1 / 16

Rheumatology: SLE

Rheumatology: SLE. Dr. Meg- angela Christi Amores. Systemic Lupus Erythematosus (SLE). autoimmune disease in which organs and cells undergo damage mediated by tissue-binding autoantibodies and immune complexes 90% are women in childbearing years all ethnic groups are susceptible.

essien
Télécharger la présentation

Rheumatology: SLE

An Image/Link below is provided (as is) to download presentation Download Policy: Content on the Website is provided to you AS IS for your information and personal use and may not be sold / licensed / shared on other websites without getting consent from its author. Content is provided to you AS IS for your information and personal use only. Download presentation by click this link. While downloading, if for some reason you are not able to download a presentation, the publisher may have deleted the file from their server. During download, if you can't get a presentation, the file might be deleted by the publisher.

E N D

Presentation Transcript

  1. Rheumatology: SLE Dr. Meg-angela Christi Amores

  2. Systemic Lupus Erythematosus (SLE) • autoimmune disease in which organs and cells undergo damage mediated by tissue-binding autoantibodies and immune complexes • 90% are women in childbearing years • all ethnic groups are susceptible

  3. Pathogenesis • Interactions between susceptibility genes and environmental factors result in abnormal immune responses • (1) activation of innate immunity • (2) lowered activation thresholds of adaptive immunity cells • (3) ineffective regulatory and inhibitory CD4+ and CD8+ T cells • (4) reduced clearance of apoptotic cells and of immune complexes

  4. result in abnormal immune responses • generate pathogenic autoantibodies and immune complexes that deposit in tissue, activate complement • cause inflammation • lead to irreversible organ damage

  5. Pathogenesis • The result of these abnormalities is sustained production of pathogenic autoantibodies : • ANA (antinuclear antibodies) • Anti-dsDNA • Anti-Sm • Anti-RNP • Anti-Ro • Anti-La • Antihistone

  6. Pathogenesis • SLE is a multigenic disease • In most genetically susceptible individuals, normal alleles of multiple normal genes each contribute a small amount to abnormal immune responses • if enough variations accumulate, disease results

  7. Pathogenesis • Female sex is permissive for SLE • make higher antibody responses than males • Women exposed to estrogen-containing oral contraceptives or hormone replacement have an increased risk of developing SLE • environmental stimuli may influence SLE : • Exposure to ultraviolet light • some infections

  8. Diagnosis • based on characteristic clinical features and autoantibodies • Most patients experience exacerbations interspersed with periods of relative quiescence • Antinuclear antibodies (ANA) are positive in >98% of patients during the course of disease; repeated negative tests suggest that the diagnosis is not SLE

  9. diagnosis • Mnemonics: SOAP BRAIN MD • Serositis • Oral ulcers • Arthritis • Photosensitivity • Blood (all are low, anemia, etc) • Renal (protein, nephritis) • ANA • Immunologic (ds DNA) • Neurologic (seizures) • Malar rash • Discoid rash • > 4 out of 11 suggests SLE is highly likely

  10. Manifestations • Arthritis – polyarthritis • soft tissue swelling and tenderness in joints, most commonly in hands, wrists, and knees • Myositis with clinical muscle weakness

  11. Manifestations • Dermatitis • Discoid rash - roughly circular with slightly raised, scaly hyperpigmentederythematous rims and depigmented, atrophic centers • Malar rash - photosensitive, slightly raised erythema, occasionally scaly, on the face (particularly the cheeks and nose—the "butterfly" rash), ears, chin, V region of the neck, upper back, and extensor surfaces of the arms

  12. Manifestations • Neprhritis • most serious manifestation of SLE • leading causes of mortality in the first decade of disease • Nervous system manifestations • cognitive dysfunction, including difficulties with memory and reasoning • seizures

  13. Manifestations • Vascular occlusions • Pleuritis with or without pleural effusion • Pericarditis • Anemia, Leukopenia, Lymphopenia, Thrombocytopenia

  14. Treatment • There is no cure • physician should plan to control acute, severe flares and then develop maintenance strategies

  15. Treatment • Conservative strategies: (for non-life threatening cases) • suppression of symptoms • Analgesics and antimalarials • NSAIDs are useful analgesics/anti-inflammatories, particularly for arthritis/arthralgias • Antimalarials (hydroxychloroquine, chloroquine, and quinacrine) often reduce dermatitis, arthritis, and fatigue

  16. Treatment • Life-threatening: • systemic glucocorticoids • methylprednisolone sodium succinate • Prednisone • doses are tapered as rapidly as the clinical situation permits • Cytotoxic/immunosuppressive agents added to glucocorticoids

More Related