於强-SLE

null Systemic Lupus Erythematosus Systemic Lupus ErythematosusZhongshan Hospital 於强 一. Types of lupus 一. Types of lupus ▲ Discoid ▲ Systemic ▲ Drug-induced 二. Characteristic of disease: 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三. Pathogenic factor ▲ Susceptibility gene：HLA-DR3 ▲ Environmental Factors： a. ultraviolet light b. food c. drugs hydralazine procainamide D-penicillamine ▲ Sex hormone ▲ Infections agents 四. pathogenesis 四. pathogenesis unclearSusceptibility geneSusceptibility 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 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 六.Clinical menifestations of SLE -----Multiple system involvement 1. Nonspecific symptom ● fever ● loss of weight ● inertia 1. Nonspecific symptom ● fever ● loss of weight ● inertia 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% ● Raynaud’s phenomenon 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% ● Raynaud’s phenomenon nullnullnull 3. Joint and muscle 80% ● arthritis---non erosion ● large and small joints ● myosalgia 40% ● myositis 5% 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 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 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 6. Lungs ● acute lupus penumonitis 3% ● interstitial pneumonia pulmonary hypertension ● pleuritis pleural friction sound hydrothoraxinterstitial pneumoniainterstitial pneumonia 7.Neuropsychiatric involvement ● CNS unfavourable prognosis activity cranial nerves spinal cord ● peripheral nervous system ● mental disorder 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 ● ALT\AST ● pancreatitis ● intestinal perforation obstruction ● mesentery vasculitis 8.Digestive system ● appetite loss vomiting ● abdominal pain diarrhea ascites ● ALT\AST ● pancreatitis ● intestinal perforation obstruction ● mesentery vasculitis 9.Hematologic system ● anemia 60% hemolytic anemia 10% ● leukopenia 40% ● thrombocytopenia 20% ITP ● lymphadenectasis 9.Hematologic system ● anemia 60% hemolytic anemia 10% ● leukopenia 40% ● thrombocytopenia 20% ITP ● lymphadenectasis 10.eye 10.eye 11.Overlap CTD RA SS 11.Overlap CTD RA SS 七. Laboratory findings ● ESR ● CRP 七. Laboratory findings ● ESR ● CRP Antibody 1. ANA (antinuclear antibody) screening test Antibody 1. ANA (antinuclear antibody) screening test 2. Anti double-stranded DNA antibody (ds-DNA) ● specific ● activity ● strong association of lupus nephritis 2. Anti double-stranded DNA antibody (ds-DNA) ● specific ● activity ● strong association of lupus nephritis 3.Anti Smith antibody (Sm) ● marker antibody ● non-activity 3.Anti Smith antibody (Sm) ● marker antibody ● non-activity 4.Antiphospholipid antibody ● arterial\venous thrombosis ● pregnancy morbidity ● thrombocytopenia 4.Antiphospholipid antibody ● arterial\venous thrombosis ● pregnancy morbidity ● thrombocytopenia 5. Anti SS-A SS-B RNP(ribonucleoprotein) RF 5. Anti SS-A SS-B RNP(ribonucleoprotein) RF 6. Complement ● depressed C3\C4\CH50 ● activity 6. Complement ● depressed C3\C4\CH50 ● activity 7. CT X-ray ultrasound 7. CT X-ray ultrasound 八.Diagnosis The 1982 Revised Criteria for Classification of SLE 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 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 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 8. Neurologic disorder 9. Hematologic disorder a) Hemolytic anemia OR b) Leukopenia < 4,000 OR c) Lyphopenia < 1,500 OR d)Thrombocytopenia <100,000 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 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 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 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 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 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 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 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 十.Prognosis ● has improved ● death is caused most frequently by infection or severe nephritis or diffuse CNS lupus