内科学系统性红斑狼疮於强

单击此处编辑母版标题样式,单击此处编辑母版文本样式,第二级,第三级,第四级,第五级,*,单击此处编辑母版标题样式,单击此处编辑母版文本样式,第二级,第三级,第四级,第五级,*,*,单击此处编辑母版标题样式,单击此处编辑母版文本样式,第二级,第三级,第四级,第五级,*,*,内科学系统性红斑狼疮於强,What is Lupus,a chronic, relapsing, inflammatory, and often febrile multisystemic disease of connective tissue, characterized principally by involvement of the skin. Joints, kidneys and serosal membranes. an autoimmune disease,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-DR/DQ,Environmental Factors,：,a. ultraviolet light,b. food,high caloris,saturated fat,sprout,c. drugs,isoniazid,methyldopa,D-penicillamine,chlorpromazine,Sex hormone,Infections agents,四,. pathogenesis,with a complex set of,immunologic abnormalities that,appear to involve multiple,mechanisms of dysregulation,Susceptibility gene,Environmental Factors Sex hormone,Helper T cell activity,Hyperactivated B cell,Immune complex,Self antibody,+ self antigens,Multiple system involvement,vasculitis,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,debilitity,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%,Raynauds 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,ALTAST,pancreatitis,intestinal perforation obstruction,mesentery,vasculitis,9.Hematologic system,anemia 60% hemolytic anemia 10%,leukopenia 40%,thrombocytopenia 20% ITP,lymphadenectasis,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,arterialvenous thrombosis,pregnancy morbidity,thrombocytopenia,5. Anti SS-A SS-B RNP,(ribonucleoprotein),RF,6. Complement,depressed C,3,C,4,CH,50,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,汇报结束,谢谢大家,!,请各位批评指正,