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Understanding Systemic Lupus Erythematosus (SLE): Key Aspects and Management

Explore the definition, epidemiology, pathogenesis, genetic basis, environmental factors, clinical manifestations, and diagnosis of Systemic Lupus Erythematosus (SLE). Understand the involvement of various organ systems, autoantibodies, pathology, and possible drug-induced lupus. Learn about treatment approaches and preventive measures for managing SLE effectively.

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Understanding Systemic Lupus Erythematosus (SLE): Key Aspects and Management

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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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