系统性红斑狼疮SystemicLupusErythematosus.pptx

Systemic Lupus Erythematosus系统性红斑狼疮;SLE病因不详，疾病活动与疾病缓和交替过程 目前无治愈SLE办法 治疗目标-使疾病和机体和平相处 现今，许多患者不是死于疾病本身，而是死于医生治疗上过度热情 N E J M 2023;SLE诊断临床思绪;三步曲;1. Malar rash: Fixed erythema over malar areas, sparing nasolabial folds 2. Discoid rash: Erythematous raised patches with keratotic scaling and follicular plugging 3. Photosensitivity: Skin rash after exposure to sunlight, history or physical exam 4. Oral ulcers: Oral or nasopharyngeal, painless, by physical exam 5. Arthritis:Tenderness, swelling, effusion in 2 or more peripheral joints 6. Serositis: A) pleuritis or B) pericarditis 7. Renal disorder A) proteinuria0.5g/24hour or 3+ or B) cellular casts 8. Neurological disorder: A) seizures or B) psychiatric disorder (having excluded other causes, e.g. drigs) 9. Haematological disorder: A) haemolytic anaemia or B) leucopenia or C) thrombocytopenia 10. Immunologic disorder: A) positive LE cells or B) raised anti-native DNA antibdy binding or C) anti-Sm antibody or D) false positive serological test for syphilis. 11. Positive antinuclear antibody:;-狼疮活动度评定;SLE disease activity index (SLEDAI);常用药品;病人教育和随访;轻型SLE--Mildly active lupus;轻型SLE--Mildly active lupus;HCQ-治疗SLE基础药品;HCQ能够预防SLE复发、减轻器官损害、有助于对MMF治疗反应;SLE系统体现及处理标准;一般症状;2023年1月至2023年5月，SLE住院患者共1949人 发热定义：连续3d以上口温37.5℃。排除手术后应激性发热（≤38.0℃，＜3d）、输血反应或输液反应引发发热 ;;;Figure 1. Response of SLE fever to steroid therapy: the effective dose to suppress SLE fever (temperature resolved).;Figure 2. Response of SLE fever to steroid therapy: the maximum dose that failed to suppress SLE fever (temperature unresolved).;大部分患者（80.6%）在给予相称于强松剂量（P）≤100mg/d（相称于甲基强松龙80mg）时，体温即可恢复正常。 反观不能控制体温激素剂量，当P100mg/d时只有6.3%患者病情不能得到缓和。其中， 当激素剂量在150mg/dP≤300mg/d患者有4例，包括3例狼疮脑病和1例嗜血综合征患者；300mg/dP≤625mg/d为1例狼疮脑病患者； 625mg/dP≤1250mg/d时，只有1例严重狼疮脑病患者接收甲基强松龙1250mg冲???治疗无效，加用利妥昔单抗（商品名：美罗华）治疗后好转。;; ;皮肤体现;Malar rash;Discoid lupus;Alopecia ;皮疹处理;关节和肌肉;肾 脏;第31页;WHO classification of lupus nephritis;ISN/RPS 2023年狼疮性肾炎分型简化版 ;半定量测定狼疮肾炎病理活动和慢性指标;狼疮肾炎治疗;第36页;event-fre