资源描述
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,*,Systemic Lupus Erythematosus,Zhongshan Hospital,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,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,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,
展开阅读全文