内科学系统性红斑狼疮於强课件.ppt

1、 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 2020/11/32 一一.Types of lupus Discoid Systemic Drug-induced2020/11/33 二二.Charact

2、eristic of disease:Atuoimmune Target tissue damage:connection tissue(shin、Joint、muscles)Predominantly population：women during their reproductive year 90%morbidity：70 per 100，000 2020/11/34三三.Pathogenic factor Susceptibility gene：HLA-DR/DQ Environmental Factors：a.ultraviolet light b.food high caloris

3、,saturated fat,sprout c.drugs isoniazid,methyldopa D-penicillamine chlorpromazine Sex hormone Infections agents2020/11/35 四四.pathogenesis with a complex set of immunologic abnormalities that appear to involve multiple mechanisms of dysregulation 2020/11/36Susceptibility gene Environmental Factors Se

4、x hormone Helper T cell activity Hyperactivated B cell Immune complex Self antibody +self antigensMultiple system involvement vasculitis2020/11/37五五.Pathology 1.vasculitis 2.lupus nephritis a.minimal change b.mesangial proliferative c.focal segmental lesions d.diffuse e.membranous f.sclerosing 2020/

5、11/38 六六.Clinical menifestations of SLE -Multiple system involvement 2020/11/39 1.Nonspecific symptom fever loss of weight debilitity2020/11/310 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

6、 ulcer 60%Raynauds phenomenon2020/11/3112020/11/3122020/11/3132020/11/314 3.Joint and muscle 80%arthritis-non erosion large and small joints myosalgia 40%myositis 5%2020/11/315 4.Kidney renal biopsy can be found renal involvement is all present in about 70%of patients 25%patients are dead in cause o

7、f renal involvement 2020/11/316 5.Heart pericarditis pericardial effusions myocarditis endocarditis cardiac valves vegetation2020/11/317 6.Lungs acute lupus penumonitis 3%interstitial pneumonia pulmonary hypertension pleuritis pleural friction sound hydrothorax2020/11/318interstitial pneumonia2020/1

8、1/3197.Neuropsychiatric involvement CNS unfavourable prognosis activity cranial nerves spinal cord peripheral nervous system mental disorder 2020/11/320 8.Digestive system appetite loss，vomiting abdominal pain diarrhea ascites ALTAST pancreatitis intestinal perforation obstruction mesentery vasculit

9、is2020/11/321 9.Hematologic system anemia 60%hemolytic anemia 10%leukopenia 40%thrombocytopenia 20%ITP lymphadenectasis 2020/11/322 10.eye2020/11/323 11.Overlap CTD RA SS 2020/11/324 七七.Laboratory findings ESR CRP2020/11/325 Antibody 1.ANA(antinuclear antibody)screening test2020/11/326 2.Anti double

10、-stranded DNA antibody (ds-DNA)specific activity strong association of lupus nephritis 2020/11/327 3.Anti Smith antibody (Sm)marker antibody non-activity2020/11/328 4.Antiphospholipid antibody arterialvenous thrombosis pregnancy morbidity thrombocytopenia 2020/11/329 5.Anti SS-A SS-B RNP(ribonucleop

11、rotein)RF2020/11/330 6.Complement depressed C3C4CH50 activity2020/11/331 7.CT X-ray ultrasound2020/11/332 八八.Diagnosis The 1982 Revised Criteria for Classification of SLE2020/11/333 1.Malar rash 2.Discoid rash 3.Photosensitivity 4.Oral ulcers 5.Arthritis 6.Serositis a)Pleuritis b)Pericarditis 2020/1

12、1/334 7.Renal disorder a)Persistent proteinuria 0.5 g/d or 3+OR b)may be red cell,hemoglobin,granular,tubular,or mixed 2020/11/335 8.Neurologic disorder2020/11/336 9.Hematologic disorder a)Hemolytic anemia OR b)Leukopenia 4,000 OR c)Lyphopenia 1,500 OR d)Thrombocytopenia 100,000 2020/11/337 10.Immun

13、ologic disorder a)Positive LE cell OR b)Anti-DNA OR c)Anti-Sm OR d)False positive serologic test for syphilis 2020/11/338 11.Antinuclear antibody 2020/11/339 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 observati

14、on2020/11/340 九九.Management of SLE 1.remove the cause drug food uv light infection have rest2020/11/341 2.Discoid nonsteroidal anti-inflammatory drugs （NSAIDs）+antimalarials(chloroquine）OR local steroids or low dose GC2020/11/342 3.SLE glucocorticoid(GC)+cytotoxic drugs (cyclophosphamide)moderate dose GC 1mg/kg/d 2020/11/343 4.lymphocyte-specific cytotoxic drug intravenous gamma globulin immunoablation with autologous stem cell transplantation2020/11/344 十十.Prognosis has improved death is caused most frequently by infection or severe nephritis or diffuse CNS lupus 2020/11/345谢谢！