内科学系统性红斑狼疮-於强.ppt

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(ribonucleoprotei n) 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 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 cy