内分泌学-系统性红斑狼疮 SLE课件

,系统性红斑狼疮(SLE) Systemic Lupus Erythematosus,Systemic Lupus Erythematosus,Many Different Auto- antibodies,Multiple System Involvement,SLE onset by sex and age,Etiology and pathogenesis pathology Clinical findings Laboratory findings Diagnosis Treatment prognosis,Etiology,genetic factors environmental factors Sex hormonal factors,Pathogenesis,Excessive, abnormal production of “self” antibodies and formation of IC. Autoantibodies against nuclear, cytoplasmic and membrane components of multiple cell types in multiple organs .,Etiology and pathogenesis pathology Clinical findings Laboratory findings Diagnosis Treatment prognosis,Pathology,光镜:1.结缔组织的纤维蛋白样变性 2.基质黏液性水肿 3.坏死性血管炎 特征性:疣状心内膜炎 苏木紫小体 “洋葱皮样”病变 临床应用：皮肤狼疮带试验 肾活检,Etiology and pathogenesis pathology Clinical findings Laboratory findings Diagnosis Treatment prognosis,Systemic features,Fever Weak Weight loss,skin and mucosa,光敏感 photosensitivity 脱发 alopecia 雷诺现象Raynauds phenomenon 口腔溃疡oral ulcer 皮疹 rash,skin and mucosa,skin and mucosa,skin and mucosa,skin and mucosa,skin and mucosa,skin and mucosa,Muscle and Joint,arthritis -Jaccouds arthropathy Muscle pain、myositis femoral head necrosis,Muscle and Joint,Renal lesion,Proteinuria hematuria cylindruria Nephritic syndrome Renal insufficiency,lupus nephritis,Class I 正常 Class II 系膜增殖性 mesangial Class III 局灶增殖性 focal proliferative Class IV 弥漫增殖性 diffuse proliferative Class V 膜性 membranous Class VI 肾小球硬化性glomerulosclerosis,Nervous system,clinical manifestation: -headache、vomiting -psychogenia -epilepsy -convulsion 、 conscious disturbance -coma pathology-vasculitis cerebrospinal fluid-no special (intracranial pressureproteincell population glucose) Identify with other central nervous system disease,Hematology system,leukopenia thrombocytopenia anemias: hemolytic anemias anemias of chronic disease lymphadenectasis splenectasis,Respiratory system,pleurisy Pleural effusion Pulmonary interstitial fibrosis Lupus pneumonia Pulmonary hypertension Respiratory failure,lung,Circulatory system,heart -pericarditis -myocarditis -endocarditis Libman-Sack endocarditis -cardiac arrhythmias -cardiac failure vessel -vasculitis,Digestive system,Clinical features -anepithymia -nausea and vomiting -abdominal pain、diarrhea -peritoneal effusion -acute abdomen pathology -mesenterium vasculitis,抗磷脂抗体综合征 (antiphospholipid antibody syndrome),clinical manifestation: -arterous and/or venous thrombosis -spontaneous abortion -thrombocytopenia laboratory examination: -positive anti-phospholipid antibody,干 燥 综 合 征,30%的SLE患者可有继发干燥综合征 患者有临床症状 唾液腺ECT可有改变 干燥抗体可为阴性,Etiology and pathogenesis pathology Clinical findings Laboratory findings Diagnosis Treatment prognosis,一般检查,自身抗体,补体等,狼疮带试验,肾活检病理,影像学检查,血常规 尿常规 血沉,抗核抗体谱 抗磷脂抗体 抗组织 细胞抗体,C3 C4 CH50 Ig r-G,诊断 治疗 预后,MRI CT,实验室和其他辅助检查,SLE50% 代表SLE 活动性,抗核抗体谱,抗核抗体ANA,抗dsDNA抗体,抗ENA抗体,Jo-1,Sm,RNP,SSB,SSA,Etiology and pathogenesis pathology Clinical findings Laboratory findings Diagnosis Treatment prognosis,美国风湿病学会(ACR)1997年SLE诊断标准 1.蝶形红斑 2.盘状红斑 3.光过敏 4.口腔溃疡 5.关节炎 6.浆膜炎(胸膜炎/心包炎) 7.肾病变a.蛋白尿 0.5g/day b.细胞管型 8.神经系统病变 a.癫痫发作 b.精神症状 4项阳性可诊断SLE,9. 血液系统异常 a.溶血性贫血 b.白细胞减少 c.淋巴细胞绝对值减少 d.血小板减少 10.免疫学异常 a. anti-dsDNA b. anti-Sm c. anti-CL and/or LA 11. ANA,CRITERIA FOR THE DIAGNOSIS OF SLE ACCORDING TO THE ACR(1997) 1. Butterfly rash 2. Discoid lupus 3. Photosensitivity 4. Oral ulceration 5. Polyarthritis 6. Nephritis a. proteinuria over 0.5g/day b. cellular casts 7. Pleuritis/pericarditis 8. Neuropsychiatric symptoms a. convulsions b. psychosis 4 or more symptoms are required for the diagnosis,9. Haematological alterations a. haemolytic anaemia b. leucopenia (4.0 G/l) c. lymphopenia (1.5G/l) d. thrombocytopenia (100G/l) 10. Immunologic alterations a. anti-dsDNA b. anti-Sm c. anti-CL and/or LA 11. ANA,SLE-DAI,狼疮性头痛 精神症状 器质性脑病 视觉障碍 颅神经病变 脑血管意外 血管炎 癫痫发作,发热1分 血小板减少 白细胞减少,关节炎 肌炎 管型尿 血尿 蛋白尿 脓尿,新出皮疹 黏膜溃疡 高效价 dsDNA 低补体血症 心包炎 胸膜炎 脱发 2分,8分,4分,总积分为105分，10分以上为活动,20以上者提示很明显的活动,提示SLE活动性的指证,症状体征:乏力、体重下降 发热 新出现的皮肤黏膜改变 关节炎 浆膜炎 尿少、浮肿 头痛、癜痫 辅助检查：血细胞减少 蛋白尿、血尿、管型尿、非感染性白细胞尿 补体下降 DNA抗体滴度升高,SLE病情轻重的评估,1.轻型SLE：无系统受累 2.重型SLE：有系统受累 3.狼疮危象(lupus crisis)：危及生命的重型SLE，包括：急进性狼疮肾炎，严重的中枢神经系统损害，严重的溶血性贫血，血小板减少性紫癜，粒细胞缺乏症，严重心脏损害，严重狼疮肺炎，严重狼疮肝炎，严重血管炎等。,SLE的诊断思路(3个是否): 是否狼疮-是否活动-是否严重,Etiology and pathogenesis pathology Clinical findings Laboratory findings Diagnosis Treatment prognosis,治疗层次,基因 发病 机制,诱因,感染 药物 光照 激素等,SLE症状,发热 关节 皮肤 肾脏 心血管等,彻底治愈,祛除诱发因素,对症处理,致病基因位点,纠正免疫异常,general treatment drug treatment plasmapheresis haemopoietic stem cell transplantation,General treatment,心理治疗 急性活动期卧床休息，避免过劳 及早发现和治疗感染 避免使用可能诱发狼疮的药物 避免阳光暴晒和紫外线照射(SPF of at least 25) 缓解期才可作防疫注射 定期随访,Medications treatment,NSAIDS,Antimalarials,Corticosteroids,Immunosuppressant,Biological therapy,轻型SLE的治疗,1.非甾体抗炎药-关节炎 2.抗疟药-皮疹 3.小剂量激素 强的松0.5mg/kg口服6-8周后减量 4.酌情应用免疫抑制剂,非甾体抗炎药(NSAIDS),主要作用-抗炎止痛和退热 -对症治疗、无免疫抑制作用 用于治疗-发热 -关节痛 -肌肉痛 -轻度的浆膜炎 代表药物-阿斯匹林、消炎痛 -布洛芬、双氯酚酸、舒林酸、洛索洛芬 -塞来昔布、罗非昔布 副作用 -胃肠道,抗疟药(Antimalarials),主要作用-抗炎、免疫抑制