《狼疮性肾炎》ppt课件

狼疮性肾炎的维持缓解治疗 狼疮性肾炎ESRD的治疗 小结
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增殖性与严重增殖性狼疮性肾炎
增殖性狼疮性肾炎（Lupus Nephritis，LN）是指Ⅲ型、Ⅳ 型、Ⅲ+Ⅴ型、Ⅳ+Ⅴ型肾炎
严重的增殖性狼疮性肾炎包括Ⅲ型、Ⅳ型、Ⅲ+Ⅴ型、Ⅳ+Ⅴ 型重叠广泛的新月体、TMA、或严重的间质性肾炎
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确定狼疮性肾炎治疗策略的四原则
Stichweh D,. Curr Opin Rheumatol 2004 16:577–587
Bogdanovic R Pediatr Nephrol 2004 19:36–44
Ortega LM, et al: Lupus (2010) 19, 557–574
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International Society of Nephrology/ Renal Pathology Society (2003) Classification of Lupus
•治疗的短期和长期目标 •了解该患者狼疮性肾炎的性质（病理类型、严重程度、可
逆性、可能的反应） •系统性疾病的肾外表现，累计的肾外器官
Lupus nephritis class IV. Electron micrograph show- ing a large subendothelial electron-dense deposit as weห้องสมุดไป่ตู้l as a few small subepithelial deposits (arrow) (×1200).
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Lupus nephritis class III
Lupus nephritis class III. There is focal
segmental endocapillary proliferation (Jones methenamine silver, ×100).
The glomerular endocapillary proliferation is
spikes projecting outward from the
electron-dense deposits as well as
glomerular base- ment membranes toward mesangial deposits (×5000).
the urinary space (Jones methenamine
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狼疮性肾炎概述
•在未选择的病例大约有25%-50% 在SLE发病时就有临床肾 脏疾病，病程中成人中的60% ，儿童中的 80% 的SLE患者
会患狼疮性肾炎。 •LN累计患病率:亚裔为55%、非裔51%、西班牙裔43%、高
加索白人14%。 •非裔、西班亚裔和亚裔往往有更为严重的病例
•儿童易合并严重的肾炎，而老年人则较少。
狼疮性肾炎
北京协和医院
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目录
狼疮性肾炎概述 增殖性狼疮性肾炎的诱导缓解 狼疮性肾炎的维持缓解治疗
狼疮性肾炎ESRD的治疗 小结
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系统性红斑狼疮概述
SLE的人群发生率1.8 -7.6 ／10万人，患病率为40-200／10 万女性与男性之比为10:1
男性与女性狼疮，肾炎发生比例相同 发病15-45 岁，85%以上的患者发病年龄在55岁以前
discretely segmental with necrotizing
features and an early cel- lular crescent
h(Jones methenamine silver, ×400).
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Lupus nephritis class IV
Lupus nephritis class IV. There is global endocapillary pro- liferation with infiltrating neutrophils and segmental wire loop deposits (hematoxylin and eosin, ×320).
silver, ×800).
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其他病理学改变
间质－小管性改变 血管炎：非炎症性坏死性血管炎，类似于多血管炎，TMA
足细胞病：微小病变、FSGS或塌陷性肾小球病的
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狼疮性肾炎的临床表现
临床表现与肾小球病变相关，少数与血管炎及小管 间质性损害有关
临床沉寂性狼疮性肾炎及隐匿性h狼疮
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目录
狼疮性肾炎概述 增殖性狼疮性肾炎的诱导缓解治疗
global mesangial hypercellularity (periodic acid–Schiff, ×400).
Lupus nephritis class II. Electron
micrograph showing abundant mesangial electron-dense deposits (×12,000).
Nephritis (LN)
I Minimal mesangial LN II Mesangial proliferative LN III Focal LN* (<50% of glomeruli)
A A/C C IV Diffuse LN* (50% of glomeruli) Diffuse segmental (IV-S) or global (IV-G) LN
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Lupus nephritis class V
Lupus nephritis class V. Silver stain
Lupus nephritis class V. Electron micro-
highlights glomerular basement membrane graph showing numerous subepithelial
A A/C C V Membranous LN, III+V IV+V VI Advanced sclerosing LN (90% globally sclerosed glomeruli without residual activity)
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Lupus nephritis class II
Lupus nephritis class II. There is mild