1、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, Dis
2、coid Systemic Drug-induced,二. Characteristic of disease:, Atuoimmune Target tissue damage: connection tissue (shin、Joint、 muscles) Predominantly population：womenduring their reproductive year 90% morbidity：70 per 100，000,三. Pathogenic factor, Susceptibility gene：HLA-DR/DQ Environmental Factors：a. ul
3、traviolet lightb. food high caloris, saturated fat, sprout c. drugs isoniazid, methyldopaD-penicillaminechlorpromazine Sex hormone Infections agents,四. pathogenesis,with a complex set of immunologic abnormalities that appear to involve multiple mechanisms of dysregulation,Susceptibility gene,Environ
4、mental Factors Sex hormoneHelper T cell activityHyperactivated B cellImmune complex Self antibody + self antigensMultiple 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. sc
5、lerosing,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% Ra
6、ynauds 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 effusio
7、ns 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 pe
8、ripheral 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
9、,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
10、arterialvenous thrombosis pregnancy morbidity thrombocytopenia,5. Anti SS-A SS-B RNP(ribonucleoprotein) RF,6. Complement depressed C3C4CH50 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
11、 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)Thromb
12、ocytopenia 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 o
13、f 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,
