Factors Immune comlexes Apoptosis Phagocytosis Auto antigen Apoptotic Material Auto antibody Immunogenic Ag DC T cell CD4 B cell

  16. CLINICAL MANIFESTATION

  17. CLINICAL MANIFESTATION • ANY ORGAN CAN BE AFFECTED

  18. SYSTEMIC MANIFESTATION • Fatigue, Malaise, Fever, Anorexia, Weight loss 95%

  19. MUSCULOSKELETAL • Polyarthritis (95%) • Most patients • Hands, Wrists, Knees • Deformity 10% • Erosion Rare • Weakness (25%) • Myositis • Glucocorticoid • Antimalaria

  20. SYSTEMIC MANIFESTATION Pain persist in a single joint Ischemic necrosis of bone

  21. CUTANEOUS(80%)

  22. CUTANEOUS • Butterfly rash (50%): - Most common - Flare

  23. CUTANEOUS • Discoid rash (DLE) (20%)

  24. RENAL • Nephritis (50%): • Most serious manifestation • U/A: any person with suspected SLE • Class III or IV: - Microscopic hematuria - Proteinuria (> 500 mg/24h) - HTN

  25. HEMATOLOGIC • Anemia (70%) • Chronic disease • Hemolytic • Leukopenia (65%) • Lymphopenia • Infection: rare • Not require therapy • Thrombocytopenia (15%)

  26. PULMONARY • Pluritis (30%) - Most common • Interstitial inflammation • Pulmonary hemorrhage

  27. CARDIAC • Pericarditis (30%) • Myocarditis (10%) • Endocarditis (10%) • Valvular insufficiencies • Libman-Sacks • Ischemia

  28. VASCULAR • Risk of vascular events 7-10 fold • TIA, Strok, MI • Causes: • APS • Embolization - Carotid plaque - Libman-Sacks • Vasculitis • Atherosclerosis

  29. GASTROINTESTINAL • Peritonitis • Vasculitis

  30. OCULAR • Sicca • Conjunctivitis • Retinal vasculitis • Optic neuritis

  31. NERVOUS SYSTEM • Central • Peripheral • Other causes

  32. ANTIPHOSPHOLIPID SYNDROM • Risk of - Clotting (arterial or venous) - Fetal loss • Tests: - Anticardiolipin - Lupus anticoagulant

  33. ANTIPHOSPHOLIPID SYNDROM • High titer of IgG ACL - Risk of clotting • Diagnosis: - One clinical - One test (repeated 12w apart)

  34. AUTOANTIBODIES • Most patients 3 y or more before symptom

  35. AUTOANTIBODIES • FANA: • Prevalence: 98% • Best screaming test • Anti-dsDNA: • Prevalence: 70% • Specific (high titer) • Correlate with disease activity

  36. AUTOANTIBODIES • Anti-Sm: - Prevalence: 25% - Specific - No clinical correlation • Anti-Ro (SS-A): - Sicca, Neonatal lupus, Nephritis • Antiphospholipid: - 50% - Criteria and APS syndrome

  37. PATHOLOGY • Class I: Mesangial lupus nephritis - LM: NL - IF: Mesangial deposit • Class II: Mesangial prolipherative

  38. PATHOLOGY • Class III: Focal proliferative • Class IV: Diffuse proliferative • Class: V: Membranous • Class: VI: Sclerotic

  39. DIAGNOSIS

  40. DIAGNOSIS • Malar rash • Discoid rash • Oral ulcer • Photosensitivity

  41. DIAGNOSIS • Arthritis: • Nonerosive • ≥ 2 or more peripheral joints • Serositis: • Pleuritis or pericarditis • Renal: • Proteinuria > 500 mg or ≥ 3+, or cellular casts

  42. DIAGNOSIS • Neurologic: • Seizures or psychosis without other causes • Hematologic: • Hemolytic anemia or • Leukopenia (< 4000) or • Lymphopenia (< 1500) or • Thrombocytopenia (< 100,000)

  43. DIAGNOSIS • Immunologic disorder: • Anti-dsDNA, anti-Sm, antiphospholipid • Antinuclear antibodies: • By immunofluorescence

  44. DIAGNOSIS • Criteria for classification • ≥ 4 criteria • Specificity: 95% • Sensitivity: 75%

  45. DRUG-INDUCED LUPUS • Milder • Rarely renal or CNS involvement • Drugs: hydralazine, procainamid… • Positive ANA and Anti histone but rarely Anti-dsDNA • Reversible

  46. TREATMENT

  47. TREATMENT • No cure • Patients education • Prophylactic measures: • Sunscreen • Low dose aspirin for antiphospholipid Ab positive • Routine immunization

  48. TREATMENT • Glucocorticoids: - For almost any manifestation • Immunomodulating agents: - Antimalaria Fever, Arthritis, Cutaneous Prevents flare - Azathioprine - Mycophenolate mofetile - Cyclophosphamide

  49. CORSE • Range from mild to sever diseases • Survival: - 95% at 5y and 78% at 20y • Causes of death: - First decade: disease activity, Renal, Infection - After: Thromboembolic • Critical: - Nephritis, Cerebritis, Pulmonary hemorrhage, Hematologic, Carditis

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