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于强-SLE.ppt
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医学类型:国产软件 - 医药 - 医学ppt
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医学大小:1.66 MB
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于强-SLE.ppt介绍

  Systemic Lupus  Erythematosus  Zhongshan Hospital 於强一. Types of lupus ▲Discoid  ▲Systemic  ▲Drug-induced  二. Characteristic of disease: ▲Atuoimmune ▲Target tissue damage: connection  tissue (shin 、Joint 、muscles) ▲Predominantly population :women  during their reproductive year  90% ▲morbidity :70 per 100 ,000  三. Pathogenic factor ▲Susceptibility gene :HLA-DR3 ▲Environmental Factors :a. ultraviolet light  b. food  c. drugs hydralazine  procainamide  D-penicillamine ▲Sex hormone ▲Infections agents 四. pathogenesis  unclear Susceptibility gene 五. Pathology 1. vasculitis  2. lupus nephritis a. minimal change b. mesangial proliferative c. focal segmental lesions d. diffuse e. membranous f. sclerosing 六.Clinical menifestations  of SLE  -----Multiple  system  involvement  1. Nonspecific symptom ●fever ●loss of weight ●inertia    2.Skin and mucosa  ●skin symptoms in 80% of patients   ●specific features: 40%  malar rash discoid rash ●nonspecific features 60%  ●photosensitivity 40%  ●baldness 40%  ●oral ulcer 60% ●Raynaud’s phenomenon   3. Joint and muscle 80% ●arthritis---non erosion ●large and small joints ●myosalgia 40% ●myositis  5%  4. Kidney  ▲renal biopsy can be found   renal involvement is all ▲present in about 70% of   patients ▲25% patients are dead in    cause of renal   involvement  5. Heart ●pericarditis  pericardial effusions ●myocarditis ●endocarditis    cardiac valves  vegetation  6. Lungs ●acute lupus penumonitis 3%  ●interstitial pneumonia  pulmonary hypertension  ●pleuritis  pleural  friction sound hydrothorax interstitial pneumonia  7.Neuropsychiatric  involvement ●CNS    unfavourable prognosis  activity cranial nerves  spinal cord ●peripheral nervous system  ●mental disorder  8.Digestive system ●appetite loss  vomiting ●abdominal pain  diarrhea  ascites ●ALT\AST ●pancreatitis ●intestinal perforation  obstruction ●mesentery vasculitis 9.Hematologic system ●anemia 60% hemolytic anemia 10%  ●leukopenia 40%  ●thrombocytopenia 20%   ITP  ●lymphadenectasis 10.eye 11.Overlap CTD RA SS  七. Laboratory  findings  ●ESR    ●CRP   Antibody 1. ANA (antinuclear antibody) screening test        2. Anti double-stranded  DNA  antibody (ds-DNA) ●specific  ●activity ●strong association of  lupus nephritis   3.Anti Smith antibody (Sm) ●marker antibody ●non-activity    4.Antiphospholipid antibody ●arterial\venous thrombosis ●pregnancy morbidity ●thrombocytopenia   5. Anti SS-A SS-B RNP(ribonucleoprotein) RF  6. Complement  ●depressed  C3\C4\CH50 ●activity  7. CT X-ray ultrasound    八.Diagnosis The 1982 Revised Criteria for   Classification of SLE   1. Malar rash  2. Discoid rash 3. Photosensitivity  4. Oral ulcers  5. Arthritis  6. Serositis  a) Pleuritis b) Pericarditis  7. Renal disorder  a) Persistent  proteinuria > 0.5 g/d or > 3+   OR  b)may be red cell, hemoglobin,   granular, tubular, or mixed  8. Neurologic disorder   9. Hematologic disorder  a) Hemolytic anemia  OR   b) Leukopenia < 4,000  OR   c) Lyphopenia < 1,500  OR   d)Thrombocytopenia <100,000  10. Immunologic disorder a) Positive LE cell   OR   b) Anti-DNA  OR   c) Anti-Sm  OR   d) False positive serologic  test for syphilis   11. Antinuclear antibody  a person shall be said to have SLE  if any 4 or more of the 11 criteria are present, serially or simultaneously, during any interval of observation    九.Management of SLE 1. remove the cause   drug   food   uv light infection  have rest      2. Discoid  nonsteroidal anti-  inflammatory drugs  (NSAIDs )+ antimalarials (chloroquine ) OR  local steroids or low dose GC   3. SLE  glucocorticoid (GC)  +   cytotoxic drugs  (cyclophosphamide) ●moderate dose GC  1mg/kg/d   4. ●lymphocyte-specific   cytotoxic drug ●intravenous gamma  globulin  ●immunoablation with  autologous stem cell   transplantation     十.Prognosis ●has improved ●death is caused most  frequently by infection  or severe nephritis or  diffuse CNS lupus  *  *  Environmental Factors Sex hormone  Helper T cell activity Hyperactivated  B cell  Immune complex  Self antibody  + self  antigens Multiple  system involvement vasculitis

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